ABNORMAL TONE AND MOVEMENT 95 whereby the extended leg is hitched forwards in foot, the triceps surae and the Achilles tendon in order to step through (Dietz 1992). This action the first instance, in an attempt to desensitise demands greater activity from, in particular, against both the intrinsic and extrinsic stimuli latissimus dorsi with a resultant shortening of (Lynch & Grisogono 1991). Therapists are often the trunk side flexors. The insertion of latissimus advised not to stimulate the ball of the foot but in dorsi at the humerus produces medial rotation of this instance appropriate handling and facilita- the upper limb and each time the patient at- tion of transference of weight over the foot are tempts to hitch the leg forwards, the trunk side important to reduce the effects of this reaction. flexors and the upper limb demonstrate an increase in pathological flexor activity, through Botulinum toxin is increasingly being used to the action of latissimus dorsi. The hypertonus of weaken the dominant muscle groups responsible the upper limb appears to be influenced by the for the positive support reaction (Dengler et al amount of effort expended by the patient to step 1992, Hesse et al 1994, Dunne et al 1995, forwards with the affected leg. This seemingly Richardson et al 2000) (see Chapter 7). Used in proportional response, of increased hypertonus conjunction with physiotherapy and in some in the upper limb being determined by the effort cases splinting (Scrutton et al 1996), this has necessary to accomplish a step, is an example of shown promising results. what is referred to as an associated reaction (see p. 97). Flexor withdrawal response of the leg Flexor withdrawal is a response which occurs in The positive support reaction will also affect the normal subjects and is well illustrated by with- ability to stand up from sitting and sit down from drawal of the hand from a hot stove, or the foot standing. The resistance from the hypertonic when stepping on a drawing pin. It is associated plantar flexors prevents the transference of weight with the crossed extensor reflex (Magnus 1926 as forwards over the foot as the individual prepares cited by Bobath 1990, Schomburg 1990). The to stand. The leg becomes stiff in extension and the withdrawal response is determined by the direc- patient is pushed back into the chair. The situation tion of the noxious stimulus and therefore may is reversed when attempting to sit down. The leg is not necessarily be into flexion (Rothwell 1994). unable to flex and the patient falls back into the chair. Patients with hemiplegia will tend to stand Patients with neurological impairment may up and sit down predominantly on their sound leg demonstrate this response in a more stereotyped because of this inability to take weight through the and consistent pattern. A minimal stimulus, not affected limb. necessarily noxious - for example, removing the bedclothes from a patient following spinal cord More recently, a flexor component of the posi- injury - may be enough to elicit this response tive support reaction has been observed in clini- cal practice. As the patient attempts to bring the Figure 5.2 Flexor withdrawal. leg forwards there is a notable increase in flexor activity. In this situation, the sensitivity of the foot remains the primary problem, but the weight-bearing mechanics are altered. The foot is maintained in a position of inversion and the ball of the foot does not make contact with the ground, leading to adaptive shortening of affected muscle groups. Weight is taken over the lateral border of the foot with an almost instant withdrawal into flexion as an attempt is made to step through. Treatment of the positive support reaction should be directed towards mobilisation of the
96 NEUROLOGICAL PHYSIOTHERAPY (Edwards 1998). The response in the lower limbs may occur through forcing a malaligned joint. As is that of flexion, abduction and lateral rotation at a general rule, any intervention should be pain- the hip, flexion at the knee and inversion of the free, it being impossible to inhibit pathological foot in conjunction with dorsiflexion or plantar or, indeed, normal protective muscle spasm in flexion of the ankle (Fig. 5.2). the presence of pain. The problem is often exacerbated by shorten- Davies (1985) advocates the use of a back slab ing of the dominant muscle groups, particularly to maintain the leg in extension and thereby facil- the hip and knee flexors which may lead to sub- itate weight bearing. This procedure may be of sequent shortening of the erector spinae. This benefit in the early stages of counteracting response may be stimulated by stretch of these increasing flexor activity of the leg but should be muscles, which prevents the patient from stand- used with caution in patients with more long- ing up effectively (Lynch & Grisogono 1991) and standing problems, particularly where there is from placing the foot on the floor. loss of range. Attempts to force the leg into a back slab may cause pain and thereby nullify any In clinical practice this response may be seen in positive effects. patients with severe neurological damage such as may occur following complete spinal cord tran- More recently, intrathecal baclofen has been section (Brown 1994). It may also be observed in used to good effect in carefully selected patients patients with neurological impairment following with severe flexor hypertonus (Porter 1997). painful trauma such as a fracture of the neck of Details of this treatment intervention are to be femur. Pressure sores, or other painful stimuli of found in Chapter 7. the foot in particular, may also give rise to a flexor response (Schomburg 1990). Grasp reflex Removal of the noxious stimulus and/or man- The grasp reflex is a normal phenomenon in agement of the primary trauma are of immediate human infants which disappears within a few concern in treating the manifestations of this months of birth. In the adult it is always patho- response. The short-term influence of such a logical (Rothwell 1994). The stimulus for initiat- stimulus may prove to be readily reversible, ing this response is to the hand and fingers, whereas prolonged exposure, particularly to particularly the palm. Desensitisation of this pain, will have more residual effects, such as the reflex must therefore incorporate mobilisation establishment of contractures. and appropriate tactile stimulation to the hand within the patient's tolerance. This must involve Treatment by physiotherapy will be deter- all personnel involved in the management of mined by the extent and severity of structural such patients, not least the patients themselves changes which may have occurred. Attempts to and their carers. Movements of the hand should stand a patient on a leg which is mechanically also include an appropriate response from the altered due to contracture will further aggravate whole of the upper limb and, wherever possible, the situation, not least by imposing an additional produce a functional outcome. painful stimulus. It is essential first to regain the required range of movement at the hip and The use of splinting to prevent contracture in the pelvis, thereby allowing the leg to be released presence of a severe grasp reflex remains a contro- sufficiently to attain its normal length. versial issue and is discussed in Chapter 10. 'Normal' standing can only be achieved on the Extensor response basis of mechanical and structural alignment of the pelvis and lower limbs. In their absence com- This response may be observed following severe pensation, particularly at the trunk and pelvis, head injury and in people with multiple sclerosis is inevitable. Mobilisation of shortened muscles or dystonic/athetoid cerebral palsy. Extreme and soft tissues may cause some discomfort, but cases will include extension of all four limbs it is important to distinguish between pain result- ing from muscle stretch and joint pain which
ABNORMAL TONE AND MOVEMENT 97 with arching of the spine (see Rigidity, p. 105). Figure 5.3 Mobilisation of extensor response over ball. It is initiated and perpetuated by stimulation and contact between the back of the head and patient attaining the standing position. Dif- the supporting surface and is exacerbated in ficulties may arise using this method as the table extensor positions such as supine lying. provides a rigid support against which the patient can push and, at the same time, mini- Patients described as displaying the 'out of mises the opportunity of mobilising the patient line' or 'pusher' syndrome (Davies 1985) may while upright against gravity (see Ch. 6). also demonstrate an extensor thrust of the head and upper trunk but this is often in conjunction The extensor response is invariably accompa- with flexor hypertonus of the affected lower leg. nied by plantar flexor hypertonus. Treatment as advocated above may have some influence on There are differing degrees of severity but it is this particular aspect of management. Unfor- important to recognise the influence of positions tunately, contracture of the triceps surae is a of extension and the susceptibility of a stimulus - well-documented complication of extensor hyper- for example, the head support of a wheelchair - tonus (Booth et al 1983, Yarkony & Sahgal 1987, in triggering this response. It may be an extensor Kent et al 1990, Davidson & Waters 1999). thrust which is responsible for preventing the Application of below-knee inhibitory casts as a patient from accepting the support of a chair, prophylactic measure has been recommended as with the result that he is constantly pushing a means of preventing or minimising this dis- himself out (see Ch. 9). If this is the case, it is abling complication (Conine et al 1990, Moseley important to desensitise this response rather than 1993,1997) and is discussed in Chapter 10. merely attempting to restrain the patient in the chair. Associated reactions The extensor response is indicative of gross Associated reactions (ARs) were originally cerebral pathology, and it may be questioned described as 'released postural reactions in whether any physiotherapeutic intervention can muscles deprived of voluntary control' (Riddoch have an effect on outcome. It is difficult to & Buzzard 1921, Walshe 1923) and appear as prevent the development of contractures if the involuntary stereotyped abnormal patterns of patient is constantly held in this extreme position movement at a body segment of the affected side, of extension. Physiotherapy is based on the prin- which coincide with or follow effort exerted at ciple that changes in the environment will influence potential for change in the CNS. Modification of the extended position to intro- duce an element of flexion will create a tem- porary release from what can only be assumed to be a painful maintenance of extensor hypertonus. This may be achieved not only by modifying the supporting surface of the bed, as described in Chapter 6, but also by the use of the gymnastic ball whereby the patient is mobilised into flexion over an adaptable as opposed to a rigid surface (Fig. 5.3). The patient may also be facilitated into stand- ing, preferably with a therapist in front to support the knees and another behind to provide a mobile support in an attempt to prevent stimu- lation of the thrust into extension. A tilt table is often used for this purpose and in some instances this may be the only practicable way of the
98 NEUROLOGICAL PHYSIOTHERAPY another body site (Davies 1985, Dickstein et al to describe involuntary limb movement. In addi- 1996). They are thought to be pathological move- tion to the terms ARs and associated movements, ments which are indicative of the potential for motor overflow has also been used to describe development of hypertonus or an accentuation of this phenomenon (Lazarus 1992). the prevailing spastic synergy (Bobath 1990). ARs have been described previously in rela- ARs may occur throughout the body but are tion to compensation for the positive support most apparent distally, such as in the arm and reaction (see p. 95). The extensor hypertonus in hand and the leg and foot. They may be initiated the lower limb necessitates increased effort in not only as a result of attempted movement but taking the leg forwards during the swing phase also at the preparatory stage of movement of gait. This effort is reflected in an increase of (Dickstein et al 1995) and they may also be ob- tonus throughout the affected side, producing, in served with involuntary actions such as yawning, many instances, a stereotyped response of flexor coughing and sneezing (Mulley 1982). Attempts hypertonus in the arm and side flexion of the to communicate by patients with dysphasia or trunk. It is suggested that this pathological cognitive problems may also produce this response is indicative of the severity of the response. hypertonus (Dvir et al 1996). Dickstein et al (1995) also reported an increase in EMG activity Following damage to the central nervous of the biceps muscle of the paretic upper limb in system, it is generally believed that the severity stroke patients, which was associated with foot of the ARs is directly related to the degree of lifting, and in many instances this EMG activity hypertonus: the more severe the hypertonus, the started before the foot was lifted. However, this more forceful and longer lasting the associated response also occurred, although to a lesser reaction and the larger the magnitude of the limb degree, in the non-paretic limb and in healthy excursion (Davies 1990, Cornall 1991, Dvir & controls. Panturin 1993, Dvir et al 1996). However, Dickstein et al (1996) suggest that this relation- The use of ARs has been advocated to describe ship is not so clearly defined. When examining and grade the severity of hypertonus, to evaluate the displacement of the paretic elbow into flexion and direct therapeutic intervention and as a of hemiplegic patients during walking, they prognostic indicator (Davies 1985, 1990, Bobath identified two distinct movement stages. The 1990, Lynch & Grisogono 1991). Dvir & Panturin first abrupt increase in elbow flexion, which (1993) found that improvement in effort-related occurred during the first four steps, appeared to ARs mirrored a decrease in hypertonus and, in a be reflexive in nature, whereas they suggest that study by Cornall (1991), ARs were used to assess the preservation of elbow flexion during walking the effects on hypertonus when self-propelling a may be an expression of stiffening of the elbow wheelchair. flexor muscle fibres. In a recent small-scale survey of physiotherapy Stephenson et al (1998) highlighted the con- departments in the Yorkshire region of England, fusion in terminology between ARs and associ- the use of ARs to grade hypertonus and to guide ated movements. Bobath (1985) makes a clear therapeutic intervention was found to be the distinction between ARs, which are always most common means of assessing hypertonus in pathological, and associated movements, which the clinical setting (Stephenson 1996). The use of are normally coordinated movements occurring this assessment scale, referred to as a 'Bobath' as a result of reinforcement of movements initi- assessment, as a clinically relevant measure, was ated and performed with effort, such as when supported by Lynch-Ellerington (1998) on the learning a new skill. In contrast, Carr & Shepherd basis that 'the facilitation of the control of associ- (1980) and Shepherd (1998) refer to associated ated reactions by the patient produces the movements in the context of both normal and rewards of recovery of sensation, proprioception, pathological response to concentrated or effortful posture and selective movement and hence movement, and Brunnstrom (1970) uses this term function'. However, although it may appear
ABNORMAL TONE AND MOVEMENT 99 logical that ARs are a positive feature of the Secondary effects include muscle atrophy and the UMN syndrome, there is no conclusive evidence potential for development of contractures. with regard to reliability, validity or responsive- ness that this is the case (Stephenson et al 1998). Specific interventions such as mobilisation, Furthermore, they are variable, their magnitude stretch of affected muscle groups and possibly and duration differing drastically on repeated splinting are clearly indicated to prevent muscle trials (Dvir et al 1996) and, therefore, it has been shortening and to enable muscles to be activated suggested that they may not be the most appro- more efficiently, but to what extent should the priate means of measuring hypertonus (Haas patient be encouraged to use graded resisted 1994). exercises? The theory that resisted exercise causes a marked loss of coordination and an Clinical implications. It is suggested that sus- increase in co-contraction was refuted in a recent tained ARs, may give rise to a deteriorating level study by Miller & Light (1997), who concluded of function in that movement. Repeated move- that strength training with paretic muscles could ments, for example of the arm into flexion, may benefit individuals post-stroke. Furthermore, lead to a gradual loss of range and ultimately aerobic exercise has also been shown to be contracture (Davies 1990, Dvir & Panturin 1993). related to improvement of overall sensorimotor Movement requiring excessive effort is cited as function after stroke (Potempa et al 1996). causing this pathological response, and assis- tance from the therapist to reduce the amount of Tone reduction is clearly indicated for patients effort required is recommended (Davies 1994). with pathological overactivity in dominant muscle groups. However, if functional tasks Although ARs are generally associated with produce an increase in tone, for example an hypertonus, their appearance and severity may associated reaction, should this activity be dis- be due to underlying low tone. Patients with couraged? Walking is an oft-cited example of hypotonia may demonstrate marked ARs due to function versus quality of movement and many lack of stability at the trunk and proximal key therapists recommend that patients avoid this points. Unless this true cause is identified and activity until they are deemed (by the physio- addressed in treatment, 'inhibition' of ARs will therapist) to be 'ready to walk' (Davidson & have no functional carry-over. While mobilising Waters 2000). However, as suggested in this and stretching the upper limb may be effective in study, if delay in walking is advocated on the maintaining range of movement, there will be grounds of quality issues, the benefits and effect- no lasting change without improved proximal iveness of treatment need to be substantiated. stability and without the patient being able to actively use the affected limb. Physiotherapy intervention to assess and treat the patient with hypertonus is determined by Aims of physiotherapy intervention assumptions about its nature and cause. If the in the management of hypertonus neural components of hypertonus are the per- ceived problem, the purpose of therapy will be to There are many assumptions and beliefs regard- 'inhibit' the stereotyped response and to facilitate ing the treatment of hypertonus, many of which more normal postures and movement patterns. are unsubstantiated. One major issue is whether However, while reducing or eliminating hyper- weakness is apparent or real. Bobath (1990) tonus may increase range of movement, this may attributes the 'weakness' of hypertonic muscles to reveal underlying weakness rather than under- exaggerated co-contraction of opposing muscle lying control (Giuliani 1992, Nielsen & Sinkjaer groups and hypothesises that resisted exercise 2000). The main influence of tone reduction would and effort will further increase the hypertonus. appear to be on the non-neural components. Main- However, as discussed above, there is now con- taining or increasing the length of muscle gives a clusive evidence that weakness is a very real and better biomechanical advantage for optimal force disabling feature following UMN lesions. production, more efficient activation of muscle and the potential for carry-over into functional tasks.
100 NEUROLOGICAL PHYSIOTHERAPY Conventional clinical practice in the UK is occur with multi-joint movements such as reach- based on the Bobath approach. This approach ing (Sainburg et al 1993, 1995). When assessing emphasises the facilitation of normal muscle tone the impact of sensory loss on upper limb func- and movement; activities which perpetuate or tion, the importance of cutaneous input from increase abnormal tone are strongly discouraged the palmar aspect of the hand and fingers must (Bobath 1990). However, research evidence for be recognised (Johansson 1996). These patients the effectiveness of interventions indicates that often compensate for poor grip control by apply- resisted exercise training and using equipment ing excessive force (Carey 1995). such as the treadmill, splints and walking aids enhance performance of movement (Pomeroy & Vestibular ataxia Tallis 2000). This may occur with peripheral vestibular dis- The primary goal of physiotherapy is to orders or central disorders which affect the ves- change functional performance, and one aspect tibular nuclei and/or their afferent/efferent of treatment to achieve this goal is the reduction connections, for example with medullary strokes of hypertonus. However, merely to reduce (Brandt & Dieterich 2000). Peripheral lesions hypertonus is not the answer. The patient must may be either bilateral or, more commonly, be active in order for there to be any influence on unilateral (Fetter 2000). the neural components of tone because, without this, no carry-over is possible. The vestibular apparatus comprises the semi- circular canals and otoliths that detect angular ATAXIA and linear acceleration of the head, respectively. The otoliths also detect the position of the head Ataxia is a general term meaning a decomposi- with respect to gravity, thus contributing to the tion of movement (Holmes 1939). Morgan (1980) person's orientation to gravity and the midline describes three main types of ataxia: sensory, (Hain et al 2000). The vestibular apparatus is labyrinthine (vestibular) and cerebellar. involved in the initiation and modulation of pos- tural adjustments and in stabilising the head Sensory ataxia against gravity (Allum et al 1997, Horak & Shupert 2000). The patient with vestibular ataxia This results from lesions affecting peripheral may have poor righting reactions and abnormal- sensory nerves conveying proprioceptive and ities in postural strategies in both sitting and cutaneous information or in conditions affecting standing. the dorsal columns or the primary sensory cortex (Morgan 1980). Symptoms vary depending on Patients with bilateral peripheral lesions often the pattern of sensory loss. For example, proprio- lean backwards in standing while those with a ceptive input from both the distal legs and the unilateral lesion tend to lean towards the side lumbar spine and pelvis are essential for initiat- of the lesion. They tend to stagger and have a ing and modulating postural adjustments in wide-based gait. To maintain standing balance, standing (Ingilis et al 1994, Allum et al 1995, patients are often over-reliant on one of the Allum 1996). Patients with sensory loss of the remaining sensations, such as vision, often to the lower limbs have a wide-based gait with eyes exclusion of other potentially useful sensations fixed to the ground for visual feedback (Lajoie such as somatosensory information or any et al 1996). The amplitude of postural sway when remaining vestibular information. This inappro- standing with feet together is greatly increased priate reliance may lead to disequilibrium when with the eyes closed (Notermans et al 1994). these sensory cues are absent, reduced or in- appropriate (Shumway-Cook et al 1996, Herdman Patients with loss of proprioception of the & Whitney 2000). Head, trunk and subsequently upper limb are unable to plan for or compensate upper limb movements are often decreased and for the biomechanical variations that naturally disequilibrium is increased by head movement
ABNORMAL TONE AND MOVEMENT 101 while walking (Gill-Body & Krebs 1994, Herdman • Postural tremor, which occurs when holding a & Whitney 2000). limb in a given position. Vestibular ataxia may be accompanied by poor • Titubation, which is tremor affecting the head perception of the midline, vertigo, blurred vision and upper trunk, typically occurs after lesions and nystagmus due to the vestibular system's of the vermis. role in sensing and perceiving self-motion and in stabilising gaze (Horak & Shupert 2000). It is • Postural truncal tremor, which affects the legs important to determine the factors that precipi- and lower trunk, is typically seen in anterior tate these distressing but potentially treatable cerebellar lobe lesions as often results from symptoms as they may greatly limit functional chronic alcoholism (Thompson & Day 1993). recovery. Patients may limit head movements to avoid precipitating vertigo, leading not only to Dyssynergia and visuomotor incoordination. neck stiffness but also to inadequate use of somato- sensory or remaining vestibular information Dyssynergia is the incoordination of movement which is important for the recovery of gaze involving multiple joints. The cerebellum is control and balance (Herdman & Whitney 2000). involved in programming, initiation and ongoing control of multi-joint movements towards visual Cerebellar ataxia targets (Stein 1986, Thach et al 1992, Haggard et al 1994). The influence of the cerebellum in the initi- This results from lesions affecting the cerebellum ation of a movement can be seen in the increased or its afferent or efferent connections. The cere- reaction time to a given task after cerebellar bellum may be divided into specific functional dysfunction (Diener & Dichgans 1992). areas (Thompson & Day 1993). Lesions of the midline structures, the vermis and flocculo- The cerebellum is also vitally important for nodular lobe produce bilateral symptoms affect- the control of eye movements and eye-hand co- ing axial parts of the body, which manifest as ordination (Dichgans 1984, van Donkelaar & Lee truncal ataxia and titubation and abnormalities 1994). During reaching, coordination of the eye of gait and equilibrium. Occulomotor abnormal- and hand is essential for accuracy (Jeannerod ities and dysarthria may also be associated with 1988). Abnormalities in multi-joint movements, this pathology (Lewis & Zee 1993). Lesions eye movement and their coordination all occur in affecting the hemispheres give rise to ipsilateral cerebellar ataxia and therefore all contribute to limb symptoms (Ghez 1991). the inaccuracy of reaching seen after a cerebellar lesion (van Donkelaar & Lee 1994). Symptoms associated with cerebellar ataxia Dysdiadochokinesia. This is the inability to Dysmetria. This refers to inaccuracy in achiev- perform rapidly alternating movements such as ing a final end position (hypermetria equals alternately tapping with palm up and palm overshoot; hypometria equals undershoot). This down. The rhythm is poor and the force of each is clearly demonstrated by the patient attempting tap is variable (Diener & Dichgans 1992, the finger-nose test. The movement may over- Rothwell 1994). shoot or undershoot the target, with over- correction resulting in additional movements. Posture and gait. Patients with anterior lobe lesions show an increased postural sway in an Tremor anteroposterior direction, which is increased with eye closure. Patients with lesions of the • Kinetic tremor, which is the oscillation that vestibulocerebellum (flocculonodular lobe) show occurs during the course of the movement. an increased postural sway in all directions. The effect of eye closure on postural sway in these • Intention tremor, which is the increase in patients is minimal (Mauritz et al 1979, Diener tremor towards the end of the movement. et al 1984). Following cerebellar lesions, postural adjust- ments may be impaired. Patients with anterior lobe damage show poorly scaled, hypermetric responses to a sudden unexpected movement
102 NEUROLOGICAL PHYSIOTHERAPY that leads to instability (Horak & Diener 1994, (Thach et al 1992). This theory remains somewhat Timman & Horak 1998). The abnormalities in controversial (Cordo et al 1997). Patients with predictive and reactive postural adjustments and cerebellar cortical atrophy have recently been the presence of dysynergic leg movements may shown to be deficient in learning a conditioned contribute to the staggering gait, with poor pre- reflex (Topka et al 1993) and more complex motor cision of foot placement seen in these patients skills (Sanes et al 1990, Thach et al 1992), which (Diener & Dichgans 1996). may have implications for the effectiveness of physiotherapy intervention (Hardie 1993). Other symptoms associated with cerebellar dysfunction Cognition. A cognitive affective syndrome has been described in subjects with specific Hypotonia. This occurs with acute cerebellar cerebellar lesions (Schmahmann 1997, 1998). lesions (Holmes 1922) but it is rarely seen in Symptoms include executive deficits, such as dif- chronic lesions (Gilman 1969, Diener & Dichgans ficulties with planning, working memory and 1992, Rothwell 1994). Physiotherapists often view poor visuospatial memory, as well as abnormali- hypotonia as a symptom of ataxia (Atkinson 1986, ties of personality and behaviour. These cog- Davies 1994). However, although hypotonia is a nitive deficits may further affect both the symptom of cerebellar dysfunction it is not a patient's function and the treatment strategies causative factor of ataxia and usually disappears that the clinician may adopt (Chafetz et al 1996). within a few weeks following acute lesions (Diener & Dichgans 1992). Treatment strategies Weakness and fatigue. Holmes (1922, 1939) Cerebellar ataxia. Many strategies have been describes a generalised non-specific weakness as advocated in the treatment of cerebellar-type a feature of cerebellar dysfunction. This occurs ataxia. They include the use of weights (Morgan more often with extensive and deep lesions and et al 1975), manual guidance/resistance (DeSouza is most apparent in the proximal musculature. 1990), rhythmic stabilisations (Gardiner 1976), The weakness decreases over months; the horse riding (Saywell 1975) and the gymnastic maximal voluntary contraction of a patient with ball (Hasler 1981). Factors that increase the a chronic cerebellar disorder is similar to that of a weight of a limb or its resistance to movement, normal subject (Mai et al 1988). However, on clin- such as the application of weights, decrease ical observation, it would seem that patients with tremor (Morgan et al 1975). However, tremor will long-term ataxia do show signs of weakness. return after removal of a weight, often greater This may result from their lack of spontaneous than before, and can cause fatigue, particularly in movement and the adoption of compensatory patients with multiple sclerosis (DeSouza 1990). movement strategies. Frustration may lead to the The use of weights to reduce truncal tremor has patient with chronic ataxia becoming increas- shown variable results (Lucy & Hayes 1985). ingly inactive and dependent and, therefore, More recently, there has been renewed interest in merely preventing disuse may be helpful (Hardie thalamotomies and thalamic stimulation as treat- 1993). Fatigue has also been noted as a common ments for the symptomatic relief of tremor feature of cerebellar dysfunction (Holmes 1922). (Nguyen et al 1996, see Ch. 7). Motor learning. It is generally agreed that Ataxia is primarily a disorder affecting pos- motor learning is a process which involves the tural control and the coordination of multi-joint whole nervous system (Halsband & Freund movements (DeSouza 1990). The patient is aware 1993). However, some researchers feel the cere- of the movement disorder and adopts com- bellum to be a major site of motor learning in that pensatory strategies to effect function (Gordon it is specialised to combine simple movements 1990). An example of this is the use of excessive into more complex synergies. These synergies or fixation when using gait aids to improve stability motor patterns are then stored in the cerebellum (Balliet et al 1987).
ABNORMAL TONE AND MOVEMENT 103 There is a greater deficit in multi-joint as This need to facilitate vestibular adaptation by opposed to single-joint movement (Goodkin et al stressing the relevant system may also have 1993) and Bastian (1997) has suggested that the important implications for the improvement of use of single-joint, as opposed to multi-joint balance control. As Brandt et al (1981) state, a movements, should be encouraged to improve therapist should: function. However, although some fixation may expose the patient increasingly to unstable body be inevitable and possibly even useful, it does positions in order to facilitate rearrangement and limit the potential for improving proximal con- recruitment of control capacities. Stance and gait aids trol and multi-joint movements. Specific mobil- will alleviate only transiently patients' balance isation, particularly of the head and trunk, in problems, but when used continuously, they will conjunction with goal-directed, multi-joint worsen the symptoms. movements have been recommended (DeSouza 1990). This may be because incorrect use of a gait aid does not allow the patient to generate an error Vestibular ataxia signal that is required to improve their balance. However, in clinical practice it has been observed Unilateral vestibular lesions. Improvement of symptoms following a unilateral vestibular lesion Figure 5.4 Patient with ataxia using a rollator walking requires the active participation of the patient, frame. although some spontaneous recovery may occur (Zee 2000). Treatment approaches vary: with habituation exercises, symptoms decrease with repeated exposure to the provocative stimulus (Herdman & Whitney 2000). The mechanisms of this action are unclear but it is this principle that underlies treatments for vertigo and impaired balance (Cawthorne 1944, Cooksey 1946, Norre & DeWeerdt 1980). Although specific regimes differ, positioning and movements that provoke symp- toms are performed two to three times a day. As the symptoms improve, the position may be changed or the speed of movement increased. Other exercises have been developed that utilise the vestibular system's ability to increase the size (or gain) of an eye movement or postural response to a given stimulus induced by head motion or position. When the patient moves, an error signal is produced that the vestibular system is not functioning optimally. The CNS then attempts to reduce this error signal by increasing the response of the vestibular system (Curthoys & Halmagyi 1995). Such an error signal must be produced by active movement of the patient, such as when moving the head while attempting to visually fixate a target. If gaze sta- bility is poor, the image will move, producing an erroneous retinal slip. With repeated movements over time the vestibular system adapts, the eye movements increase in amplitude and this retinal slip is decreased (Herdman & Whitney 2000).
104 NEUROLOGICAL PHYSIOTHERAPY that use of a rollator walking frame often serves (Igarashi et al 1988, Krebs et al 1993) and com- to provide the patient with a means of more inde- pensatory strategies, such as turning on the light pendent control. Used selectively, with emphasis before getting out of bed, are recommended on it being an aid to balance as opposed to a (Herdman & Clendaniel 2000). point of fixation, patients may use this aid as a means of progression to an independent gait Central lesions also demonstrate more variable (Jeka 1997; Fig. 5.4). Eventually, the majority of recovery as the areas damaged - the brain stem, patients with unilateral lesions should be able to cerebellum and vestibular nuclei - are implicated walk without an aid. in the process of recovery (Igarashi 1984). This is of particular relevance in the treatment of trau- Importantly, the vestibular system demon- matic head injury where vestibular disorders strates context specificity; that is, improvement are thought to occur in 30-65% of patients will only be seen in those movements and posi- (Shumway-Cook 1994). tions performed by the patient. Any treatment regime must therefore address those movements Sensory ataxia. Compensation with remaining that precipitate, for example, disequilibrium senses is encouraged, such as that of vision as in the environment that they are normally advocated by Frenkel's exercises (Hollis 1989). experiencing (Curthoys & Halmagyi 1995). However, the over-reliance on conscious effort with these exercises detracts from the desired Finally, improvement in symptoms may occur automatic response. through substitution of absent vestibular input with other sensory inputs. For example, patients Studies have demonstrated that the sensory should be encouraged to learn to use all available cortex can display some capacity for reorganisa- sensations to balance. If they are over-reliant on tion and recovery following discrete lesions one sensation, such as vision, they should be (Dannenbaum & Dykes 1988, Dannenbaum & taught to balance in situations where visual cues Jones 1993, Xerri et al 1998) and, in these are absent or reduced. Although useful, visual cases, sensory re-education may be beneficial. and somatosensory information cannot perfectly However, recovery in the sensibility of the replace the lost vestibular function. Even follow- hand will only occur if the area of sensory loss is ing a unilateral lesion, some gaze control and small and if the patient has the required mobility balance symptoms may still be experienced, and strength to use the arm functionally (Carey especially with fast movements (Herdman & 1995). Whitney 2000). Finally, if sensory loss is accompanied by a loss In summary there are many ways in which of thermal sensibility, care must be taken to vestibular function may be improved. The exer- avoid injury and maintain skin integrity (Carey cises recommended are similar despite different 1995). underlying principles. The accumulation of exper- imental studies of individually prescribed exer- MOVEMENT DISORDERS cises, performed under supervision or at home, ASSOCIATED WITH BASAL GANGLIA highlights the effectiveness of this approach DISEASE (Strupp et al 1998, Yardley et al 1998, Blakely 1999, Herdman et al 2000). The movement disorders that result from basal ganglia damage or disease are often dramatic, Bilateral vestibular lesions. With a complete and depending on the site of the lesion, can cause bilateral vestibular lesion, improvement in ataxia extreme slowness of movement and rigidity, or can only occur by substitution of visual and, uncontrollable involuntary postures and move- more importantly, somatosensory cues. Recovery ments. There also appears to be some defect of after a bilateral vestibular lesion often takes attention that is important in diseases of the basal longer and is more incomplete than after a uni- ganglia. This may be observed in patients with lateral lesion. Even so, physical exercise has been Parkinson's disease who are so akinetic that they demonstrated to affect the rate of recovery can barely move and yet may deftly sidestep an
ABNORMAL TONE AND MOVEMENT 105 oncoming car or flee from a fire (Brown & RIGIDITY Marsden 1998) and in dystonia, where in spite of grossly distorted postures, the patient may Rigidity is clinically defined as increased resist- accomplish surprisingly skilled tasks (Marsden ance to stretch and the inability to achieve com- 1984, Britton 1998). plete muscle relaxation (Wichmann & DeLong 1993). The stiffness or involuntary muscle con- Mink (1996) hypothesised that the basal traction is maintained throughout the range of ganglia do not generate movements, but that the movement, relatively independent of the velocity tonically active inhibitory output of the basal of stretch and for as long as the stretch is main- ganglia acts as a 'brake' on motor pattern gener- tained (Burke 1987). In parkinsonian rigidity, ators (MPGs) in the cerebral cortex (via the thala- tendon jerks are usually normal (Marsden 1982, mus) and brain stem. When a movement is Messina 1990, Rothwell 1994). initiated by a particular MPG, basal ganglia output neurones projecting to competing MPGs Several factors may contribute to the increase in increase their firing output, thereby increasing resistance during passive movement of the limbs. inhibition and applying a 'brake' on those gener- These include (a) inability of the patient to relax ators. Other basal ganglia output neurones pro- and completely eliminate activity in the muscles; jecting to the MPGs involved in the desired (b) increased stiffness due to altered viscoelastic movement decrease their discharge, thereby properties of the muscles; (c) abnormal co-activa- removing tonic inhibition and releasing the tion of agonist-antagonist muscle groups; and (d) 'brake' from those desired generators. In this increased stretch reflexes (Lee 1989). Enhancement way, selected movements are enabled and com- of long-latency stretch reflexes and a reduction in peting postures and movements are prevented inhibition of interneuronal circuits have been from interfering with the one selected. implicated in parkinsonian rigidity (Hallett 1993) and it is suggested that there may be an inability to The example cited in this review is that of suppress unwanted reflex activity generally (Mink reaching to pick an apple off a tree. While the 1996). most obvious feature is that of reaching, multiple other motor mechanisms act together to maintain Rigidity may occur in different forms. It is one of the upright posture of the body. Prior to the the cardinal features of Parkinson's disease and reach, these mechanisms are also active in the may also occur with midbrain and brainstem le- reaching arm to maintain its posture. However, sions. However, decerebrate and decorticate rigid- as the arm reaches towards the apple, the pos- ity are abnormal postures associated with coma tural mechanisms must be turned off selectively rather than a specific type of hypertonus, and in the arm while they maintain activity in the Britton (1998) suggests that the terms decorticate rest of the body. When the reach is completed, and decerebrate posturing would be more appro- the reaching MPGs must be turned off and priate. Burke (1987) supports this view in that, the postural mechanisms turned back on. If the with 'decerebrate rigidity', the patient is usually competing posture-holding and reaching mech- flaccid at rest and transiently assumes the rigid anisms were inappropriately active at the posture when stimulated. Rigidity also occurs with same time, the result may be instability of pos- stiff man or stiff limb syndrome (Barker et al 1998). ture or slowing of movement or both (Mink 1996). Marsden (1982) identified excessive and uncon- trollable supraspinal drive to alpha motoneurones When the balance of inhibition and excitation as the most important cause of rigidity in in the basal ganglia and motor cortex is upset, the Parkinson's disease. He concluded that rigidity symptoms and signs of rigidity and involuntary movements supervene along with abnormalities represents a positive symptom resulting from release of posture and associated movement, and slow- of other brain structures that are normally inhibited ness of movement (Jones & Godwin-Austen by basal ganglia function. Rigidity indicates the 1998). operation of remaining intact motor systems and tells little of the normal function of the basal ganglia.
106 NEUROLOGICAL PHYSIOTHERAPY The clinical presentation of rigidity and poss- Although these different definitions are used, ible treatment strategies will be discussed in the they would appear to be of academic rather than context of Parkinson's disease, as opposed to practical value. Marsden (1986) and Hallett rigidity as an independent movement disorder. (1993) provide a more global definition in refer- ring to akinesia as an inability to move and Clinical presentation of Parkinson's bradykinesia as slowness of movement. In disease Parkinson's disease, rigidity is considered to be a positive symptom resulting from release of other Features which are associated with rigidity in brain structures that are normally inhibited by patients with Parkinson's disease include akine- basal ganglia function, whereas akinesia or sia and bradykinesia. However, neither is caused bradykinesia are the key negative features of loss by restraint of active movement since both con- of normal basal ganglia function (Marsden 1982). ditions can be present when rigidity is absent (Gordon 1990). The delay in initiation of movement in Par- kinson's disease is more evident with internally Akinesia. This term describes the paucity of generated or self-initiated movements than with movement and 'freezing' and has been attributed movements occurring in response to sensory to an impairment in the initiation of movement or stimuli (Lee 1989). Various types of sensory input delay in the reaction time. In patients with Par- may partially compensate for akinesia. Brooks kinson's disease this is most prominent for move- (1986) suggests the use of visual, auditory and ments which are internally generated rather than vestibular cues, such as bouncing a ball, rocking those which occur in response to sensory stimuli the patient's shoulders back and forth or tilting (Miller & DeLong 1988, Lee 1989). Other features them forwards to facilitate movement. It is well of akinesia in relation to Parkinson's disease are: recognised that patients with Parkinson's disease may 'freeze' at a doorway and yet have no prob- • lack of spontaneous movements lem passing through if, for example, a stick is • lack of associated movements, such as arm placed on the floor for them to step over. They may be unable to reach forwards with their arm swinging during walking on command but may be able to pick up a glass. • abnormally small amplitude movements, Parkinson's disease is characterised by an such as very small steps during walking or inability to perform two separate motor actions micrographia at the same time. For example, the patient may be • a tendency for repetitive movements to show a able to stand up and shake hands as two individ- decrease in amplitude and eventually fade out ual motor acts but he is unable to perform these • difficulty performing two simultaneous move- two actions simultaneously (Marsden 1982, ments (Lee 1989). Rothwell 1994). Bradykinesia. Bradykinesia is a term which Aims of physiotherapy should be used exclusively to refer to slowness in the execution of the movement (Lee 1989). This Normal movement is dependent upon an intact has been associated with both reduced mag- CNS providing for an adaptable and integrated nitude of agonist muscle activity (Hallett & level of tonus. Patients with Parkinson's disease Khoshbin 1980) and excessive co-contraction of become more flexed as the condition deteriorates the antagonist during movement (Hayashi et al (Gordon 1990). Rotation is dependent upon 1988), with the inability to 'turn off the antag- balanced activity between flexor and extensor onist being more impaired than the ability muscle groups and, as previously described in to 'turn on' the agonist (Corcos et al 1996). Chapter 3, a dominance of either muscle group Bradykinesia appears as a disorder in voluntary will impair rotation. The lack of rotation with movement in daily life and is considered to be reduced or loss of arm swing is a notable feature partly responsible for disorders in posture and locomotion (Yanagisawa et al 1989).
ABNORMAL TONE AND MOVEMENT 107 of Parkinson's disease (Marsden 1984, Lee 1989, ment disorder will identify the lack of extension Pentland 1993). as a significant factor. Although the increased tone is equally distributed on passive manipula- In the past, the emphasis of physiotherapy tion of the joints, the evidence of increasing treatment was to improve rotation. To that effect, flexion is evident (Fig. 5.5). walking sticks were placed in the patient's hands with which the therapist would initiate arm Turnbull (1992) proposed a 'progressive' swing. In normal subjects, arm swing, a rela- model of management for patients with Par- tively passive action, occurs as a result of inter- kinson's disease which includes early and action between the pelvis and shoulder girdles ongoing intervention by physiotherapy. This on the basis of an extended, upright posture. The model was designed to prevent secondary com- ageing process clearly supports this analysis, in plications and maintain function, instead of that, as the elderly adopt a more flexed posture, waiting until the disability is advanced before the arm swing is reduced (Elble et al 1991). initiating a therapy programme. Patients with Parkinson's disease may become Assessment is essential to identify treatment more flexed both through the CNS pathology priorities and to monitor progress with early and the ageing process. Attempts to superimpose implementation of an exercise programme and arm swing as described above, with this pre- targeted intervention, focusing on areas of dominantly flexed posture, will inevitably be deterioration (Jones & Godwin-Austen 1998). doomed to failure. Analysis of the primary move- Awareness on the part of the patient of the expected progression of the disease in terms of the impairment of movement and an appropriate home exercise programme to reduce the insidi- ous onset of flexion and maintain extension may go some way to maintaining function. The administration of dopamine agonists often has a dramatic effect for patients with Parkin- son's disease and in many cases determines the functional options (Marsden 1986). In severe cases, physiotherapy may only be possible after administration of these drugs. In providing the experience of more normal movement and the maintenance of an upright posture, physiother- apy as an adjunct to medication is appropriate and beneficial in the management of this patient group. Equally, advice and management of spe- cific problems which arise in the more disabled population, such as the inability to turn over in bed, will be of tremendous benefit to patients and their carers. They will direct therapy in that they identify a problem, and then the therapist determines the causative factors and instigates appropriate treatment or coping strategies to enable a greater degree of function. Figure 5.5 Parkinson's posture. DYSTONIA Dystonia is a syndrome dominated by muscle contraction frequently causing twisting and
108 NEUROLOGICAL PHYSIOTHERAPY repetitive movements, or abnormal posture that abnormalities. Primary dystonia shows may be sustained or intermittent (Fahn et al no consistent pathological change and 1987). appears to be neurochemical in origin, rather than a neurodegenerative disorder Classification and prevalence with neuronal loss. The current classification of dystonia describes (b) Dystonia-plus is also a neurochemical dis- each patient with dystonia in three separate cate- order that includes neurological features gories: age at onset, distribution and aetiology such as parkinsonism and myoclonus. The (Fahn et al 1987). The distribution of dystonia dystonic-parkinsonian syndrome is also may be either focal, affecting a single body part; referred to as dopa-responsive dystonia. segmental, affecting adjacent body parts or a seg- ment of the body; hemidystonia, involving one (c) Secondary dystonia is a disorder that side of the body; or generalised dystonia, affect- develops mainly as a result of an insult to ing two or more body segments (Berardelli et al the brain. This includes dystonias associ- 1998). Dystonia can affect virtually any area of ated with cerebral palsy, cerebral hypoxia the body and there are familiar names for dysto- and following stroke. nia at different sites (Table 5.1). (d) Heredodegenerative dystonia occurs The aetiological classification of dystonia has when there is underlying brain degenera- recently been subdivided in the light of gen- tion: for example, in Wilson's disease and etic, pathological and biochemical advances multiple system atrophy (Fahn et al 1998). (Fahn et al 1998): Dystonia is estimated to be more common than (a) Primary dystonia is the most common other well known neurological diseases such as form of dystonia and has been found to be Huntington's disease, motor neurone disease associated with several different gene and myasthenia gravis (Marsden & Quinn 1990), with a prevalence of 108 per million, of which 40 per million have cervical dystonia (Warner 1999). These figures are based on those patients with primary dystonia who have been diagnosed by a neurologist. However, many general prac- titioners are unfamiliar with the signs and symp- toms and referral to a neurologist may be delayed or not occur (Dystonia Society 1999). Pathophysiology Dystonia is the least well understood, in terms of the underlying pathophysiology of all move- ment disorders associated with basal ganglia dysfunction (Crossman & Brotchie 1998). Evid- ence suggests that primary dystonia results from a functional disturbance of the basal ganglia which causes altered thalamic control of cortical motor planning and executive areas and abnor- mal regulation of brain stem and spinal cord inhibitory interneuronal mechanisms (Berardelli et al 1998). Reciprocal inhibition, for example, is reduced in patients with both generalised and focal dystonia (Rothwell et al 1983, Panizza et al
ABNORMAL TONE AND MOVEMENT 109 1990) and this may be abnormal even in asymp- Although there have been no studies reported tomatic limbs, in people with blepharospasm on change in the biomechanical properties of (Hallett 1998). muscle, Hallett (1993) suggests that such changes are as likely to occur in dystonia as in any other The involuntary movements are often exacer- long-standing disease where there have been bated during voluntary movements and are fixed postures. characterised by an abnormal pattern of EMG activity with co-contraction of agonists and The spasms may contort the body into antagonists and overflow into inappropriate grotesque postures which may severely limit muscles (Berardelli et al 1998). activities of daily living with considerable socio- economic implications (Crossman & Brotchie Hallett (1995) has proposed that dystonia could 1998). While this is more apparent in those with be primarily a sensory disorder in that sensory generalised dystonia, problems also arise for symptoms or trauma may precede the onset of those with focal dystonia. For example, a person dystonia. Voluntary repetitive movements, to re- with cervical dystonia may be unable to drink lieve the sensory symptom, have been reported by from a glass and one with blepharospasm may be patients to have become uncontrolled (Ghika et al unable to drive. 1993) and it has been suggested that repeated use of a body part may be a precedent for dystonia of Dystonia exhibits a variability under different that body part (Byl et al 1996, Hallett 1998). Inter- conditions such as stress and fatigue and in the estingly, it has been reported that there is abnormal performance of certain motor acts such as perception of movement, but not of position, in walking (Lorentz et al 1991). In many instances, dystonic subjects (Grunewald et al 1997). people develop their own strategies to control their movements: those with cervical dystonia It is only recently that dystonia has become a may touch the side of the chin (the 'geste antag- recognised, organic condition as opposed to a hys- oniste'); people with blepharospasm may apply terical disorder (Hallett 1995). However, Rondot et pressure on the eyelids; and a toothpick in the al (1991) found that no fewer than 61% of 220 mouth may relieve tongue dystonia. patients suggested different events which they held responsible for the onset of cervical dystonia Treatment (spasmodic torticollis), and the authors concluded that 'in the complex of factors leading to spas- Symptomatic treatment, particularly of the focal modic torticollis, psychological aspects do indeed dystonias, has been revolutionised with the play a role'. advent of botulinum toxin (see Ch. 7). Its use has proved so successful that, as recommended by Clinical presentation Marsden & Quinn (1990), the majority of re- gional neuroscience centres in the United Dystonia can be present at rest but, in general, is Kingdom now provide this treatment. Botulinum more likely to appear during voluntary activity toxin is administered by injection to the pri- (Hallett 1993). These dystonic spasms are charac- marily affected muscles. This is now the treat- terised by co-contraction of agonist and antag- ment of choice for most focal dystonias, onist muscles rather than the more usual particularly blepharospasm (Britton 1998) and reciprocal pattern seen in normal voluntary cervical dystonia (Anderson et al 1992). The movement (Hallett 1993, Rothwell 1994). There is injections are usually repeated, on average, at a loss of selectivity when attempting to perform 3- to 4-monthly intervals. The use of botulinum discrete independent movements which results in toxin is thought to be less effective in the treat- an overflow of activity to remote muscle groups ment of cervical dystonia than in blepharo- that are not normally activated in the movement spasm, possibly because there are more and often and the time taken to switch between com- deeper-sited muscles affected (Berardelli et al ponents of a complex movement is prolonged 1993). (Berardelli et al 1998).
110 NEUROLOGICAL PHYSIOTHERAPY Surgical treatment has been advocated in the keys. It has been hypothesised that this cortical past, such as facial nerve sectioning or myo- plasticity/learning-based degradation of sensory mectomy for patients with blepharospasm and feedback information from the hand contributes various procedures for cervical dystonia. These to the genesis of occupationally derived repeti- include selective peripheral denervation and tive strain injuries and focal dystonia of the hand stereotactic thalamotomy (Bertrand et al 1987, (Byl et al 1996). In a single case study of a patient Bertrand 1993). Surgery may still be indicated for with a focal hand dystonia that developed after some patients who do not respond to botulinum excessive use of a computer keyboard, Byl & toxin. Topp (1998) reported improvement in cortical sensory processing, motor control, physical per- There is little documented on physiotherapy formance and independent function, including for patients with dystonia. Marsden & Quinn work status. This followed an intensive pro- (1990) consider that physiotherapy is of great gramme lasting 1 year, which included sensory benefit in preventing contractures but cannot discriminative therapy and keyboard retraining. teach the brain to deliver normal motor pro- Although the amount of repetition necessary grammes. Braces and other forms of restraint are for sensory retraining required a high level of thought to be ineffective (Marsden & Quinn patient compliance, restoration of sensory func- 1990). tion was associated with improved motor control. Electromyography has revealed complete inhi- bition of antagonist muscles in cervical dystonia In addition to the sensory retraining of patients (Bertrand 1993), and retraining of these muscles with occupational focal dystonias, this may also is recommended (Dykstra et al 1993). On the prove effective in the management of the more basis of this evidence, providing patients with an generalised dystonias. The term 'torsion dysto- individual exercise programme following injec- nia' is frequently used with these patients. This tion of botulinum toxin might enable more effect- aptly describes the effect of the dystonic move- ive activity of the antagonist muscles and thereby ments pulling the patient into distorted asym- improve postural control of the head and neck metrical postures which may lead to further for a longer period of time between injections. deformity and degradation of cortical sensory Indeed, specific exercises for people with cervical information. Advice on posture and seating for dystonia have been developed and are reported people with generalised dystonia is of para- to be effective in improving postural control of mount importance. Patients requiring wheel- the head and neck (Bleton 1994). However, while chairs must have a detailed assessment of their the beneficial effect of specific exercises seems particular needs with appropriate adaptations as plausible theoretically, few carefully planned indicated (see Ch. 9). studies have been undertaken and little objective evidence exists to support this. Similarly, other As with all people with disability, it is import- approaches such as increasing proprioceptive ant to recognise that the individuals with the dis- input by compression or stretch and using ability are often more knowledgeable about the 'mobile weight bearing through the hands and management of their condition than the majority feet' to allow the patient to feel postural adapta- of health care workers involved in their manage- tion, as recommended by Thornton & Kilbride ment. This is particularly so for people with (1998), require further evaluation. dystonia. Basic science and clinical research can and CHOREA AND ATHETOSIS should be used to validate the efficacy of physio- therapy (Byl & Topp 1998). Repetitive, highly Chorea and athetosis are common symptoms of stereotyped movements applied in a learning basal ganglia disease or damage and describe context have been shown to actively degrade involuntary, writhing movements of the body. cortical representations of sensory information The term chorea is usually applied to movements guiding fine motor hand movements in mon-
ABNORMAL TONE AND MOVEMENT 111 at proximal or axial joints, while athetosis is used Clinical presentation more frequently to describe movements of the distal parts of the limbs (Rothwell 1994). Mink The general features of athetosis are those of (1996) defines the sudden, random twitch-like fluctuating abnormal tone with poor grading of movements that involve any body part as posture and movement associated with involun- chorea and, if they are accompanied by slower tary movements that are accentuated by voli- writhing voluntary movements, as choreo- tional activity and stimulation. The resting tonus athetosis. is generally low but attempts at volitional activ- ity reinforce the involuntary movement due to All muscle groups tend to be affected, includ- insufficient and unstable postural tone. In the ing those of the eyes and tongue, with the move- majority of patients, the whole body is involved, ments moving randomly from one part of the with the upper part more affected than the legs. body to another. Writhing of the head and limbs, grimacing and twitching often occur unpre- Course notes from the Bobath Centre (1997) dictably. Speech is invariably affected. Hallett describe three different types of involuntary (1993) comments that the most appropriate movements: adjective to describe chorea is 'random'. Random muscles throughout the body are affected at • Mobile spasms. These are alternating writhing random times and make movements of random limb movements and are often rhythmical in duration. Chorea occurs at rest, on posture and nature. on movement whether it be fast or slow (Marsden 1984). • Fleeting irregular, localised contractions. These affect muscle groups throughout the body It is difficult to distinguish between chorea and and, if severe, may produce exaggerated pos- athetosis as they display similar characteristics. tures and movements such as facial grimacing Athetosis is the term most frequently used to and bizarre distal movements (Fig 5.6). More describe this dyskinesia in children with cerebral localised or weaker contractions may be palsy whereas chorea is used to describe the observed as minor twitches. movement disorder in, for example, Huntington's disease. In most cases there is involvement of • Intermittent tonic spasms, so-called 'dystonic'. both proximal and distal musculature and for the These are predictable in pattern but not in purpose of discussion regarding treatment the timing and are often dependent on changes in two will be considered as one. head position. The individual may be tem- porarily fixed in extreme patterns which may The motor plan involves the selection, sequenc- involve the whole body, proximal body parts ing and delivery of the correct collection of motor or only a limb. These extreme postures are programmes required to achieve the desired more commonly seen in children but their motor behaviour. In chorea, the correct attempt influence may persist throughout life. to move is made by the patient but its success is disrupted by the abnormal co-contraction of syn- Problems associated with ergists, antagonists and postural fixators, so the communication and eating details of the motor programme are abnormally specified (Marsden 1984). The initiation and exe- People with athetosis often have the additional cution of movement are disturbed but chorea problem of impaired communication. In some tends to increase on attempted movement and cases, the involuntary movements affecting the this movement-related increase in chorea occurs face may preclude intelligible speech. The auto- in both the moving and non-moving body parts biography, My Left Foot (Brown 1989), gives great (Mink 1996). However, in spite of this, function insight into the frustration arising from this may be achieved, often in a somewhat contorted inability to communicate. Computers are now manner, in spite of the severity of the dyskinesia widely used to provide a means of both com- (Marsden 1984). munication and education. Although communi- cation may be considered primarily the remit
112 NEUROLOGICAL PHYSIOTHERAPY Figure 5.6 Athetoid hands. of the speech and language therapist, control the mouth. Very few people with athetosis are of posture and respiration are essential pre- overweight, which may be a combination of the requisites and will also be addressed by the eating difficulties and the constant involuntary physiotherapist in collaboration with the speech movement throughout the body. Advice and treat- and language therapist. ment to address this problem should include: Similarly, eating is a problem both in terms • Desensitisation of the mouth including the of the uncontrolled orofacial movements and lips, tongue, gums and, where present, the bite the mechanical difficulty of taking the food to
ABNORMAL TONE AND MOVEMENT 113 reflex. Instruction should be sought from an bilise the hip in extension by 'hanging' on the experienced speech and language therapist or iliofemoral ligament. physiotherapist. • Supported sitting, with the arms resting for- The swing-through phase differs depending wards supported on a table. This will reduce on the fluctuations and degree of increased tone. the tendency to push back into extension as For example: the food approaches the mouth. • Maintenance of elongation of the cervical • Those people with greater fluctuations in tone spine to facilitate movement of the head tend to initiate a step using a total pattern of towards the food. flexion to break up the prevailing extensor tone required to maintain them upright against Communication and eating difficulties are two gravity. In this instance the impression is one of of the most distressing aspects of athetosis and, utilisation of alternate flexor and extensor for this reason, treatment techniques or strategies synergies, affecting the whole body. which prove to be of benefit must be constantly reinforced by the patient and/or his assistant. • People with predominantly low tone, using the iliofemoral ligament to provide stability, Problems arising with posture and demonstrate increased extensor activity in the gait head and trunk in their attempt to step forwards. Inevitably there are many patients with athetosis who will become, or remain, wheelchair depend- Similarly the stance phase differs: ent. Determining the appropriate chair and adaptations is complex and requires detailed • People with marked fluctuations in tone assessment and continual reappraisal. demonstrate a more rigidly extended leg in the extensor synergy, often with flexion of the hip Those born with athetosis initially present due to the overall increase in flexion as the with very low tone and may be referred to as opposite leg steps through. 'floppy children'. Depending on the severity of symptoms, it may take several years for children • Those with lower tone use the more mechan- to develop sufficient tone to maintain themselves ical stability of extension of the hip and hyper- upright against gravity. The constant fluctuations extension of the knee counteracted by of tone throughout the body severely hamper increased extension of the trunk. grading of all movement. Facilitation and con- stant repetition of a more controlled movement is Pure athetosis is rare, particularly in the cere- essential for the child to develop adequate bral palsy population. Those with predominant stability. Positioning to improve symmetry and hypotonia may also have what are described as alignment and use of bilateral grasp is also dystonic spasms, whereas others with higher recommended (Bobath Centre 1997). tone may also demonstrate symptoms associated with the positive features of an upper motor People with athetosis who are ambulant often neurone lesion (Fig. 5.7). demonstrate a somewhat bizarre gait pattern characterised by marked retraction of the head, While people with pure athetosis are unlikely trunk and hips. On clinical observation it would to develop contractures due to the prevailing low seem that the shoulder girdles are retracted with tone and constant movements, those with asym- the arms in either flexion or extension depending metrical dystonic spasms may develop severe on the stability afforded at the pelvis. This stabil- deformity, even joint displacement, depending ity may be provided by adequate tone in the less on the severity of these spasms. Dislocation of severely affected legs or by mechanical factors, the hip in children with cerebral palsy is a not as in the patient with lower tone, who may sta- infrequent complication with repeated spasms of the leg into adduction and medial rotation. Those with a combination of athetosis and hypertonus are more susceptible to the development of more general contractures because there is less variety
114 NEUROLOGICAL PHYSIOTHERAPY mine appropriate treatment. For example, many people with athetosis defy the laws of balance in their means of ambulation, and physiotherapists must recognise that this is successful and func- tional for them. Attempts to improve their gait pattern, unless this is established to be the cause of the complication, will generally be met with a distinct lack of enthusiasm. Figure 5.7 Athetoid standing position. SUMMARY of movement and the basic tone is higher and in Physical disability is often characterised by more stereotyped patterns. abnormal tone and movement which contribute to the functional deficit. Some of the different Management types of movement disorders have been described, with emphasis on the clinical presentation and People with long-standing choreoathetosis examples of physiotherapy intervention which develop strategies, often unique to each individ- may prove of benefit. ual, to compensate for their unstable tone and involuntary movement. These strategies are Although the neurological impairment in essential for them to function. In most instances many instances is non-progressive, the disability physiotherapy intervention is required for com- arising from the pathology may increase over plications arising from the disability, such as pain time. This deterioration may occur as a result or contracture, as opposed to treatment of the of enforced immobility or dominance of stereo- movement disorder itself. Invariably it is the typed movement patterns. Any individual who abnormal movement which causes the problem is restricted to a limited or inappropriate reper- for which the person seeks treatment, but mutu- toire of movement is in danger of developing ally agreed goals must be established to deter- structural changes of affected muscle groups, which may further decrease functional capability. Physiotherapists should have an understand- ing of the pathophysiology of the different types of movement disorder to formulate effective treatment interventions. Many patients, par- ticularly those with severe symptoms, develop compensatory strategies which are appropriate to their functional needs. The physiotherapist must recognise that restoration of 'normal move- ment' is often an inappropriate and unattainable goal and work with the patient to ensure the optimal functional outcome. In the majority of cases, there should be a balance between re- education of more normal movement patterns and acceptance and promotion of necessary and desirable compensation to attain optimal func- tion. While in many instances it is necessary to work for component parts of movement patterns, the patient must appreciate that the outcome of this improved activity will have functional sig- nificance, otherwise motivation and cooperation
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CHAPTER CONTENTS Treatment and management of respiratory General principles of dysfunction 121 treatment Traumatic brain injury 121 Philippa Carter Intracranial dynamics 122 Susan Edwards Factors affecting the cerebral vasculature 123 The effect of brain injury on the respiratory The purpose of this chapter is to describe prin- system 124 ciples of treatment which apply to all stages of Medical management of intracranial management of patients with neurological dis- hypertension 124 ability. This includes acute and chronic disorders. Physiotherapy intervention 125 The emphasis is on early physiotherapy inter- vention when patients are usually hospitalised Respiratory muscle impairment 127 and includes management of respiratory and Physiotherapy intervention 127 neurological dysfunction. Conclusion 130 Some sections of this chapter are well refer- Management of neurological dysfunction 130 enced whereas in others there is no literature to support current practice. The description of Basic principles 130 physiotherapy management is based on the authors' clinical experience and should not be Positioning 132 considered as definitive. It is hoped that many Principles of positioning in bed 133 research questions will be raised to either sub- Principles of positioning in sitting 136 stantiate or disprove this treatment approach. Principles of positioning in standing 137 TREATMENT AND MANAGEMENT OF Movements 144 146 RESPIRATORY DYSFUNCTION Orofacial movements 144 Shoulder girdle and upper limb This section concentrates on how the neuro- The elbow joint 148 logical status of a patient may affect the res- The wrist and fingers 148 piratory management rather than examining The lower limb 149 the more detailed aspects of respiratory care. The The foot and ankle 149 management of patients with traumatic brain injury (TBI) and patients with respiratory muscle Summary 150 impairment following spinal cord injury (SCI) and neuromuscular disease is discussed. References 150 TRAUMATIC BRAIN INJURY Brain injury can be divided into primary and secondary brain damage. Primary damage is 121
122 NEUROLOGICAL PHYSIOTHERAPY caused at the time of insult and is irreversible. responding increase in ICP (Arbour 1998). With Secondary damage occurs later and is caused by an expanding intracranial lesion, compensatory several factors, including raised intracranial mechanisms include: pressure (ICP) and decreased cerebral perfusion pressure (CPP) which may follow hypotension, • displacement of CSF from the cranial to the haemorrhage, hypoxia and hypercapnia. Recov- spinal subarachnoid space ery from any type of brain injury depends on the extent of the initial injury and the secondary • decreased CSF production damage (Reilly & Lewis 1997, Arbour 1998). • decreased CBV. Medical intervention is directed at maintaining When compliance is normal, increases in vol- adequate cerebral blood flow (CBF) and mini- ume of brain tissue, blood or CSF do not ini- mising secondary damage which can have tially produce increases in ICP (Fig. 6.1 A-B). devastating effects on the overall outcome When compliance is poor, a small increase (Snyder 1983, March et al 1990). In order to plan in volume may cause a dramatic increase in appropriate physiotherapy intervention one ICP (Fig. 6.1 B-C) demonstrated by the intra- must understand intracranial dynamics and the cranial pressure volume curve (Lindsay & Bone physiology of intracranial hypertension. 1997). Intracranial dynamics Cerebral perfusion pressure The skull forms a rigid box containing brain Cerebral perfusion pressure is the pressure at tissue (80% of the total volume), cerebrospinal which the brain tissue is perfused with blood and fluid (CSF) (10%) and blood, which is the final is a measure of the adequacy of the cerebral 10% (Andrus 1991). These components are in circulation (March et al 1990). It is the difference dynamic equilibrium: if the volume of one between the mean arterial pressure (MAP) component increases, there must be a relative and the ICP. The normal range for CPP is decrease in other components to keep the overall 70-100 mmHg. A CPP less than 50-60 mmHg volume constant (Odell 1996, Arbour 1998). may adversely affect prognosis (Leurssen & Marshall 1990, Rosner et al 1995). Intracranial pressure At present, ICP and CPP monitoring remain Intracranial pressure (ICP) is defined as the pres- the most commonly used clinical parameters for sure exerted within the skull and meninges by assessing intracranial dynamics. By continuous the brain tissue, CSF and cerebral blood volume observation and regulation of the ICP and the (CBV) (Andrus 1991) and can be measured by a MAP, the CPP can be maintained (Brucia & Rudy variety of monitoring devices (German 1998, 1996, Imle et al 1997). Chitnavis & Polkey 1988). Cerebral blood flow The normal range of values for ICP is between 0 and 15 mmHg (Garradd & Bullock 1986, Chudley Cerebral oxygen delivery is the product of cere- 1994). Intracranial hypertension occurs when the bral blood flow (CBF) and arterial oxygen ICP exceeds 15 mmHg, and treatment is normally content. The CBF is closely coupled to the energy required above 20 mmHg. A persistently high ICP requirements of brain tissue; if the cerebral meta- may be associated with a poor prognosis and bolic rate (CMR) is high then the oxygen con- uncontrollable ICP is often fatal (Tobin 1989). sumption is high and oxygen demand is great (Kerr et al 1995). Intracranial compliance The average CBF is 50-55 ml/100 g/min and is Intracranial compliance is the ability of the brain calculated as to tolerate increases in volume without a cor-
GENERAL PRINCIPLES OF TREATMENT 123 Figure 6.1 Intracranial pressure-volume curve. where CPP = MAP - ICP and CVR is the cerebral drop in SABP the cerebral vessels vasodilate to vascular resistance (the pressure across the cere- maintain CBF and vasoconstrict if there is a rise brovascular circulation from the arteries to the in SABP. Above a MAP of 130 mmHg, the vessels jugular veins). It may be altered by oedema, are unable to compensate and an increase in ICP vasospasm and pre-existing hypertension occurs. If the MAP falls to below 60 mmHg, CBF (Johnson et al 1989). may decrease, causing cerebral ischeamia (Arbour 1998). In many neuroscience units new methods such as jugular bulb oximetry, transcranial Doppler Chemoregulation ultrasonography and radioactive zenon are used to measure CBF, brain tissue metabolism and its The cerebral vessels are very sensitive to arterial need for oxygen (Kerr et al 1995, Reilly & Lewis partial pressures of carbon dioxide (PaC02) and 1997, Berre et al 1998, Cruz 1998). oxygen (PaO2), being more sensitive to changes in P a C 0 2 than P a 0 2 . An increase in PaCO2 or or Factors affecting the cerebral reduction in P a 0 2 causes reflex cerebral vaso- vasculature dilation, which increases the CBV and ICP. If the SABP remains unchanged, a reduction in the Autoregulation CPP and CBF will occur (Rudy et al 1986). Autoregulation is the ability of the brain to main- Intracranial hypertension tain a constant CBF, by regulating the CVR despite wide variations in systemic arterial blood A variety of injuries and conditions may cause pressure (SABP) and metabolic demands. changes in intracranial dynamics (Arbour 1998). Autoregulation is effective between a MAP of 60 and 130 mmHg. Within these limits if there is a
124 NEUROLOGICAL PHYSIOTHERAPY Altered patterns of cerebral blood flow occur Medical management of intracranial when autoregulation is lost and intracranial com- hypertension pliance diminished. All these factors put the patient at risk of intracranial hypertension and Patients with severe neurological injuries who cerebral ischaemia which is associated with a are at risk of hypoxia require early aggressive poor neurological outcome (Dexter 1997, Kerr airway management to prevent secondary com- et al 1997). plications by maintaining CBF (Brucia & Rudy 1996, Dexter 1997). Sedation and occasionally The effect of brain injury on the paralysing agents are used in the first 48-72 h respiratory system and then withdrawn to assess the patient's state of consciousness (Garradd & Bullock 1986). If the Traumatic brain injury may impair lung function patient becomes agitated or has severely elevated both directly and indirectly, at the time of the ICP, it may be necessary to resedate and paralyse original trauma or later. The resulting respiratory the patient. insufficiency leads to hypoxaemia and hyper- capnia, both of which may compromise cerebral A venticular drain may be used to measure ICP oxygenation (Warren 1983). Pneumonia ranks and drain CSF as a means of reducing ICP. The second to intracranial hypertension as the drain is clamped when the patient is moved leading cause of death in patients with TBI (Imle during physiotherapy and undamped and et al 1997). realigned afterwards. Direct damage to the chest wall, respiratory Hyperventilation muscles, pleura or lungs may produce ineffective respiratory mechanics and hypoventilation Hyperventilation has been the cornerstone in (Prasad & Tasker 1990, Parataz & Burns 1993, treating patients with intracranial hypertension, Arbour 1998). but its use has become increasingly controversial (Dexter 1997, Arbour 1998). Hyperventilation Indirect respiratory insufficiency may be due decreases the P a C 0 2 to between 4 and 4.5 kPa, to: producing cerebral vasoconstriction and a de- crease in CBF and ICP. The effect is short lived. • Depressed level of consciousness, resulting in What is not clear is whether the resulting de- an inability to maintain the airway and crease in CBF places patients with TBI at risk of hypoxia (Warren 1983). regional cerebral ischaemia (Dexter 1997). Ex- treme hyperventilation, where the P a C 0 2 falls • Compromise of the respiratory centre in the below 3 kPa, may cause profound cerebral vaso- brainstem, leading to altered respiratory pat- constriction, resulting in cerebral ischaemia terns, hypoventilation and increased PaC02. (Kerr & Brucia 1993, Dexter 1997). Concurrent suppression of the cough and gag reflex will result in poor airway protection and Positive end-expiratory pressure increase the risk of aspiration (Warren 1983, Prasad & Tasker 1990). Positive end-expiratory pressure (PEEP) is often used in ventilated patients to maintain the • Neurogenic pulmonary oedema, the exact patency of airways at the end of expiration and cause of which is unknown but which in most improve oxygenation. However, it must be used cases develops within 24 hours after the initial with caution, as PEEP in excess of 7.5 c m H 2 0 injury. The symptoms are similar to those seen may decrease intracranial compliance by increas- in acute respiratory distress syndrome (ARDS) ing intrathoracic pressure (ITP), CBV and ICP (Warren 1983). (Feldman et al 1997). • Immobility or tonal changes resulting in reduced tidal volumes, small airway closure and atelectasis (Warren 1983, Arbour 1998).
GENERAL PRINCIPLES OF TREATMENT 125 Drug therapies While coughing assists with removal of secre- tions, increased intrathoracic pressure reduces Propofol is often used for sedation, decreasing cerebral venous return and increases CBV and cerebral metabolic rate, CBF and ICP. However, it ICP (Wainwright & Gould 1996, Kerr et al 1998). will reduce the SABP, which may compromise the CPP. Paralysing agents immobilise the pa- Findings from several studies show that tient and reduce the systemic consumption of increased ICP during suctioning occurs due to oxygen. Inotropic support may be necessary to direct tracheal stimulation (Brucia & Rudy 1996, maintain an adequate MAP and CPP. Bolus doses Fisher et al 1982). Transient increases in ICP of mannitol, an osmotic diuretic, are used to result from direct carina stimulation but a sus- reduce ICP by establishing an osmotic gradient tained rise in ICP is due to the increase in ITP between the plasma and brain tissue, thereby associated with coughing. Patients with reduced reducing cerebral oedema. High-dose barbitu- intracranial compliance have the most pro- rates, for example thiopentone, reduce the cere- nounced elevations in ICP on suctioning and are bral metabolic rate of oxygen and stabilise or less able to tolerate these elevations. Care must reduce the cerebral blood flow (Lindsay & Bone be taken when suctioning patients with an ICP 1997, Arbour 1998). above 20 mmHg (Kerr et al 1998). Physiotherapy intervention Considerations when suctioning The physiotherapist must understand intra- • Hyperoxygenation, for example, 100% Oz for cranial dynamics and the effect of physiotherapy 2 minutes prior to suctioning, minimises tran- in order to assess accurately and treat effectively. sient periods of hypoxia and hypercapnia The following factors should be considered: (Rudy et al 1986, Chudley 1994, Kerr et al 1997). • conscious level, as measured on the Glasgow • Crosby & Parsons (1992) recommend at least Coma Scale (GCS) 60 seconds of manual hyperventilation between each suction with 2 minutes undisturbed rest to • pupil response before and after intervention allow ICP, MAP and CPP to return to baseline. • vital signs including ICP, SABP and CPP and • Disconnection from the ventilator during how these are influenced by turning, pressure suctioning causes a rapid drop in ITP and relief and oral hygiene procedures. sudden loss of PEEP. A reduction in ICP, due to an increase in cerebral venous return, or an Prior to physiotherapy, sedation or painkillers increase in ICP,_due to abrupt elevations of the may be given. The timing of the drug adminis- SABP, may result. Disconnection may also tration is vital and close liaison with the nursing cause hypoxia, resulting in cerebral vaso- staff is essential. dilation (Kerr et al 1993, Imle et al 1997). Suctioning via a port adaptor on the catheter Suctioning mount, or using a closed suction circuit is therefore recommended. Suction should not Suction is a necessary adjunct to ventilator ther- exceed 10-15 seconds (Kerr et al 1993). apy. It ensures airway patency, removes pul- monary secretions and promotes ventilatory • The catheter should pass beyond the endo- exchange (Brucia & Rudy 1996, Kerr et al 1997). tracheal tube (ETT) but not contact the carina Suction causes a progressive increase in ICP with to minimise irritant receptor stimulation. The each insertion of the suction catheter. In most ETT must be stabilised to avoid any unnecess- patients, elevations in ICP are transient and ary tracheal stimulation (Kerr et al 1993, Brucia return to baseline level within minutes (Kerr et al & Rudy 1996) 1998). Induced hypercapnia and hypoxia may cause ICP to rise during suction (Chudley 1994). • Suction passes should be limited to a maxi- mum of 2-3 (Garradd & Bullock 1986, Rudy et al 1991, Kerr et al 1993, Arbour 1998).
126 NEUROLOGICAL PHYSIOTHERAPY • Suction pressure should be high enough to constrictive effect on the cerebral vasculature, remove secretions but not reduce functional reducing the CBV and ICP (Rudy et al 1991, Kerr residual capacity or cause tracheal mucosal & Brucia 1993). Hyperinflation should be inter- trauma: 120 mmHg pressure is recommended spersed with short-duration hyperventilation to (Kerr et al 1993). lower P a C 0 2 prior to or following suction (Kerr et al 1997). Application of MH should be brief, as • The external diameter of the catheter should ICP may increase over time (Garradd & Bullock not exceed half the internal diameter of the 1986, Paratz & Burns 1993). ETT to minimise the changes in airway resist- ance and negative pressure (George 1983, Manual techniques Young 1984). Shaking and vibrations. These are intermittent • Intratracheal or intravenous lignocaine may be coarse or fine compressions of the chest wall used to blunt ICP responses to suctioning during expiration, usually combined with MH. (Rudy et al 1986). They augment the expiratory flow and facilitate large and small airway clearance by mobilising • Neuromuscular blocking agents suppress the and advancing secretions (Ciesla 1989, Pryor & cough reflex and induce paralysis of the inter- Webber 1998). Manual techniques with shaking costal muscles and diaphragm, increasing may increase ICP over time; vibrations in isola- chest wall compliance. This may be respons- tion have no effect on ICP (Prasad & Tasker 1990, ible for the attenuation of expected increases in Imle et al 1997). ICP occurring during suction (Kerr et al 1998). Chest clapping. These are slow rhythmical Suctioning usually increases ICP but the MAP movements of the cupped hands over affected may also increase. It therefore follows that what lung segments to mobilise secretions (Pryor & may be a dangerous increase in ICP may be offset Webber 1998). Chest clapping does not adversely by a rise in MAP maintaining an adequate CPP. affect ICP and may even lower it (Garradd & Suctioning should only be performed when Bullock 1986, Paratz & Burns 1993, Imle et al 1997). clinically indicated (Wainwright & Gould 1996, Imle et al 1997). Manual hyperinflation Positioning Manual hyperinflation (MH) provides artificial Patients with high ICP should be nursed in ventilation by means of a rebreathing (Water's) 30 degrees head and trunk elevation as this or non-rebreathing (Ambu) bag attached to an increases cerebral venous return and lowers ICP, oxygen source. The aim is to mobilise and assist without a significant decrease in CBF (Brimioulle the clearance of excess bronchial secretions, to et al 1997). Head elevations above 30 degrees increase lung volumes and re-expand areas of may cause a decrease in SABP, which may com- lung collapse (Pryor & Webber 1998). promise the CPP (March et al 1990, Imle et al 1997, Simmons 1997). The head and trunk should A larger tidal volume is delivered, producing be in alignment, as neck flexion and rotation may an increase in ITP, which leads to a reduction in increase ICP by compressing the internal jugular cerebral venous return and increase in CBV and vein (Williams & Coyne 1993, Chudley 1994). ICP (Snyder 1983, Imle et al 1997). If the SABP is low, the induced increase in ITP reduces systemic Postural drainage is a means of positioning venous return, lowering the cardiac output, that enables gravity to assist the clearance of further lowering the SABP and compromising bronchial secretions. Positions such as a head the CPP (Ersson et al 1990, Prasad & Tasker 1990). down tilt may cause an increase in ICP; however, If SABP is high, MH can increase it to a critical they can be performed safely when strict guide- level (Ada et al 1990). The increased levels of lines are followed and effects of treatment care- PaOz induced by MH may have a slight vaso- fully monitored (Lee 1989, Imle et al 1997).
GENERAL PRINCIPLES OF TREATMENT 127 Turning patients into side-lying may increase physiotherapists understand the relationship ICP, with only small changes in CPP (Mitchell et between brain and lung function, intracranial al 1981, Lee 1989, Rising 1993). If the baseline ICP dynamics and how all aspects of physiotherapy is less than 15 mmHg and the CPP is greater than are integrated with intracranial physiology. 50 mmHg, it is considered safe to turn the patient with TBI (Parsons & Shogan 1984). Hip flexion RESPIRATORY MUSCLE greater than 90 degrees limits venous drainage IMPAIRMENT and will increase ICP (Arbour 1998). Neuromuscular diseases often affect the respira- In summary: tory muscles and may involve bulbar muscles, producing impairment of the gag reflex, laryn- • Patients' positioning is individually deter- geal dysfunction and poor airway protection mined by ICP, SABP and CPP (March et al (Kelly & Luce 1991). Pulmonary complications 1990, Simmons 1997). following cervical SCI are the leading cause of death (Mansel & Norman 1990). Respiratory • Care should be taken with cervical collars and muscle weakness or paralysis causes hyper- ETT ties: if too tight, these may impair cerebral capnic respiratory failure associated with hypo- venous drainage (Arbour 1998). xaemia. This results from hypoventilation, an ineffective cough and an inability to clear • Adequate time should be given to allow ICP to secretions ( Kelly & Luce 1991, Roth et al 1997). return to baseline (Chudley 1984). The cumu- lative effect of increased ICP is a major deter- The lower motor neurone (LMN) (see Ch. 5) mining factor in prognosis (Shalit & Umansky begins at the motoneurone and includes the 1977). peripheral nerve, neuromuscular junction and muscle. Disorders can effect the LMN anywhere Passive movements along its pathway. Motoneurone disease (MND), peripheral nerve disorders, for example Guillain- Passive movements to monitor range of motion Barre syndrome (GBS), neuromuscular junction and assess muscle tone are performed routinely disorders such as myasthenia gravis and dis- on patients in intensive care units. They can be orders of muscle, for example muscular dystro- performed safely on patients with normal or phies, together with SCI, can have devastating elevated levels of ICP (Brimioulle et al 1997). effects on respiratory muscle function. Stereotyped patterns of hypertonus, producing isometric contractions with pressure of the feet Following an acute SCI the patient may be in a against a foot board, are associated with eleva- state of spinal shock, with absent reflexes and tions in ICP (Andrus 1991). flaccid paralysis lasting from days to months. For those with cervical SCI, the resultant tetra- Conclusion plegia may be no different from that of a patient with GBS. Similar principles of assessment and Physiotherapy usually increases ICP, but the treatment therefore apply. positive benefits often outweigh the transient increase. Cerebral perfusion pressure may Physiotherapy intervention remain unchanged due to a corresponding increase in the SABP (Garradd & Bullock 1986, Accurate assessment and regular review is vital Paratz & Burns 1993). The decision whether or to initiate preventative measures and treatment. not to treat must be made in conjunction with the The third to fifth days after SCI are most crucial, nursing and medical staff. as increased secretions may lead to atelectasis and chest infection (Mansel & Norman 1990). The Physiotherapists have a role to play in the pre- following details should be considered when vention of secondary brain damage, which may result from hypoxia or hypercapnia arising from respiratory dysfunction. It is essential that
128 NEUROLOGICAL PHYSIOTHERAPY assessing patients with respiratory muscle The patient must be encouraged to cough impairment: 3-4 times a day, and collaboration with the nursing staff is essential (Ward 1998). • examination of the chest wall and upper abdomen for paradoxical movements indi- Positioning cating weakness of intercostal muscles or diaphragm (Brownlee & Williams 1987) Positional changes affect respiratory function in patients with tetraplegia: for example, vital • effectiveness of the cough (Brownlee & capacity may increase in supine compared with Williams 1987, Ward 1998) upright positions of sitting or standing (Brownlee & Williams 1987). In the supine position, paraly- • high respiratory rate, which may indicate dia- sis of the abdominal muscles causes the abdomi- phragmatic weakness or fatigue (Ward 1998) nal contents to force the diaphragm into a higher resting position, increasing its mechanical advan- • repeated measurements of vital capacity: a tage (Mansel & Norman 1990). In an upright vital capacity of less than 1 litre may indicate position, the abdominal contents fall downwards the need for mechanical ventilation. and forwards, causing the diaphragm to descend lower into the abdominal cavity and thereby Breathing exercises decreasing its effectiveness (Ward 1998). Breathing exercises must be established at an Effects of autonomic dysfunction early stage to encourage maximal inspiration. Patients with SCI may have absent or impaired Autonomic dysfunction is a vascular reflex which sensation below the level of the lesion. Verbal may occur in SCI patients with lesions above T4 or and visual feedback, using an incentive spiro- in neuromuscular disorders. There is involvement meter, supplements tactile feedback (Brownlee & of both the sympathetic and parasympathetic Williams 1987, Ward 1998). systems and symptoms include sweating above the lesion, slow pulse, hypertension, headache Intermittent positive pressure breathing and, in some cases, cardiac arrest (Kelly & Luce 1991). Autonomic dysreflexia may occur in Intermittent positive pressure breathing (IPPB) response to impaired bladder and bowel function increases inspiratory volume and aids clearance or other noxious stimulus and, although antihy- of secretions in patients with sputum retention pertensive drugs may be given as a temporary and lung collapse (Mansel & Norman 1990). Many measure (Bromley 1998), it is essential to identify neuroscience units use IPPB prophylactically in and remove the cause. patients whose vital capacity is between 1 and 1.5 litres. Treatment should be short to avoid Following cervical cord injury, the thora- tiring the patient (Brownlee & Williams 1987). columbar sympathetic outflow is impaired and parasympathetic tone unopposed. Stimulation of Assisted coughing the parasympathetic system, for example during nasopharyngeal suction, may result in brady- Assisted coughing is vital for patients lacking cardia, hypotension and even cardiac arrest. Loss sufficient intercostal and abdominal force to of vasoconstrictor tone resulting in peripheral produce an effective cough. The therapist vasodilation and diminished venous return due replaces the function of weakened or paralysed to paralysis of the abdominal and lower limb abdominal muscles by applying a compressive musculature may lead to postural hypotension force upwards and inwards against the thorax as (Mansel & Norman 1990). In the early stages of the patient attempts to cough (Fig. 6.2). Care acute paralysis this may impact on the patient's must be taken to avoid pressure on the abdomen, ability to sit up in bed. particularly if paralytic ileus is suspected, and stability must be maintained at the fracture site for patients with traumatic SCI (Bromley 1998).
GENERAL PRINCIPLES OF TREATMENT 129 (A) Figure 6.2 Assisted coughing. (B) Abdominal binders may be used to provide the lower rib cage (Brownlee & Williams 1987). support to the abdominal contents, allowing the They improve the vital capacity of patients with diaphragm to assume a more normal resting tetraplegia in sitting but not in the supine position in upright positions, but care must be position (Goldman et al 1986) and are a useful taken that they do not restrict the movement of adjunct in the early stages of mobilisation, par-
130 NEUROLOGICAL PHYSIOTHERAPY ticularly when standing the patient. Surgical MANAGEMENT OF NEUROLOGICAL stockings may also be used to good effect to DYSFUNCTION control postural hypotension. Non-invasive ventilation Shumway-Cook & Wollacott (1995) propose that there are four key concepts that contribute Non-invasive ventilation (NIV) can improve to a comprehensive conceptual framework for gaseous exchange and reduce the work of breath- clinical practice. These are: ing in patients with hypercapnic respiratory failure following respiratory muscle impairment. • the clinical decision-making process, whereby A nasal or full face mask is used, avoiding the essential information is gathered to develop a problems associated with endotracheal intuba- plan of care consistent with the problems and tion and mechanical ventilation (Simonds 1996, needs of the patient Pryor & Webber 1998, Parton et al 1999). • a hypothesis-orientated clinical practice, Mechanical ventilation whereby assumptions about the nature and cause of motor control may be tested Depending on the extent and severity of neuro- logical impairment, patients may require mechan- • a model of disablement which analyses the ical ventilatory support and early tracheostomy effects of disease and enables the clinician to to avoid respiratory failure. As respiratory mus- develop a list of problems towards which cle function improves, an active weaning pro- treatment may be directed gramme should be instigated. This may be more appropriate in the supine position for patients • a theory of motor control from which the with cervical cord lesions, as the vital capacity is assumptions about the cause and nature of increased in this position (Mansel & Norman normal and abnormal movement are derived. 1990). Patients are encouraged to breathe spon- taneously for short periods several times a day This framework enables the therapist to make and given ventilatory support at night to ensure decisions about key elements to assess and treat adequate rest and the avoidance of dia- when retraining patients with a movement phragmatic fatigue (Gardner et al 1985, Ferner disorder. et al 1987). BASIC PRINCIPLES In patients with acute SCI, as spinal shock resolves, chest wall flaccidity may be replaced The control of body posture includes the align- with hypertonus. In this instance, pulmonary ment of body segments to each other and to the function may improve significantly as the more supporting surface. It requires a reference point rigid chest resists collapse (Kelly & Luce 1991). around which movement can occur. The choice of position, both for support and for the per- Conclusion formance of movement, must be considered in respect of changes in tone, the influence of Physiotherapists are closely involved in the gravity, potential structural deformities and assessment, treatment and management of the preservation of tissue viability (Dufosse & respiratory system of patients with SCI and Massion 1992, Pope 1992). neuromuscular disease. A full understanding of the function of muscles involved in respiration is Movements are regularly performed on pa- essential to predict potential problems due to tients who are unconscious, paralysed, or who weakness or paralysis and to plan prophylactic have hypertonus, in order to maintain muscle measures or treatment where necessary. and joint range. An understanding of normal movement as described in Chapter 3 is essential to ensure that movements are performed cor- rectly. Emphasis should be placed on functional
GENERAL PRINCIPLES OF TREATMENT 131 movements, ensuring an appropriate response of thoracic excursion which complements this throughout the whole body, rather than on action. If the spine becomes stiff and immobile purely movements of an isolated part. and is unable to accommodate to the arm move- ment, this movement will be performed ineffect- Passive joint motion refers to any movement of ively and may traumatise the shoulder joint and an articulation that is produced by some external surrounding structures. force (Frank et al 1984). By implication, the term 'passive' indicates that what is done is outside Limitation of range of movement is a common the patient's control and, as such, passive move- complication in patients with musculoskeletal ment must be considered a powerful tool. Pa- and neurological conditions (Williams 1990, Pope tients who are unconscious or who have flaccid 1992). Many authors stress the importance of paralysis have no resistance to any force applied, passive movements to prevent this occurrence whereas those dominated by stereotyped hyper- (Frank et al 1984, Pope 1992, Soryal et al 1992, tonic posturing tend to resist movements out of Daud et al 1993, Davies 1994). However, Davies these patterns. (1994), while advocating the use of passive move- ments, emphasises the need for caution, and In a recent study, Nelles et al (1999) demon- Silver (1969) and Daud et al (1993) identified a strated that passive elbow movements in hemi- relationship between passive movement and plegic stroke patients before clinical recovery heterotopic bone formation in patients with elicit some of the brain activation patterns spinal injury. Patients who are unable to signify described during active movement after sub- pain and discomfort are at particular risk if the stantial motor recovery (Chollet et al 1991, limb is taken to the extreme of range by a ther- Weiller et al 1993). It is suggested that the recruit- apist intent on 'maintaining range of movement'. ment of ipsilateral sensory and motor pathways early after stroke may be critical for return of The use of passive movement remains contro- voluntary control. versial. Vigorous, forceful movements carried out in the presence of severe hypertonus may cause In the clinical setting, the term 'passive' is microtears in muscle which may lead to calcifica- often inappropriate in that, irrespective of their tion of the muscle (Ada et al 1990). Movement of level of consciousness, patients should be involved a joint where there is inadequate muscular control in the activity. Verbal instruction should be given may also lead to overstretching of tissues around as each movement is carried out, to inform the joint (Frank et al 1984). This is in part due to patients of the desired response and what is the lack of reciprocal activity which under normal expected of them, and attention given to the circumstances provides proximal control and degree of proximal mobility and alignment to stability. Soryal et al (1992) described three effect optimal limb movement. It is only in this patients with Guillain-Barre syndrome who had way that the maintenance of muscle and joint 'profound disability and social handicap over 1-3 range becomes a dynamic activity rather than a years after acute neurological presentation pri- mindless, routine procedure performed on the marily as a result of reduced joint mobility rather patient. than neurogenic weakness'. This was considered to be the result of either inadequate passive It is impossible for the physiotherapist to repli- movement or excessive passive movement, in the cate, for a paralysed patient, the full gamut of presence of hypotonia, traumatising the joints functional activity undertaken in everyday life. and surrounding structures. Normal movement is dependent upon the activ- ation of appropriate agonists and antagonists, When muscle is immobilised in a shortened with adjustment of synergists and postural fixa- position there is both a reduction in the muscle tors (Marsden 1984). Appropriate positioning is fibre length due to a loss of serial sarcomeres and of great importance in enabling effective move- a remodelling of the intramuscular connective ment with this concurrent response throughout tissue, leading to increased muscle stiffness the body. For example, elevation of the arm (Goldspink & Williams 1990). Mobilisation of the in normal subjects is influenced by the degree
132 NEUROLOGICAL PHYSIOTHERAPY muscle is therefore essential to minimise these Davies (1994) advocates the use of specific structural changes. Periods of stretch as short as tension tests for the maintenance or restoration of half an hour each day have been found to be adaptive lengthening of the nervous system. effective in preventing loss of sarcomeres and Therapists are advised to study the techniques reducing muscle atrophy in neurologically intact carefully and to practice them on normal subjects mice (Williams 1990). However, Tardieu et al prior to using them in the treatment of patients (1988), in a study of the soleus muscle of children with neurological dysfunction. with cerebral palsy, demonstrated that it was necessary for the muscle to remain in a length- POSITIONING ened position for 6 hours to prevent contracture. Different postures and positions, and their influ- Passive movements of the limbs may be per- ence on tone and movement, are discussed in formed on patients once or twice a day. This Chapter 3. These should be considered in deter- is particularly so for patients in an intensive mining the most appropriate position for treat- care setting where passive movements are often ment of patients with differing types of abnormal carried out as a routine procedure. The time tone. However, the optimal position is not taken in carrying out these movements varies, always possible due to the patient's medical but due to other constraints, rarely exceeds condition or the presence of contractures, and 1 hour a day in total. If, for the rest of the time, modification is necessary. the patient is dominated by stereotyped patterns which maintain the muscles in a shortened posi- Throughout this section 'acceptance of the base tion, this intervention, on its own, is unlikely to of support' refers to the ability of the patient be effective in the maintenance of muscle and to adjust appropriately to the contours of the joint range. Other strategies must be developed supporting surface. to produce a more lasting effect in the control of body posture and movement. Positioning in a variety of postures is recom- mended particularly for patients dominated by Early mobilisation of the patient should be encouraged as and when the medical condition Figure 6.3 Windswept hips. allows. Movements of the limbs while the patient is confined to bed are difficult in terms of facili- tating activity within the trunk. Where bed rest is unavoidable, it is important to maintain ade- quate movement of the trunk to maintain mobil- ity and to stimulate postural adjustments when moving the limbs. Of equal importance is the mobility of the nervous system itself and how it adapts to body movement (Maitland 1986, Butler 1991, Hall et al 1993, Shacklock 1995). Mobilisation of the ner- vous system is used extensively in the assess- ment and treatment of pain syndromes but it is only quite recently that its importance has been recognised in the treatment of patients with neurological disability (Davies 1994, Panturin & Stokes 1998). Patients who are unable to move or who are dominated by hypertonic stereotyped postures are in as great a danger of developing shortening of neural structures as they are of loss of range of the musculoskeletal system.
GENERAL PRINCIPLES OF TREATMENT 133 severe hypertonus. Many patients with increased tions and movements must not be seen as sepa- tone demonstrate a degree of asymmetry which, rate entities; each is dependent on the other to without treatment, may lead to contracture ensure the optimal functional outcome. within the stereotyped patterns. An example of this pattern of contracture is illustrated in Supine lying Figure 6.3. Patients with hypotonia totally accept the large The pattern includes: supportive surface and it is important to ensure that the support provided by the bed preserves • rotation of the pelvis relative to the thorax the optimal alignment of muscles and joints. • lateral flexion of the trunk • 'windswept' lower limbs Although patients are rarely placed on a totally • unilateral hip adduction flat surface it is important to recognise the poten- • abduction of the opposite hip tial complications which may arise from this • bilateral knee flexion (Pope et al 1991). positioning. With a totally flat surface, there is a tendency for the spine to adapt to the contours of This pattern of contracture not only precludes the support, with the lumbar spine becoming sitting and standing but also adversely affects the flatter and the thoracic spine becoming more patient's ability to accept the base of support in extended. The shoulder girdles tend to elevate supine and side-lying. and fall back into retraction. The feet, unless sup- ported, become plantar flexed with the danger of Principles of positioning in bed contracture of the triceps surae. The patient may be confined to bed, particularly Patients with extensor hypertonus may be in the early stages, and is therefore able to experi- unable to accept this large, flat base of support, ence only a limited variety of positions and move- pushing back against the resistance offered by ment. The positions are generally restricted to the surface and thereby exacerbating this domi- those of side-lying, supine, half-lying and occa- nant posture. The patient with flexor hypertonus sionally prone. Movements are dependent upon may be unable to release this tone to accommo- the position and are compromised by limitations date to the flat surface. In most instances, both imposed by proximal inactivity or inappropriate extensor and flexor hypertonus are compounded activity as may result from hypertonus. The posi- by an element of asymmetry, making appropriate Figure 6.4 Supine lying with wedges.
134 NEUROLOGICAL PHYSIOTHERAPY placement virtually impossible. Alterations to loading of tissues and can improve the alignment spinal and pelvic alignment are determined by of body segments (Pope 1992). the prevailing tone. This posturing is very much dependent upon Modification of supine lying, by the use of a the type of bed and firmness of the mattress. wedge, T-roll or pillows, may be of value in con- Many different types of mattresses are now avail- trolling the alignment of the spine and in facili- able and air beds are frequently used in the tating acceptance of this position. Wedges may management of people with neurological dis- be used to good effect both under the head ability. Elevation of the head of the bed is often and shoulders and under the knees (Fig. 6.4). For preferred to placing additional pillows or wedges those with predominant extensor hypertonus, to control the alignment of the head, neck and the introduction of flexion provides a point of fix- shoulder girdles. However, although elevation of ation at the pelvis about which movement from the head of the bed improves the alignment of lying can be facilitated. The head is in alignment the upper body, it produces flexion at the hips, with the trunk in the sagittal and coronal planes, and hip flexor contractures are second only reducing the tendency for increased extension of to plantar-flexor contractures as a consequence the cervical spine and for the shoulder girdles to of neurological impairment (Yarkony & Sahgal pull back into retraction. The flexion introduced 1987). at the hips and knees breaks up the dominant extensor hypertonus of the legs. Side-lying Patients with predominant flexor hypertonus This position is often utilised in the manage- may be unable to release the flexor activity if the ment of patients with neurological dysfunction supporting surface is flat and fully extended. In (Bobath 1990, Davies 1994). It introduces oppos- cases where there is severe flexor hypertonus, ing influences of tone on the two sides of the a larger wedge may be required in the first body: on the supporting side, one of extension, instance, its size being gradually reduced as the and on the non-weight-bearing side, one of patient becomes able to accommodate to a more flexion. The side-lying position is similar for extended position. If there is established shorten- patients with either low tone or increased tone. ing of the hip flexors, the graduated use of The patient may be positioned with either the wedges can be effective in regaining range of underneath leg extended and the uppermost leg movement. Many patients with either flexor or flexed or with both legs flexed. The underneath extensor hypertonus present with an asym- shoulder is protracted to prevent pressure falling metrical posture. The use of a T-roll (Fig. 6.5) may over the acromion process, and the pelvis is be of value in that it promotes more equal rotated slightly towards prone. Pillows are required, one or two in front of the patient to Figure 6.5 Supine lying with T-roll. support the arm and encourage maintenance of this position towards prone, and one between the knees to stabilise the pelvis and prevent adduc- tion of the uppermost leg. A pillow behind the patient should not be necessary, providing the pelvis is tilted slightly forwards, unless there is uncontrolled movement. Asymmetrical posturing of the head and trunk may also be corrected or improved with the use of pillows or foam wedges. For example, pillows are used to maintain the head in alignment in the coronal plane, and positioning of a pillow under the left side, when in left side-lying, may
GENERAL PRINCIPLES OF TREATMENT 135 prove effective in maintaining range within the under the thorax alters the contour of the spine opposing trunk side flexors. and enables controlled weight-bearing through the forearms. The protracted position of the arms Davies (1994) advocates the use of wedges facilitates flexion at the thoracic spine, thereby and/or pillows to fill in the 'gaps', particularly overriding the tendency towards extension when for patients with perceptual and /or cognitive the patient is positioned in supine. The extended impairments. This additional support is con- position of the lumbar spine serves to maintain sidered to provide a feeling of greater security for the lumbar lordosis. From this position, normal these often-disorientated patients. extensor activity may be recruited in the head and trunk (Fig. 6.6). Prone lying Positioning in this way may also be of benefit This position is recommended as a means of main- for patients with extensor hypertonus, as the taining or correcting range into extension particu- overall influence is one of flexion. For these larly at the hips and knees (Pope 1992, Davies patients, the emphasis is more on mobilising the 1994). However, prone lying should be used with shoulders forwards into protraction to counter- caution in that it may exacerbate flexor hyper- act the commonly observed retraction. Extensor tonus. Patients with flexor hypertonus rarely ac- activity of the cervical musculature may also commodate to this position without the additional be inhibited when the patient is in prone support of a wedge or pillows. Davies (1994) advo- lying. cates the use of pillows under the trunk or pelvis to allow acceptance of the support, gradually reduc- Prone lying is recommended for improving ing them until the patient can he in full extension. oxygenation in ventilated patients with acute The feet should be positioned over the end of the respiratory failure (Wong 1998) and the presence bed to prevent shortening of the triceps surae of a tracheostomy need not be a precluding muscle group. factor. The main disadvantage of this position is that there is little stimulation when lying face For patients with hypotonus, prone lying may downwards and, in some instances, the degree of be a useful adjunct to treatment. A wedge placed neck rotation may be limited. Figure 6.6 Prone lying with forearm support over pillows.
136 NEUROLOGICAL PHYSIOTHERAPY Summary from behind the patient are more effective in reducing tone and increasing mobility. Smaller Limb movements are compromised if there is range movements with less support and inter- reduced spinal mobility and inadequate or mittent pressure, as required to gain a 'holding' inappropriate muscle tone to enable effective response, are more effective in stimulating activ- proximal adjustments. Where bed rest is unavoid- ity. Recently, Thornton & Kilbride (1998) have able, it is important to maintain adequate move- described handling techniques which may be ment within the trunk to maintain mobility and used for patients with varying levels of tonus. to stimulate the proximal control associated with limb movements. Although frequently less Patients with hemiplegia develop compen- obvious than contractures affecting the limbs, satory strategies to contend with their asym- loss of range and impaired coordination of metry. Those with hypotonus may develop the trunk musculature has a dramatic effect on excessive activity of the 'unaffected' side as they function (Davies 1990). struggle to support the weight of the flaccid hemiplegic side. A general assumption among Movement between lying positions and when clinicians is that it is this overactivity of the coming up from lying to sitting should be carried sound side that precludes activity of the affected out with care and the patient given time to ad- side. Mobilisation of the trunk is of particular just to the change of position, particularly when benefit in this situation as the physiotherapist postural hypotension prevails. Turning from can facilitate a more appropriate response side to side or being hurriedly taken into sitting between the two sides of the body. In this situ- can also be a frightening experience, particularly ation, it is the 'unaffected' side which is in danger if there are perceptual problems and sensory of becoming shortened. impairment. However, it should be noted that in a recent Principles of positioning in sitting study by Dickstein et al (1999) the muscles on the paretic side were not found to be activated to a The principles for appropriate seating are lesser extent than those on the non-paretic side described in detail in Chapter 9. during symmetrical trunk movements. Trunk mobilisations Hemiplegic patients with hypertonus may develop shortening of the trunk side flexors of the In the treatment of neurological patients, trunk affected side. Mobilisation of the trunk is again mobilisations refer to movements of the trunk, appropriate but in this instance the emphasis is on facilitated by the physiotherapist, which are used reducing the hypertonus in the affected side. to modify abnormal tone and improve align- ment. In this context they should not be con- This scenario illustrates the global effects of fused with specific vertebral mobilisations as neurological impairment. Although hemiplegia advocated by manipulative therapists. is by definition a condition affecting half of the body, the consequences of motor and sensory Treatment of the patient in sitting provides an impairment of one side inevitably affect all opportunity for the physiotherapist to mobilise aspects of movement. the trunk and facilitate correct alignment with proximal control and stability. Trunk mobilisa- Use of the gymnastic ball tion, as used in this way, is considered by some practitioners to be a somewhat passive form of The ball is made of resilient plastic and comes intervention. However, as with all techniques, it in various sizes. In treatment, it is used pre- is dependent upon the physiotherapist's skill and dominantly in eight directions - forwards, back- expertise to elicit the desired response. Slow, wards, to each side and diagonally forwards and rhythmical movements, providing full support backwards (Lewis 1989). Carriere (1999) provides a comprehensive description of the use of the gymnastic ball.
GENERAL PRINCIPLES OF TREATMENT 137 Klein-Vogelbach (1990) gives detailed reference • to mobilise the trunk and limbs to specific techniques which may be used to opti- • to retrain balance reactions and coordination mise stability and/or mobility. These are active • to strengthen postural muscles (Hasler 1981). exercises performed by the patient with assistance from the therapist as required. These techniques Patients may be mobilised, sitting on the were originally designed for the treatment of or- ball with the physiotherapist supporting from thopaedic conditions. Hasler (1981) adapted Klein- behind. In this way the potential loss of range, Vogelbach's method for the treatment of patients especially at the thoracolumbar spine and pelvis, with neurological disability and Silva & Luginbuhl may be prevented or minimised. A second (1981) and Edwards (1998) recommend its use in person may be required to control the patient's the treatment of patients with incomplete spinal legs to prevent them falling into abduction and cord injury. The gymnastic ball has also been rec- lateral rotation (Fig. 6.7). Slow, deliberate move- ommended as a treatment modality for patients ments of the ball both laterally and forwards and with hemiplegia (Lewis 1989, Davies 1990). backwards can prove most effective in inhibiting hypertonus and maintaining mobility. The ball is useful: Careful assessment of patients who may bene- • to stimulate dynamic co-contraction of the fit from this intervention is essential. Great care trunk musculature must be taken when transferring the more dis- abled patients on to the ball. The ball must be sta- bilised by another person as the patient is moved from the chair or plinth, which should be of a similar height to that of the ball. If the patient is unable to participate in the transfer, the use of a hoist should be considered. The gymnastic ball may be used to good effect in preserving or regaining range of movement and in stimulating postural adjustments, par- ticularly within the trunk and lower limbs. However, it should be used with caution because such a mobile surface may provide insufficient support for the patient to effect the desired response. It should also be appreciated that, although the gymnastic ball is widely used in many neurological physiotherapy departments, there is little evidence of its effectiveness (Jackson 1998). Figure 6.7 Patient on gymnastic ball. Principles of positioning in standing Standing is advocated as a means of maintaining joint range and stimulating anti-gravity activity (Ada et al 1990, Pope 1992, Brown 1994). Dif- ferent types of equipment are recommended to achieve this goal. The selection of the most appropriate aid is dependent upon the size and weight of the patient, the patient's medical status and the assistance available. In the light of moving and handling legislation, any type of
138 NEUROLOGICAL PHYSIOTHERAPY standing aid must ensure the protection and protection of the skin by ensuring that any rigid well-being of staff and carers. points are covered by a towel or other means of padding. Positioning of the feet is of particular concern. Patients with low tone may be placed in standing The tilt table is the method of choice where to maintain the length of soleus and gastrocne- there is cardiovascular and/or autonomic insta- mius and thereby prevent development of con- bility with the potential danger of hypotension. tractures (Ada et al 1990, Pope 1992). However, In these instances, it is imperative that the patient loss of range of the triceps surae is a recognised can be restored to the horizontal position at the complication of excessive hypertonus (Yarkony first sign of distress. In many cases, the blood & Sahgal 1987). In this situation, support must be pressure is monitored throughout the procedure provided under the heel to accommodate this to ensure there are no untoward effects. shortening. The effect of this support is twofold: The tilt table is perhaps the most passive • A foam wedge placed under the heel reduces means of taking the patient up into standing. The the stress placed on shortened structures by common characteristics of normal alignment in increasing the contact area. standing are dependent upon an intact neuro- muscular system and have been described in • This increased base of support enables more Chapter 3. Where there is abnormal tone caused equal weight-bearing through the full surface by neurological impairment, the patient is unable of the foot. If there is no support under the heel, to attain this normal alignment. The use of the tilt the pressure is exerted through the metatarsal table brings the patient up into standing but does heads, which may exacerbate the hypertonus. little to facilitate the postural adjustments essen- The pressure exerted by the patient's own body tial for normal standing (Cordo & Nashner 1982, weight may place unacceptable strain over the Friedli et al 1984). medial arch and plantar fascia, and damage to these structures may ensue. Patients with predominantly low tone adapt to the gravitational force, supported only by the By using support in this way, the patient may straps securing them to the table. The pelvis tilts gradually be able to accept a smaller wedge as anteriorly, with a resultant increase in the lumbar the range of movement increases. Mobilisation of lordosis and consequent flexion of the hips. In the plantar flexors while in standing may be this position, the iliopsoas muscle is in a short- effective in maintaining or regaining range of ened position with the potential for development movement. of hip flexion contractures. The knees tend to hyperextend, with the resultant loss of the planti- The tilt table grade position at the feet through the shortened position of gastrocnemius. To prevent the head This is frequently used, particularly in the early and shoulders falling forwards and the patient stages of treatment. Patients who are uncon- hanging on the trunk strap, the tilt table is fre- scious and/or have hypotonus require total quently positioned a few degrees from the full support to enable them to achieve an upright vertical. The shoulder girdles retract with the posture. The tilt table affords this support and is head extended, precipitating shortening of the perhaps the most commonly used form of inter- extensor muscles of the cervical spine. Clearly vention. It is of particular value for patients who if the therapist is aware of these potential com- are excessively heavy or need a graduated means plications, then adjustments can and should be of standing while monitoring for signs of pos- made. Use of the tilt table purely to attain an tural hypotension. The patient may be trans- upright posture is of little value to the patient in ferred on to the tilt table either by means of a terms of regaining correct postural control. hoist or by sliding them on an 'easy-slide', from their hospital bed on to the table. When using the The tilt table should be used only as an adjunct latter technique, attention must be paid to the to treatment. Where possible, the straps should be removed, particularly the trunk strap. In the
GENERAL PRINCIPLES OF TREATMENT 139 sion by means of the knee strap will not only exacerbate the hypertonus but may also cause considerable discomfort to the patient. Equally, the attainment of more extension at the knees by these means will inevitably create problems both proximally and distally. The use of force to attain improved alignment is ineffective in terms of controlling hypertonus or in the prevention of subsequent contractures. Summary. The tilt table is a valuable tool in enabling the patient to stand. If there is any risk to staff in standing patients by more dynamic means, then the tilt table is the method of choice. Where possible, other means of standing should be con- sidered to ensure correct alignment and to provide greater stimulation and a more dynamic response. Figure 6.8 Tilt table. The Oswestry standing frame majority of cases this need not be used if the ther- This frame was originally designed by a para- apist supports the patient from the shoulder plegic patient for paraplegic patients. These girdles with the arms extended and thus stimu- people can lift themselves up into the frame and lating head and trunk control (Fig. 6.8). A pillow stand independently once the straps are secured. or towel may be used behind the knee to prevent The Oswestry standing frame has now become hyperextension, but it must be recognised that one of the most commonly used frames, not only this in itself does not address the primary in spinal injury units but also in general hospitals problem, that of the anterior tilt of the pelvis. and certainly in the majority of neuroscience centres in the United Kingdom. It is also fre- The tilt table is not generally recommended for quently prescribed for use in the home for phys- patients with severe extensor hypertonus, pri- ically disabled people with diverse neurological marily because it provides an extensor surface pathologies. It is durable and relatively cheap. against which they may resist, thereby exacerbat- However, its use for the more severely disabled ing their tone. For those with flexor hypertonus clients is being challenged. the tilt table may be of value in controlling the legs in a more extended position. This position- In the light of current moving and handling ing should only be used in conjunction with legislation, it is essential to select carefully only mobilising techniques to first reduce the those patients who are able to assist bringing increased flexor tone. Forcing the legs into exten- themselves up into standing. In the past, heavily disabled patients have been lifted up into the Oswestry standing frame by two or more therapists. However, the possible benefit to the patient must be weighed against the difficulty for the carers in lifting the patient into the frame. In the majority of cases, certainly in the community, this is deemed to be an unacceptable risk. Alternative means of standing must therefore be considered. The wooden frame affords tremendous stabil- ity while allowing access for the therapist from
140 NEUROLOGICAL PHYSIOTHERAPY behind. Three sheepskin-covered straps attach • stimulating proximal muscle activity with behind the ankles, in front of the knees and correct alignment of the legs and pelvis behind the hips. If correctly applied, these secure the lower limbs in a position of extension and • mobilisation of the patient with hypertonus to enable the therapist, standing behind the patient, prevent shortening of structures dominated by to ensure correct alignment of the pelvis. the increased tone. It may be considered as 'an extra pair of hands' The therapist can ensure that the pelvis is in neutral or preferably in a position of slight poste- in providing proximal and lower limb stability to rior tilt. This enables full extension at the hips allow for functional use of the arms. with mechanical lengthening of the iliofemoral ligament and the iliopsoas muscle. Maintenance Manual and powered hoists have now been of the length of these two structures prevents the developed to overcome the difficulties of lifting compensatory lordosis of the lumbar spine and the more heavily disabled patients up into this extension at the shoulder girdles. This position- frame (Fig 6.9). ing also facilitates abdominal activity. Figure 6.9 Oswestry standing frame with lifter. To achieve this optimal alignment it is import- ant that the straps are correctly applied. • The hip strap must be taut to prevent the patient 'sitting' on it and thereby producing flexion, adduction and medial rotation at the hips. • The knee strap must be positioned over the patella tendon, as opposed to over the patella itself, and must maintain the knees in exten- sion. A foam wedge or towel may be placed between the knees to prevent valgus. • The foot strap is attached behind the heels. The Oswestry frame allows for more dynamic intervention in terms of movement. The hip strap may be removed, with the therapist standing behind and supporting the patient. In this way the therapist can allow the patient to move down towards sitting and facilitate movement back into standing, ensuring correct pelvic alignment. The therapist should have a plinth positioned behind her at the correct height to ensure that this activity is safe. Patients may also be mobilised by use of the table. By taking the patient forwards to rest over pillows or a wedge on the table, the thoracolum- bar spine may be mobilised and elongation of the cervical spine obtained. The patient may stand supported in this position to ensure extensibility of the hip extensors, knee flexors, ankle plantar flexors and of the neural structures. Summary. The Oswestry standing frame is designed in such a way as to enable its use for:
GENERAL PRINCIPLES OF TREATMENT 141 Figure 6.10 Standing in Richter frame using motorised lifter. Standing frame with motorised lifter ically capable, the patient operates the control button himself; otherwise, the therapist takes res- Standing frames with motorised lifters are ponsibility. At any time the lift may be stopped to increasingly being purchased to comply with allow the patient time to adjust to the more upright moving and handling legislation. Similar in posture. A quick release mechanism is provided in design to the Oswestry frame, the Richter stand- the event of an emergency, whereby the patient ing frame, which is but one example, enables the may be immediately restored to the sitting posi- patient to be taken gradually from sitting, up into tion. standing, at the touch of a button. The ther- apists) may need to support the patient as he In standing, there are a variety of accessories comes up into standing, but the 'heavy work' is which may be used to support even the most done by the motorised lifter (Fig 6.10). severely disabled patients. These include scapulae, lateral and head supports in addition to hip, knee A strap is placed under the patient's seat and and foot supports which work on a similar princi- attached to a mains or battery drive unit. If phys- ple to those of the Oswestry frame. However, the
142 NEUROLOGICAL PHYSIOTHERAPY pads which secure the hips, knees and feet provide Figure 6.11 Prone standing table. more control because they surround these joints, preventing medial rotation and adduction at the porting surface is on the flexor aspect, which hip and valgus at the knee. The cup support prevents the patient pushing backwards and exa- around the foot also maintains a more stable posi- cerbating his extensor tone. At the same time, this tion. positioning may stimulate more normal extensor tone by providing secure and stable proximal Although they are more expensive, given support. the moving and handling legislation, standing frames with a motorised lifter will inevitably The stability afforded to the trunk, pelvis and become essential equipment in the management of lower limbs enables more specific intervention people with neurological disability. Already, in the for facilitation of, for example, head control or settlement of many medicolegal cases, standing increased range of movement of the upper limbs. frames with a motorised lifter are accepted as the norm. It is to be hoped that their provision in hos- The main disadvantage of the prone standing pitals throughout the United Kingdom is not too table is the relative difficulty in positioning the far distant. patient in the frame and attaining the upright position. As with all moving and handling, care- Summary. Electric standing frames enable ful assessment is necessary to determine the ef- therapists and care staff in the community to fectiveness of this standing aid. For adults, as stand people with severe neurological disability opposed to children, with neurological disability in a graded and controlled fashion while mini- the prone standing table is now rarely used. mising the risk of injury both to themselves and to the patient. Standing using two or more therapists This frame is a solid, very heavy structure. It This is a more dynamic intervention, providing for should be placed in the optimal position in the instant response and adjustment on the part of the gym or in the patient's home to avoid having to move it. It is ironic that, while this frame reduces the strain on professional staff and carers in standing the patient, attempts to move the frame have been known to cause serious back injuries. The principles of treatment underpinning its use are similar to those of the Oswestry standing frame. Stand-up wheelchair Wheelchairs enabling the person to stand while remaining in the chair are increasingly used in the management of children and adults with neurological disability. Details are given in Chapter 9. Prone standing table The prone standing table is more commonly used in the management of children with neurological disability but may also be of value for adult patients with extensor hypertonus. Straps over the trunk, hips and feet secure the patient, and a table is attached for arm support (Fig. 6.11). The sup-
GENERAL PRINCIPLES OF TREATMENT 143 therapist to the patient's changing status. How- One therapist stands in front of the patient, sup- ever, standing in this way is more demanding on porting the knees and securing the position of the staff and should only be attempted with patients feet. Her hands are placed under the patient's who have some activity are able to participate ischeal tuberosities from where she guides him actively in the stand and with whom the therapist into standing. Once the patient is upright, the ther- feels confident. Therapists new to working with apist in front of the patient can sit on a stool or neurological patients should not feel that this chair, maintaining control of the hips, knees and method of standing must be used at all costs. In feet (Fig. 6.12). The second therapist stands behind most instances, therapists need time to develop the patient supporting the patient's head and their handling skills and confidence to attempt this trunk as he is brought up into standing. Standing a more complex intervention. patient with hypertonus may need adaptation, depending on the severity and distribution of tone, Standing a patient with minimal activity re- and more than two therapists may be required. quires greater input from the therapists and the benefit to the patient of this more dynamic stand The advantage of standing the patient in this must be weighed against the risk to the treating way is that it enables the therapists to feel and staff. respond to any change in the patient's status. Movements may be facilitated from standing to sitting back on to the therapist behind, and up again into standing. Transfer of weight from one side of the body to the other, while maintaining the correct alignment, provides the sensation of movement between the two sides, which is of par- ticular importance for patients with a hemiplegic presentation. Summary. Standing patients in this more dynamic way is often of great benefit both to the patient and to the therapist. However, the ther- apist must be competent and confident to manage this technique. If there is any doubt in the mind of the therapist, or the patient shows concern, other means should be adopted, utilising equipment aids as indicated. Many experienced neurological therapists con- tinue to use either the tilt table or a standing frame, and with good reason. The persistent, insidious strain from heavy lifting and supporting patients with severe neurological deficit can have long- term consequences, particularly in relation to back injuries. Selection of the most appropriate treat- ment intervention must therefore always consider the therapist as well as the patient. Figure 6.12 Standing, using two physiotherapists. Use of back slabs Back slabs, made from either plaster of Paris or fibreglass material, may be used to maintain the legs in an extended position to stand. Davies (1994) considers this the method of choice when
144 NEUROLOGICAL PHYSIOTHERAPY Figure 6.13 Standing with back slabs in parallel bars. For patients with severe flexor hypertonus of the legs, it may be impossible to secure the slabs support is required to stand patients following without the use of excessive force. As previously head injury. They should be made specifically for discussed in relation to the tilt table, mobilising the individual patient and be securely bandaged techniques must first be used to enable the in place. (A detailed description is given in patient to accept this support. Ch. 10.) The use of a back slab has been advocated in The purpose of the back slabs is to enable the the early management of stroke patients with therapist to work for improved trunk and pelvic lack of midline orientation (Davies 1985). These control while stabilising the lower limbs. The ther- patients are often referred to as 'pushers' in that apist may choose to stand in front of or behind the they overuse the sound side, pushing themselves patient while stimulating head, trunk and upper off balance to the affected side. The back slab, limb control, or the patient may stand in the paral- applied to the affected leg, enables the patient to lel bars to facilitate weight-bearing through the transfer weight through this limb and thereby arms (Fig. 6.13). By maintaining a posterior tilt of regain more body awareness. This should be the pelvis, shortening of the iliofemoral ligament used with caution if flexor hypertonus becomes and of the hip flexor muscles may be prevented. dominant in the lower limb. Back slabs are mainly used in the treatment of The main disadvantage of using this method is patients with low tone and for those with flexor that the patient cannot be taken up into standing hypertonus. through sitting. With the legs fixed in extension, the patient may need assistance to stand and the potential benefit to the patient must be weighed against the strain placed on treating staff. Once in standing, it is impossible to facilitate release of activity to allow the patient to move towards sitting by coordinating movement between the pelvis, hips, knees and feet. A further disadvan- tage is that there is little stimulation of the lower limb extensors. Summary. The use of back slabs enables spe- cific mobilisation of the trunk on pelvis and the pelvis on extended lower limbs. In this way, they are of benefit in preventing the onset of, or reducing, hip flexor contracture. The main dis- advantage is that back slabs preclude normal movement from sitting to standing and, unless the patient has good arm function and is able to stand independently in parallel bars, standing the patient in this way places greater and poss- ibly unacceptable demands on the treating staff. MOVEMENTS Orofacial movements Patients with severe brain damage may demon- strate significant problems relating to impaired control and coordination of facial structures. The
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