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Index Abduction vigabatrin in, 207 pathophysiology in the rodent model of hand, 146, 148 work-related injuries in, 8 multiple sclerosis, 432 hip, 151 wrist flexors in, 23 shoulder, 18, 141 Agonist/antagonist cocontraction, 75 pathophysiology of, 423 Alcohol, 108–109, 350 arising from SCI, 423–427 Action Research Arm Test (ARAT), 8, 60, administration in pediatric 175, 191–192 physiological changes in spinal activity population, 112 and spasticity, 427–432 Active function, 85, 120 motor point blocks and, 331 examples of, 62, 85 therapeutic nerve blocks and, 331 types of passive and, 53, 61 Alcoholic myelopathy, 273 rodent model of cerebral palsy, 422 treatment to improve, 139 Alcoholism, 272 rodent model of multiple sclerosis, 422 Alexander disease, 273 spastic, spasmotic, and Activities of daily living (ADL), 60, 85, 131, ALS. See Amyotrophic lateral sclerosis (ALS) oscillator mutant mouse mod- 191, 297, 344 ALS Functional Rating Scale (ALSFRS), 301 els, 422–423 Alzheimer disease, 282 spinal cord injury models of spasticity, Adduction Ambulation Index (AI), 345 419–422 hip, 150–151 Ambulatory children, 400 shoulder, 72–73 Ambulatory patients, 236 Anisotropy, 184 thigh, 125–126 4-Aminopyridine (Fampridine®), 328 Ankle Amyotrophic lateral sclerosis (ALS), 281 Adhesive capsulitis, 37 epidemiology, 298–299 dorsiflexion, 153 Adult-onset spastic paraparesis, 306 overview, 297–298 eversion, 153 Adults pathogenesis of Ankle great toe extension, 153 Alexander disease, 273 familial ALS, 299 inversion, 153 botox dosing for, 127 sporadic, 299 joint anatomy, 153 box and block test (BBT) in, 59 pathology, 300 plantar flexion, 153 with CP, 45 symptomatic (spasticity), 302–303 Ankle Ashworth test, 84 daily allowance of copper for, 309 treatment Ankle-foot orthosis, 389 dantrolene in, 211 disease modifying, 300–302 Antispasmodic agent, 349 diazepam in, 204–205 Anemia, pernicious, 308 Antispasticity medications, adverse drug epidemiology of spasticity in, 5–13 Animal models for examining spasticity, 419 gait analysis in, 61 pathophysiology in rodent models of reactions for, 208 GM2-gangliosidosis, 273, 275 Appel Amyotrophic Lateral Sclerosis Rating with hemiplegia, 88 cerebral palsy, 432 paraplegic, 42 pathophysiology in spastic, spasmotic Scale, 302 phenol injections in, 384 Ashworth Disability Scale, 384 with phenylketonuria, 272 and oscillator mutant mouse Ashworth Scale, 42, 52, 56–57, 134, polyglucosan body disease, 273 models, 432–433 QOL for, 65 316–317, 344, 381 tizanidine in, 390 for assessment of muscle tone, 58, 82, 121, 133 limitations of, 363 439

440 index Ashworth Scale (cont.) BoNT-ona in poststroke spasticity, Central alpha-adrenergic agents as a metric for spasticity, 374 133–135 clonidine, 207–208 for physical assessments of spasticity, 157 tizanidine, 208–209 psychological issues, 42 BoNT-nima in spas, 136 case report, 138–139 Central pain syndrome, 35, 36. See also ASIA Impairment Scale, 314 clinical impact, 136–137 Pain Associated antiphospholipid syndrome, 277 common presentations, 137 Associated reactions, 26–27 consensus statements and evidence Cerebellar stimulation, 243, 244 Cerebral Disorders Spastic Hypertonia and mass reflex, 368 reviews, 139 Atlastin-1 (ATL1), 283–284 dosing concerns, 132–133 Treatment Paradigm, 212 ATPases, 305 injection technique, 137–138 Cerebral disorders versus spinal disorders, Atrophy, 297 mechanism of action, 132 AutoCITE, 192 studies of BoNT-abo, 135–136 221 Axonal sprouting, 315–316 sustained improvement with repeated Cerebral lesions, 203 Cerebral palsy (CP), 12–13, 28–29, 35 Babinski sign, 26, 366 injections, 135 Baclofen, 41, 175, 347–348, 374, 375, 390. Botulinum toxin injections, 4, 37, 331–333 botulinum toxin, 391–392 case study, 391, 392–393 See also Intrathecal baclofen (ITB) for bowel and bladder, 39–40 in children. See Children, with CP comparison with clonazepam, 325, 348 Botulinum toxins (BT), 21 clinical presentations of, 387–388 comparison with cyproheptadine, 327 defined, 387 GABA receptor agonist, 290, 302 chemodenervation with, 245 etiology and epidemiology, 387 on gait in spastic MS patients, 379 dosing for adults, 127 evaluation, 388 in MS patients, 347–348 dosing guidelines, 333 intrathecal baclofen, 393 oral dosage, 330 FDA terminology for different formula- oral medications, 390, 391 overdose, 239 phenol and ethyl alcohol, 392 side effect of, 204 tions of, 133 physical treatments, 389–390 structure, 203 skills required for use of, 95 treatment planning, 388–389 withdrawal, 330, 349 for spasmodic dysphonia, 39 Chaperones, 286 Baclofen/Clonidine Pump Clinic Bowel and bladder, 39–40 Charlevoix-Saguenay syndrome, 273, 276 basic considerations in, programs, 42 Chemodenervation, 349–351, 361–362 Follow-Up Evaluation, 202 Box and block test (BBT) and range of motion (ROM), 92 Baclofen (Lioresal®), 323–325 in adults, 59 Chemoneurolysis, 101–102, 331 Baropodometric tests, 178 Brace Wearing Scale, 110 Child Health Questionnaire, 177 Barthel Index (BI), 6, 64, 83, 388 Bracing devices, 347, 406 Children. See also Hereditary spastic Beckman exercises, 37 Bruxism, 37 Bed positioning programs, 42 BT. See Botulinum toxins (BT) paraplegias (HSP) Benzodiazepines, 204, 205, 221, 325, 348 Bulbopontine tegmentum, 342 baclofen in, 374 with balance difficulties, 389 administration of, 237 Canavan disease, 207 BoNT in, 131, 391 and baclofen, 302 Cannabinoids intravenous, 330 dosing, 132 pharmacokinetics, 204 pharmacokinetics, 210 casts, 59 treatment, 204–205 treatment, 210–211 congenital abnormality in, 272 Berg Balance Scale, 53, 345 Cannabinoids (Marinol®, Cesamet®), 328 with CP, 59, 171, 388–389 Biering-Sorensen postural analysis, 83 Cannabis, 348–349 Bilateral spastic CP, 13. See also Cerebral Caregivers alcohol administration in, 108–112 and family, 52, 71, 92 ambulatory children, 400 palsy (CP) Carisoprodol, 349 BTX A injection in, 178 Blood oxygen level difference fMRI, 185 Carpal tunnel syndrome, 36, 74, 245, 256 CITB in, 401 Body image integrity, 30 Casting, 36. See also Serial casting (SC) CIT program for, 390 Body weight–supported ambulation, 168 inhibitive, 54 deformities in, 36 Body weight-supported treadmill training Casts, 78 with equinus foot, 178–179 application of, 165 musculoskeletal deformities in, 407 (BWSTT), 192–194 children, 59 orthopedic surgical interventions for, Bone immobilization, 411 lightweight, 268 405, 406 and joint integrity, 30 postoperative, 260 prevalence, 12–13 mineral status, 343 walking, 265 speech, 34 BoNT. See Botulinum neurotoxin (BoNT) Catheter subfascial placement, 402 Botulinum toxin (BoNT), 6, 83–84 imaging evaluation of, 238 surgical options for, 397, 398 chemodenervation, 87 malfunction, 238 diazepam in, 204 role in spasticity treatment, 126–128 Catheter access port (CAP), 238 dynamic splinting in, 167 for TBI treatment, 10 Cavus deformity, 266 gabapentin in, 205–206 Botulinum toxin (BoNT), treatment of Cavus foot, 266 gait analysis, 61 C elegans models, 285 intrathecal baclofen in, 236 upper extremity spasticity with, Pediatric Evaluation of Disability Inven- 131–132 tory (PEDI), 64

index 441 preambulatory, 389 botulinum toxin type A injection and SC, Diffuse axonal injury, 184 prevalence of multiple sclerosis, 11 177–179 Diffusion imaging, 184–185 with spastic ankle equinus foot, 178 Diffusion-perfusion mismatch, 184 with spasticity from Canavan botulinum toxin type A injection and Disability Assessment Scale (DAS), 52, 92, therapeutic exercise, 177 disease, 207 133, 134, 384 spasticity treatment in, 156 Compartment syndrome, 107, 165 Disabling spasticity (DS), 6 with spastic quadriplegia, 392 Complex regional pain syndrome (CRPS), Distal hereditary motor neuropathy sports-related spinal cord injuries, 8 with symptomatic infantile spasms, 207 35. See also Pain (dHMN), 281 tiagabine in, 206 Composite Spasticity Scale (CSS), 161 Disuse atrophy, 305, 343 Christopher & Dana Reeve Foundation Conditional knockout model, 286 Ditunno’s model, 315–316 Confusion, 203, 205, 302, 324, 325, 349, Drawing Test, 169 Paralysis Resource Center (PRC), 5 Dronabinal, 210–211 Chronic relapsing experimental allergic 350 Drooling, 33 Consortium of Multiple Sclerosis Drosophila models, 284–285, 291 encephalomyelitis (CREAE), 422 Drowsiness, 203 Ciliary neurotrophic factor (CNTF), 302 Centers, 344 CIMT. See Constraint-induced movement Constraint-induced movement therapy due to benzodiazepines, 205 due to carisoprodol, 349 therapy (CIMT) (CIMT), 45, 186–187, 187 due to clonidine, 327 CIT. See Constraint-induced therapy (CIT) botulinum toxin type A injection and, due to dantrolene sodium, 326, CITB. See Continuous ITB (CITB) infusion Clasp-knife response, 367–368 176–177 381 Clenched fist, 92 modified, 176, 177 due to orphenadrine, 221 using AutoCITE, 192 due to progabide, 349 anatomy, 147 Constraint-induced therapy (CIT), 389–390 due to vigabatrin, 206–207 due to UMNS, 75 Continued medical education (CME), 133 during ITB trials, 233 in hemiparetic patient, 29 Continuous ITB (CITB) infusion, 229–230 Dry mouth, 303, 326, 327, 348, 349, 374, interfering with splinting and hand Contractile tension, 22–23 Contracture, 28, 120 390 hygiene, 138 Contusion/Compression Model, 421 BoNT and, 136 and skin integrity, 30 Copper deficiency myelopathy Dutch children. See also Children spastic postures, 148 overview, 309 prevalence of cerebral palsy (CP) in, 13 Clinical assessment of spasticity, 121 pathology, 309 Dynamic Gait Index, 161 adducted (scissoring) thigh, 125–126 pathophysiology, 309 Dynamic splinting, 166 equinovarus foot, 122–124 treatment, 309–310 Dynasplints, 166–167 flexed hip, 126 Cost Dynorphin, 431 hyperextended great toe, 124 comparisons of phenol neurolysis, Dysarthrias, 37, 39 impact of gait, 121–122 Dysphagia, 37 patterns of UMN dysfunction, 122 111–112 Dyssomnia, 41 stiff knee, 124 CP. See Cerebral palsy (CP) Dystonia, 24–25 Clonazepam, 204 CRASH syndrome, 289 Dystonic posturing, 77 Clonidine, 207–208 Cyclobenzaprine Clonidine (Catapres®), 326–327 Elbow Clonus, 22. See also Stretch reflexes pharmacokinetics, 212 assessment, 73–74, 144–146 Clostridium botulinum, 132 treatment, 212 extension, 167 Cocontraction, 24, 94, 119, 368 Cyproheptadine, 209–210, 349 flexion spasticity, 248, 254–255 of antagonist muscles, 86 Cyproheptadine (Periactin®), 327 due to decreased Ia interneuron activity, Electrical stimulation (ES), 168, 189–190, Daily isometric exercises, 343 322, 374 315 Dantrolene, 211–212, 381 dystonic, 272 Dantrolene sodium, 21, 348 biofeedback and, 389 nerve conduction and, 275 to hip abductors, 126 of shoulder extensors, 73 pharmacokinetics, 211 intraoperative, 397 spastic, 75, 236, 272, 273 treatment, 211–212 maximal, 318 Cold, 172–174 Dantrolene sodium (Dantrium®), 325–326 motor point stimulation using, 332–333 Cold therapy, 346 Daytime hypersomnia, 41 percutaneous, 114 Collagen, 343 Deep tendon reflexes, 366 transcutaneous electrical stimulation Colorado Spinal Cord Injury Early Notifica- brisk, 272–273, 276, 277 Delayed sleep phase syndrome, 42 (TENS), 347 tion System, 9 Dendritic arborization, 325 Emory Functional Ambulation Profile, 63 Coma, 233, 239, 324, 325, 330, 348 Depression, 43 Enteral baclofen, 375, 379. See also Combination therapies, 174 Detrusor-sphincter dyssynergia, 33 Diagnostic and Statistical Manual of Mental Baclofen botulinum toxin type A injection and Equinovarus deformity, 84 CIMT, 176–177 Disorders, Fourth Equinovarus foot, 122–124 Edition Equinovarus of foot/ankle, 112 botulinum toxin type A injection and for sleep disorders, 41 Equinus gait, 109 electrical modalities, 174–176 Diazepam, 204 Diazepam withdrawal syndrome, 325, 390

442 index ES. See Electrical stimulation (ES) epidemiology, 307 SPG4, spastin (SPAST), 284–285 Ethanol, 350, 392 overview, 306–307 SPG6, nonimprinted in Prader-Willi/ EuroQol, 65 pathology, 307 Excitatory postsynaptic potentials (EPSPs), pathophysiology, 307 Angelman 1 (NIPA1), 285 treatment, 307 SPG8, strumpellin (KIAA0196), 285 420 Fugl-Meyer Upper Limb Test, 161 SPG10, kinesin 5A (KIF5A), 285–286 Excitotoxicity model, 299 Functional ES, 168–170. See also SPG13, heat shock protein 60 Expanded Disability Status Scale (EDSS), Electrical stimulation (ES) (HSP60), 286 345 Functional goals, 85 SPG17, seipin (BSCL2), 286–287 Extensor reflexes, 26 Functional implications of spasticity, SPG31, receptor expression enhancing Extrafusal muscle fibers, 22 120–121 protein 1 (REEP1), 287 Familial spastic paraplegia, 281. See also Functional Independence Measure (FIM), 53 SPG33, protrudin (ZFYVE27), 287 Hereditary spastic paraplegias (HSP) autosomal recessive, 287–289 score, 84 emerging pathways in, 282–283 Family Functional magnetic resonance imaging epidemiology, 305 AAA protein, 284, 286 genetic heterogeneity in, 282 benzodiazepine, 325 (fMRI), 185–186 overview, 304–305 and caregivers, 52, 71, 92 Functional near infrared spectroscopy pathology, 306 FYVE-finger, 288 pathophysiology, 305–306 history of HSP, 282 (fNIRS), 186 perspective, 290–291 history of spastic disorder, 272 F waves, 321 prevalence, 282 treatment, 306 FDA terminology for different formulations Gabapentin, 349, 380–381 X-linked, 289–290 of botulinum toxins, 133 pharmacokinetics, 205 Hip, 149–151 treatment, 205–206 abduction, 151 Fecal incontinence, 40 adduction, 150–151 Fibromyalgia, 349 Gabapentin (Neurontin®), 327–328 adductor spasticity, 54 Finger flexion, 147–148 Gain, 56 extension, 150 Gait analysis, 61 spasticity, 260 spasticity, 256–258, 258–259 Gamma-aminobutyric acid (GABA) ago- external rotation, 150 Finger flexor spasticity, 72 flexion, 126, 149 Fingers, 75 nists, 200, 203 and adduction spasticity, 259–260 Fixed attitude dystonia, 24. See also Dys- pharmacokinetics, 203 internal rotation, 150 treatment, 203–204 joint anatomy, 149 tonia GBP3, 283–284 Hispanic children. See also Children Flaccid dysarthria, 37 Glasgow COMA Scale (GCS), 371 prevalence of cerebral palsy Flexed elbow, 18, 25, 30, 137, 144 Global Attainment Scale, 177 Global Spasticity Scale, 171 (CP) in, 13 musculocutaneous nerve block versus Glycine, 326, 349 Hitchhiker’s toe, 124 botulinum for, 111, 114–115 Goal Attainment Scale, 84, 136 Hmax/Mmax ratio, 52–53 Gracillis muscle, 261 Flexed hip, 126 Gross Motor Function Classification Sys- sensitivity in presence of baclofen, Flexion plus extension, 148 238–239 Flexor digitorum profundus (FDP), 147 tem, 388 Flexor reflex, 26 Gross Motor Function Measure (GMFM), Hoffman reflex (H reflexes), 421 Homocysteine, 309 afferents, 26 171 HSP. See Hereditary spastic paraplegias Flexor spasms, 26, 368 Guanylate-binding protein 3, 283–284 Flexor synergy pattern, 18–19 (HSP) fMRI. See Functional magnetic resonance Hallucinations, 203, 204, 209, 237, 325, Human spastic syndrome, 421 325, 330, 374 Hyperextended great toe, 124 imaging (fMRI) Hyperextension deformity, 267 Focal distribution, 92 Hand Hyperkinetic dysarthria, 37 Foot anatomy, 147–148 Hypertonia, 367 spastic postures, 148–149 Hypokinetic dysarthrias, 37 dorsiflexion, 153 eversion, 153 Hand hygiene, 93, 94, 97, 134, 135 Immobility due to spasticity, 343 great toe extension, 153 BoNT and, 137 Impingement syndrome, 30 inversion, 153 Indium I-111 DTPA, 238 joint anatomy, 153 Health-related quality of life (HRQL), 7. Infants, 13, 309 plantar flexion, 153 See also Quality of life (QOL), Infections, 308, 393 Foot and ankle spasticity, 262–267 measures Inhibitory casting, 164 Forearm pronation spasticity, 255–256 Injection guided therapy Fractional anisotropy (FA), 184 Heat, 174 Fractional lengthening, 250 Heat therapy, 346 botulinum toxin, 384–385 Fracture, 156, 164, 235, 251, 347, 407, 421 Hemineglect phenomena, 45 phenol, 384 Fragile X syndrome, 273 Hereditary spastic paraparesis (HSP). See Frankel grades, 9 Frenchay Arm Test, 63–64 Hereditary spastic paraplegias (HSP) Friedreich ataxia (FA), 297 Hereditary spastic paraplegias (HSP), 281 autosomal dominant, 283–287 SPG3, atlastin-1 (ATL1), 283–284

index 443 Injury and physical pressure, 30 Isolated syndrome of progressive spinobul- Magnetic resonance spectroscopy (MRS), Insomnia, 42 bar spasticity, 303 185 after traumatic brain injury, 42 Isolated voluntary time movement tests, compounds detected by, 185 Interhemispheric inhibition, 189 59–60 imaging, 185 International Classification of Disease Manual muscle testing, 121 Isotropy, 184 MASA syndrome, 289 codes, 13 ITB. See Intrathecal baclofen (ITB) Mass reflex and associated reactions, 368 International Classification of Functioning, Mast syndrome, 282, 289 Jackson’s hierarchial model, 17 Matrix AAA ATP-dependent protease (m- Disability and Health (ICF) Jackson’s release phenomena formulation, framework, 86 AAA protease), 288 model, 86, 404 26 Medical College of Wisconsin (MCW), 5–6 Interneuronal circuitry, 22 Jebsen Taylor Hand Function Tests, Medical Research Council Scale, 82, 300 Intrarater and interrater reliability, 81 Medtronic Synchromed Infusion® system, Intrathecal baclofen (ITB), 4, 6, 328–330, 60, 169 Jebsen Taylor Hand test, 365 401 361, 379–380 Joints, 76–77 Menkes disease, 309 adverse events, 402–404 Meprobamate, 349 ambulatory patients, 236 and bone integrity, 30 Methylmalonic acid, 309 in children, 236 Joints, anatomical considerations for major Mitochondrial recessive ataxia for CP treatment, 393 delivery, cessation of, 237 ankle and foot, 153 syndrome, 307 dosing, 236–237 elbow, 144–146 Modified Ashworth Scale (MAS), 56–57, indications, 401 hand instrumentation, 401–402 157, 316–317, 344, 345, 374, 388 outcomes, 404–405 anatomy, 147–148 score, 6 overdose, 239 spastic postures, 148–149 Modified Modified Ashworth Scale, rationale, 401 hip, 149–151 in stroke, 45 Joints, anatomical considerations for major 56–57 for TBI treatment, 10 knee, 151–153 Modified Penn spasm frequency scale techniques, 402 shoulder Intrathecal baclofen (ITB) implantation, 233 anatomy, 141–143 (PSFS), 317. See also Penn Spasm long-term maintenance, 235–236 spastic postures, 143–144 Frequency Scale (PSFS) titration phase and postimplantation wrist, 146–147 Modified Tardieu Scale, 57. See also Tardieu Scale management, 234–235 Kessler Institute for Rehabilitation, Monosynaptic connections, 22 Intrathecal baclofen (ITB) infusion 10–11 Mood issues, 42, 45 Motor Activity Log Self Report Action components of ITB therapy, Kinesin 5A (KIF5A), 285–286 Research Arm Test, 384 230–231 Kinesin motor proteins, 286 Motor Assessment Scale, 8 Kjellin syndrome, 288 Motor disability, 13 neurophysiological mechanism of action, Knee Motor impairment syndromes, 12 230 Motor nerve block, 392 extension, 152–153 Motor neuron degeneration, 302 patient selection and the role of preim- flexion, 152 Mouse model, 300 plant trials, 231–233 joint anatomy, 151–152 MRS. See Magnetic resonance spectroscopy (MRS) Intrathecal baclofen (ITB) therapy, 39, 45, extension spasticity, 262 MS. See Multiple sclerosis (MS) 304 flexion spasticity, 260–261 Multiple sclerosis (MS), 11–12, Kurtzke Functional Systems Scale, 11 epidemiology, 342 history of, 229–230 evaluating spasticity, 343 loss of effectiveness of, 237 Learned nonuse, 186 Ambulation Index (AI), 345 in treating HSP, 306 Leeds Adult Spasticity Impact Scale, 85 Ashworth and Modified Ashworth use of, 93 Lee Silverman Voice Treatment, 39 Intrathecal baclofen (ITB) withdrawal syn- “Lever arm dysfunction,” 405 Scale, 344 Life-threatening syndrome, 237 Berg Balance Scale, 345 drome, 237, 302–303, 330 Likert Scale, 52–53, 63 Expanded Disability Status Scale Intrathecal clonidine, 330 Liver function tests, 274 Intrathecal drug deliver failure, 237 Locus ceruleus, 207 (EDSS), 345 Intrathecal medications, 351–352 Lou Gehrig disease, 297 Multiple Sclerosis Spasticity Scale, 344 Intrathecal phenol, 361 Low back pain, 349 Penn Spasm Frequency Scale and Lower limb dysfunction administration, 352 Spasm Frequency Scale, injection, 107 functional assessment of gait, 87–89 344–345 Intrathecal treatments Lower limb joint ROM, normal, 82 Tardieu Scale, 344 chemodenervation, 361–363 “Low reservoir syndrome,” 235 timed up and go test, 345 intrathecal baclofen, 361 Low-Sacral Transaction Model, 428 Kurtzke Functional Systems Scale, intrathecal phenol, 361 Low Sacral Transection Model, 420–421 11 neuromuscular blockade, 363 Lumbar and sacral nerve blocks, 105 negative effects of spasticity, 343 Ischemic SCI Model, 421–422 Lycra garments, 167 Isokinetic dynamometers, 318

444 index Multiple sclerosis (cont.) Neurotransmitters, 34, 126, 203, 230, 239, Patient Spasticity Scale, 349 Newcastle Independence Assessment 323, 326, 331 Patient with spasticity, evaluation of Form, 11 pathophysiology, 342–343 GABAergic, 349 examination, 272–274 prevalence of spasticity in patients with, Newcastle Independence Assessment Form, history, 272 11 investigations, 274–275 treatment 11 worsening spasticity, 275 chemical denervation, 349–351 Nine-hole peg test, 52 Pediatric Evaluation of Disability Index, intrathecal medications, 351–352 NIPA protein, 285 oral medications, 347–349 N-methyl-D-aspartate (NMDA), 200, 203 401 prevention, 345–346 Nonneural stiffness, 21 Pediatric Evaluation of Disability Inventory rehabilitative strategies, 346–347 Norepinephrine (NE), 207 surgical interventions, 352 (PEDI), 64 types of, 342 Oral antispasticity medications, 213–220 Pediatric population Oral baclofen, 41 Multiple Sclerosis Spasticity Scale (MSSS- Oral medications, 34, 78, 324, 347–349, alcohol administration in, 112 88), 159 baclofen for, 379 361, 390 chemoneurolysis in, 113 Muscle and adductor tone, 96 ITB complications in, 330 cooling, 346 benefit of, 200 ordinal rating assessment tool for, 82–83 overactivity, 17, 245 in pain management, 267 spasticity in, 112–113 recession, 407 and physical therapy, 306 Pedobarography, 60 tone, 58 Oromandibular dystonia, 37 Pendulum test, 58–59, 318, 374 tonus, 21 Orphenadrine, 212, 221 Penn spasm frequency ordinal rank scale, 83 weakness, 17 Orthopedic surgery Penn Spasm Frequency Scale (PSFS), 159, adverse events, 408–411 Muscle contractures, 25, 28, 82 evaluation, 405 317, 344–345, 345 and thixotropy, 368 indications and surgical planning, Perineal hygiene, 63 Peripheral nerve blocks, 330–331 Mutant mouse models, pathophysiology in 405–407 spastic, spasmotic and oscillator, outcomes, 411 mixed motor/sensory, 105, 106, 107 432–433 rationale, 405 Pernicious anemia, 308 surgical techniques, 407–408 Persistent inward currents (PICs), 421, Myelopathy, copper deficiency Orthotics, 81, 322, 347 overview, 309 and mobility devices, 388, 389 429 pathology, 309 Personal care integrity, 30 pathophysiology, 309 Pain, 34, 45, 75, 352 Pharmacodynamic tolerance, 237–238 treatment, 309–310 botulinum toxin injections for, 35 Phasic behavior, 21 in CP, 36 Phenol, 102, 350, 384, 392 MyoBloc, 133 oral medications, 34 treatment plans for, 35–36 administration in pediatric Nabilone, 210–211 types of, 35 population, 112 National Institutes of Health (NIH), 204 Nephrotic syndrome, 309 Pain management chemodenervation, 111 Nerve root entrapment, 36 in surgery after spasticity, 267 and ethyl alcohol, 392 Neurodevelopmental training (NDT), 155 Phenol neurolysis, 95, 106 Neuroimaging technologies Paralysis and BT, 109–111 prevalence of, in the United States, 6 duration of action of, 106 body weight-supported treadmill training histologic changes, 102–103 (BWSTT), 192–194 Paraneoplastic syndrome, 275, 304 hypothetical cost comparisons, 111–112 Paraplegin, 288 side effects and complications of, constraint-induced movement therapy, Parasomnias, 41 186–187 Paresis, 5 106–108 Parkinson disease, 39, 282 technique of injection, 103–104 diffusion imaging, 184–185 Parkinsonism, 273 electrical stimulation, 189–190 Passive function, 85 site of injection, 104–106 functional magnetic resonance Passive ROM (PROM), 81, 82 uses of, 97, 114 Physical and occupational therapies imaging, 185–186 torque-controlled, 373 combination therapies, 174 functional near infrared spectroscopy Passive tissue stiffness, 25 Pathogenesis of spasticity, 299 botulinum toxin type A injection and (fNIRS), 186 Pathophysiology of spasticity CIMT, 176–177 magnetic resonance spectroscopy (MRS), overview, 358 botulinum toxin type A injection and 185 Patient Disability and Caregiver electrical modalities, 174–176 robot therapy, 190–192 transcranial magnetic stimulation, Burden Rating Scale, 136 botulinum toxin type A injection and Patient Reported Impact of Spasticity Mea- SC, 177–179 188–189 virtual reality, 187–188 sure (PRISM), 159–160, 317 botulinum toxin type A injection and Neurologic syndromes, 289 therapeutic exercise, 177 Neuromuscular disorders, 297 evaluation, 156–157 factors to consider, 155–156 functional assessments, 160–161

index 445 modalities Protrudin (ZFYVE27), 287 SATISPART stroke, 84 cold, 172–174 PSFS. See Penn Spasm Frequency Scale (PSFS) SC. See Serial casting (SC) electrical stimulation, 168 Pump malfunction, 238. See also SCI. See Spinal cord injury (SCI) functional ES, 168–170 SCI-induced spasticity (SCIID), 420 heat, 174 Intrathecal baclofen (ITB) SCI-SET, 319–320 thermal, 172 Pusher syndrome, 163 SDR. See Selective dorsal rhizotomy (SDR) transcutaneous electrical nerve stimula- Sedation, 33 tion, 170–172 QOL. See Quality of life (QOL) Quality of life (QOL), 45, 52 in CP, 390 patient reported assessments, 159–160 due to baclofen, 302, 324 physical assessments, 157–159 measures, 53 due to CNS depressant medications, 204, therapeutic exercise Quality of speech, 39 Quality of Upper Extremity Skills Test, 177 205 body weight–supported due to diazepam, 324, 325, 348 ambulation, 168 Range of motion (ROM), 58, 82, 316, 400 due to oral antispasticity agents, active, 127, 343 strength training, 168 Beckman exercises for, 37 111 unloaded cycling, 167–168 chemodenervation and, 92 due to tizanidine, 209, 348 treatment options dorsiflexion, 122 intravenous, 361 dynamic splinting, 166 exercises, 25 during ITB trials, 233, 330 dynasplints, 166–167 improving, 101 in MS, 350 lycra garments, 167 ITB and, 232–233 risks and benefits of, 112 saeboflex, 166 loss of, 75–76 during surgery, 245 serial casting, 164–165 passive, 75, 82, 121, 127, 139, 343 Seipin (BSCL2), 286–287 stretching, 161–164 shoulder assessment and, 72 Seizures, 204 Physical pressure and injury, 30 sleep relaxation and, 42 tonic-clonic, 207 Physical therapy (PT), 245, 259, 389 and stretching, 346 Selective dorsal rhizotomy (SDR) BTX-A injections and, 177 adverse events, 399–400 exercise and, 303 Rankin Scale, 6 indications for surgical treatment of interventions, 169 Receptor expression enhancing protein 1 and oral medications, 306 spasticity, 398 postoperative, 260 (REEP1), 287 outcomes of surgical treatment of spastic- poststroke, 170 Reciprocal Ia inhibition, 314 program post-SDR, 398 Reciprocal inhibition, 368 ity, 400–401 SDR combined with, 401 Reflex sympathetic dystrophy, 35 rationale, 397 Physician Global Assessment Scale Score, 84 Rehabilitation after surgery for spasticity, techniques, 398–399 Physician Rating Scale, 176 Self-reported assessments of spasticity, 158 Plasticity, 389 role of Serial casting (SC), 78, 124, 164–165, 245, PLS. See Primary lateral sclerosis (PLS) mobilization, 267–268 Polyglucosan body disease, 273 nutrition, 268 323, 347, 373 Polysynaptic excitatory postsynaptic poten- pain management, 267 Serotonergic agents skin integrity, 268 tials (pEPSPs), 428–429 Relaxation index, 59 pharmacokinetics, 209–210 Polysynaptic reflexes, 26 Renshaw cells, 349 treatment, 210 Postlumbar puncture syndrome, 233 Renshaw system, 24 Serotonin syndrome, 204, 209 Postpolio syndrome, 6 Restless leg syndrome (RLS), 42 Sexuality, 33 Posturography, 62 Rett syndrome, 236 Shaping, 187 Prader-Willi/Angelman syndrome, 285 Riluzole, 300 Sherrington’s “dorsal root” strategy, 21 Preambulatory children, 389 Rivermead Motor Assessment Scale, 83–84 Sherrington’s seminal studies, 20 Pregnancy Robot therapy, 190–192 36-Item Short Form [SF-36], 52 Rodent models Shoulder, 72–73 spinal epidural venous engorgement dur- of cerebral palsy, 422 adduction and internal rotation spasticity, ing, 273 pathophysiology in, 432 143–144, 254 Primary lateral sclerosis (PLS) of multiple sclerosis, 422 adductor tone, 73 epidemiology, 304 anatomy, 141–143 overview, 303–304 pathophysiology in, 432 bone and joint integrity, 30 pathology, 304 ROM. See Range of motion (ROM) hyperextension, 144 pathophysiology of spasticity, 304 Rotor test, 238 spastic postures, 143–144 spasticity, 304 Russell viper venom test, 277 subluxation, 144, 190 treatment, 304 Shoulder assessment Progabide, 349 Sacral and lumbar nerve blocks, 105 and range of motion (ROM), 72, 191 drowsiness due to, 349 Saeboflex, 166 Sickness Impact Profile, 45 Proprioceptive afferent activity, 23 Saebo splints, 166 Silver syndrome, 282, 285, 286 Prosody, 37 Sarcomere, 28–29 Single voxel spectroscopy, 185 Satisfaction With Life Scale (SWLS), 65 Six6-Minute Walk test, 86 Sjögren syndrome, 273, 304

446 index Skin integrity, 30 Spastic hypertonia, 8 passive and active function, 53 in surgery for spasticity, 268 Spasticity pathophysiology, 314 Pediatric Evaluation of Disability Inven- Sleep apnea 4-aminopyridine (Fampridine®), in tetraplegic patients, 41 328 tory (PEDI), 64 pedobarography, 60 Sleep disorders, 40 baclofen (Lioresal®), 323–325 pendulum test, 58–59 bed positioning programs, 42 balance, 61–62 performance-based measures, 60 classification of, 41 benzodiazepines, 325 peripheral nerve blocks, 330–331 insomnia, 41 body segment analysis, 62 pharmacologic interventions, 323 botulinum toxin injections, 331–333 quality of life measures, 53, 64 Sleep relaxation cannabinoids (Marinol®, range of motion, 58 and range of motion (ROM), 41 reflex threshold angle, 59 Cesamet®), 328 Satisfaction With Life Scale (SWLS), 65 SNAP-25, 132 chemoneurolysis, 330 serial casting, 323 SNARE proteins, 132 choice of outcome measures, 53–54 stiffness and muscle tone, 58 Soft tissue contracture, 76 clinical assessment of, 86–87 stretch and stretch reflexes, 58 Soft tissue integrity, 30 clinical measures surgical interventions, 333–334 SPASM. See Support Programme for As- Tardieu Scale, 57–58 Ashworth Scale and Modified Ash- tendon reflex, 56 sembly of Database for Spasticity worth Scale, 316–317 Timed Ambulation tests, 63 Measurement (SPASM) Tizanidine (Zanaflex®), 326 Spasm Frequency Scale, 317, 344–345 biomechanical techniques, 318 treatment. See Spasticity treatment Spastic, Spasmotic, and Oscillator electrophysiologic measurements, 318, Visual Analog Scale and Likert Scale, Mutant Mouse Models, 422–423 Spastic dysarthria, 37 320–321 62–63 Spastic dystonia, 25. See also Dystonia Patient Reported Impact of Spasticity Spasticity and problematic spasticity in the Spastic elbow flexion contracture, 248 Spastic hemiplegia, 13 Measure, 317 United States, prevalence of, 10 Spastic hypertonia, 199–200 Spasm Frequency Scales, 317 Spasticity Clinic Initial Evaluation, 201 benzodiazepine derivatives Spinal Cord Assessment Tool, 317 Spasticity treatment, 321–322 pharmacokinetics, 204 Spinal Cord Injury Spasticity Evalua- treatment, 204–205 consequences of, 358–359 cannabinoids tion Tool (SCI-SET), 317 intrathecal treatments pharmacokinetics, 210 Tardieu Scale, 318 treatment, 210–211 clonidine (Catapres®), 326–327 chemodenervation, 361–363 central alpha-adrenergic agents cyproheptadine (Periactin®), 327 intrathecal baclofen, 361 clonidine, 207–208 dantrolene sodium (Dantrium®), intrathecal phenol, 361 tizanidine, 208–209 neuromuscular blockade, 363 cyclobenzaprine 325–326 oral medication, 361 pharmacokinetics, 212 defined, 3–4, 51, 357 outcome measures, 363, 365 treatment, 212 definition and scope of problem, principles of dantrolene sodium aims of management, 359–360 pharmacokinetics, 211 313–314 management, 361 treatment, 211–212 EuroQol, 65 medical, 361 gabapentin factors aggravating, 276 patient assessment, 360–361 pharmacokinetics, 205 functional performance measures, Spastic joints, 77 treatment, 205–206 Spastic postures gamma-aminobutyric acid (GABA) ago- 63–64 clenched fist, 148 gabapentin (Neurontin®), 327–328 Spastic reactivity, 83 nists, 200, 203 gait analysis, 61 Spastic stretch reflexes, 20 pharmacokinetics, 203 intrathecal baclofen, 328–330 Spastic syndrome, 423 treatment, 203–204 isolated voluntary time movement tests, Spastic tetraplegia, 13 orphenadrine, 212, 221 Spastin (SPAST), 284–285 serotonergic agents 59–60 Spastizin, 288 pharmacokinetics, 209–210 36-item short form health survey, Speech, quality of, 39 treatment, 210 SPG10, 285–286 tiagabine 64–65 SPG21, 282 pharmacokinetic, 206 Lance’s definition, 357 Spinal activity and spasticity treatment, 206 measures, 316 physiological changes in, 427–432 topiramate Spinal Cord Assessment Tool, 317 pharmacokinetics, 207 of passive activity, 53, 56 for spastic reflexes, 318 treatment, 207 utilizing nerve conductions, 54 Spinal cord injury (SCI) vigabatrin of voluntary activity, 53 common cause of, 8 pharmacokinetic, 206 modalities, 322 models of spasticity, 419–422 treatment, 207 Modified Ashworth Scales (MASs), 56–57 motor point blocks, 331 movement detection, 60–61 nonpharmacologic interventions, 322 nontraditional interventions, 323 normal motor control, 314–316 orthotics, 322–323 outcome measures, 51–52, 55

index 447 pathophysiology of animal models arising Surgical lengthening techniques, 250–251 confounding causes and assessment, from, 423–427 Surgical techniques for treatment of spastic- 372 prevalence in United States, 8 ity, 250–253 disability rating scale, 10 prevalence of spasticity in patients with, Swallowing, 37 epidemiology, 371 Synaptobrevin, 132 functional independence measure scores, 8–9 SynchroMed-EL®, 329 sports-related, 8 SynchroMed-II®, 329 10 Spinal Cord Injury Spasticity injection guided therapy Tardeau test, 121 Evaluation Tool (SCI-SET), for pediatric population, 121 botulinum toxin, 384–385 159 phenol, 384 Spinal disorders versus cerebral Tardieu Scale, 53, 57–58, 178, 318, 344 insomnia after, 48 disorders, 221 for pediatric population, 82–83 pathophysiology and unique aspects of, Spinal headache, 233 Spinal reflexes, 366–367 TBI. See Traumatic brain injury (TBI) 372 Spinobulbar spasticity, isolated syndrome of Tendon jerk reflex, 20 postacute and pharmacologic progressive, 303 Tendon transfers, 265 Splinting, 78, 85, 389 Ten10-meter walk test (TUG), 63, 88, 160, management dynamic, 166 baclofen, 375 extension, 258 161, 169, 343 dantrolene, 381 and hand hygiene, 138 Test-Retest reliability, 81–82 enteral baclofen, 375, 379 in Lycra garments, 167 Tetraplegic patients gabapentin, 380–381 passive, 258 intrathecal baclofen, 379–380 serial, 361 sleep apnea in, 41 tizanidine, 374–375 and serial casting, 373. See also Serial Tetrapyramidal syndrome, 303 valium, 381 Thalamic pain, 35. See also Pain prevalence of spasticity after, 10–11 casting (SC) Therapeutic exercise Troyer syndrome, 288, 289 Static stretch reflex, 23 Steopenia, 407 body weight–supported ambulation, 168 UMNS. See Upper motor neuron Stiff knee, 124–125 strength training, 168 syndrome (UMNS) Stiff knee syndrome, 84 unloaded cycling, 167–168 Stiff person syndrome, 272 Thermal modalities, 172 UMNS-related dysfunction, patterns of Strengthening, 346 Thigh, 363 lower limb, 87 Strength training, 168 adducted (scissoring), 125–126 Stress relaxation, 164 muscle, 149 Unloaded cycling, 167–168 Stretching, 36, 161–164, 389 Thixotropy and muscle contractures, 368 Upper extremity assessment, 71–72 Threonine, 349 and range of motion (ROM), 346 Thumb curling, 149 anatomic considerations, 72 Stretch reflexes, 20–22 Thumb-in-palm deformity, 258 assessment of elbow, 73–74 Thumb spasticity (intrinsic spasticity), assessment of fingers, 75 phasic, 22 assessment of shoulder, 72–73 tonic, 22–24 258–259 assessment of wrist, 74 Stretch sensitive cocontraction, 24 Tiagabine Stretch sensitive dystonia, 24–25 follow-up assessments, 78–79 Stroke, 6 pharmacokinetic, 206 functional considerations ITB in, 45 treatment, 206 in nonspastic patients, 7 Tilt table, 162–163, 322 agonist/antagonist cocontraction, 75 prevalence of spasticity after, 8 Timed Ambulation Tests, 63 dystonic posturing, 77 Stroking, 346 Timed Up and Go (TUG) test, 86, 161, 345 joints, 76–77 Strumpellin (KIAA0196), 285 Tizanidine, 208–209, 348, 374–375 orthopedic complications, 76 Support Programme for Assembly of Da- Tizanidine (Zanaflex®), 326 soft tissue contracture, 76 Toe flexor spasticity, 266–267 weakness, 77 tabase for Spasticity Measurement Tone Assessment Scale (TAS), 7 goal setting, 71–72 (SPASM), 357–358 Tonic stretch reflexes, 21–22, 120 treatment plan development, 77–78 principles, 358 Topiramate Upper Extremity Function test, 169 Supraspinal lesions, 315, 366 pharmacokinetics, 207 Upper limb spasticity, 199–200 Surgery for spasticity treatment, 207 Upper motor neuron disorders future considerations, 268 Torque, 58 examples and goals of, 244–246 velocity-dependent ankle, 428 recommendations, 113 Surgery management of specific Transcranial magnetic stimulation, 188–189 lower body, 115 conditions, 253–254 Transcutaneous electrical nerve stimulation upper body, 113–115 Surgical interventions in spasticity Upper motor neuron syndrome (UMNS), appropriate timing for, 246–247 (TENS), 170–172, 347 goals of, 244–246 Traumatic Brain Injury Model Systems, 10 5, 17 history of, 243–244 Traumatic brain injury (TBI), 9, 56, 84, available support system, 94 planning, 247–250 bowel and bladder, 39–40 119 acute management of post-TBI spasticity, basic considerations in, programs, 42 case discussions, 96–98 372–374 case study, 45 causes of spasticity, 271–272

448 index Upper motor neuron syndrome (UMNS) stretch sensitive dystonia, 24–25 Visual Analog Scale (VAS), 36, 38–39, 62–63 (cont.) tonic stretch reflexes, 22–24 Vitamin B12 deficiency treatment modalities, 95 clinical expression of motor dysfunction voice, speech, and swallowing, 37 epidemiology, 308 in, 81 Upper motor neuron (UMN) bowel, 40 overview, 307–308 Upper motor neuron (UMN) lesion, 17, 82 pathology, 308 clinical features of, 81 development of contractures in patients pathophysiology, 308 determining treatment goals, 95–96 treatment, 308–309 dysarthrias, 37, 39 with, 28, 368 Voice, speech, and swallowing, 37 dysphagia, 37 flexor reflex and, 26 Voluntary capacity, 83 financial resources, 95 flexor synergy patterns caused by, 350 maladaptive consequences, 29–30 incidence of spasticity and, 5 Wallerian degeneration, 300 negative signs, 18–19 influence of, 18 Wartenberg pendulum test, 363 pain in, 34 limb posture and movement after, 360 Weakness loss of inhibition after, 120 botulinum toxin injections for, 35 nerve conduction and, 277 after surgery, 366 oral medications, 34 pathophysiology, 365 and atrophy, 297 treatment plans for, 36 phasic stretch reflex after, 366–367 BoNT dosing and, 132 types of, 35 rheologic change after, 82 and botulinum toxin A, 303 patient and clinical presentation, 92–94 stretch reflexes and, 20, 24 in dantrium, 381 anatomic distribution, 92–93 Upper motor neuron (UMN) due to dantrolene, 211 benefits of patients, 94 due to pain in CP, 36 cognitive status, 93 syndrome, 7 of hip abductors, 126 concurrent medical problems, 93 Upper motor neuron (UMN) in HSP, 282, 287–288 functional and overall prognosis, 93 muscle, 17, 127, 135, 204, 209, 346, 411 manifestation of the upper motor syndrome, pathophysiology of neuromuscular disorders and, 305 features of, 365–366 and PLS, 303 neuron syndrome, 94 associated reactions and mass reflex, 368 predominated in dantrium, 381 residual function, 93–94 clasp-knife response, 367–368 in UMN distribution, 271, 273 response to previous treatment efforts, disordered control of movement, 368 in UMNS, 18, 75, 77 flexor spasms, 368 Wee-Functional Independence Measure, 401 94 hypertonia, 367 Weight, 45 spasticity etiology, 92 spinal reflexes, 366–367 Weight drop method, 421 time since onset, 92 thixotropy and muscle contractures, 368 West syndrome, 207 positive signs that are not stretch-sensi- Urinary tract infection, 5, 37, 40, 155, 237, Wolf Motor Function Test, 161 321 Women tive depressive symptoms in, 45 associated reaction, 26–27 Valgus foot spasticity, 265 incidence of pernicious anemia, 308 flexor and extensor spasms, 26 Valium, 381 multiparous, 273 muscle rheology, 27–29 Varus foot spasticity, 264–265 prevalence of multiple sclerosis in, 11 psychosocial issues Velocity-dependent ankle torques, 428 prevalence of stroke in, 6 depression, 43 Velocity sensitivity, 20 spinal headaches in, 233 mood issues, 42 VGAT labeling, 427 Work-related injuries realistic treatment goals, 91–92 Vibratory Inhibitory Index, 54 in adults, 8 sleep disorders, 41 Vibratory inhibitory reflex, 52–53 Wrist, 74, 146–147 bed positioning programs, 42 Vigabatrin flexion spasticity, 256 classification of, 41 Wrist flexors insomnia, 41 pharmacokinetic, 206 in adults, 23 stretch sensitive positive signs treatment, 207 phasic stretch reflexes and clonus, 22 Virtual reality, 187–188 stretch reflexes, 20–22 stretch sensitive cocontraction, 24

Spasticity Diagnosis and Management Allison Brashear, MD and Elie Elovic, MD Spasticity: Diagnosis and Management is the first book solely dedicated to the diagnosis and treatment of spasticity. This pioneering work defines spasticity in the broad context of Upper Motor Neuron Syndrome (UMNS) and focuses not on a single component, but on the entire constellation of conditions that make up the UMNS and often lead to disability. A one-stop resource for physicians, therapists, and other members of the spasticity management team tasked with the goal of improving patient care and outcomes Spasticity: Diagnosis and Management clearly defines the process for the diagnosis of spasticity, the basic science behind its pathophysiology, the measurement tools used for evaluation, and the available treatment options. Divided into five sections, this comprehensive clinical text provides a roadmap for assessing the complicated picture of spasticity and choosing the appropriate interventions. Therapies including oral medications, intrathecal baclofen, chemodenervation with botulinum toxin and phenol, and surgical options are thoroughly discussed, as are non-medical therapies and the role of the emerging technologies. Experienced clinicians comprehensively review the full spectrum of diseases involving spasticity in adults and children and the unique diagnostic and management challenges they present. Special Features of Spasticity: Diagnosis and Management Include w In-depth coverage of diagnoses, interventions, and outcomes across multiple pathologies w Tools and clinical measurements for patient assessment w Treatment-focused chapters outlining current medical and other therapeutic options w Illustrated review of limb anatomy w H ands-on guidance to chemodenervation techniques with botulinum toxin and phenol, and ITB management w Disease-based chapters devoted to the full range of clinical conditions involving spasticity in adults and children w Multidisciplinary perspective supporting a team approach to care About the Editors Recommended Shelving Classification Allison Brashear, MD, Professor and Chair, Department of Neurology, Wake Forest University School of Medicine, Wake Forest Baptist Medical Neurology/Physical Center, Winston-Salem, North Carolina Medicine and Rehabilitation Elie Elovic, MD, Professor and Chief, Division of Physical Medicine and Rehabilitation, University of Utah School of Medicine, Salt Lake City, Utah 11 W. 42nd Street 9 781933 864518 New York, NY 10036 www.demosmedpub.com