Pseudotumor cerebri in 11 months old child – A case report with review of literature

Pseudotumor cerebri ISSN: 2394-0026 (P)Case Report ISSN: 2394-0034 (O)Pseudotumor cerebri in 11 months old child – A case report with review of literature Menon Narayanankutty Sunilkumar1*1Associate Professor, Department of Pediatrics, Amala Institute of Medical Sciences, Kerala, India *Corresponding author email: [email protected] to cite this article: Menon Narayanankutty Sunilkumar. Pseudotumor cerebri in 11 months oldchild – A case report with review of literature. IAIM, 2015; 2(1): 103-107. Available online at www.iaimjournal.comReceived on: 12-12-2014 Accepted on: 17-12-2014AbstractPseudotumor cerebri is defined as a clinical entity where there are signs and symptoms of raisedintracranial pressure but the higher mental and neurological functions are not altered. Many timesthe etiology is unknown. A thorough history and clinical examination is necessary along withexclusion of possible structural causes of intracranial hypertension. Headache, papilledema andvisual disturbances are the characteristic symptoms in a child but in a younger infant it becomes allthe more difficult to arrive at diagnose. The prognosis is excellent if the offending drug or cause isidentified and abated. This case study reported a case of Pseudotumor cerebri (PTC) in 11 monthsold child.Key wordsPseudotumor cerebri, Benign intracranial hypertension, Papilledema, Nalidixic acid.Introduction or infrequent visual disturbances and often experience intracranial noises. The diagnosis inBenign intracranial hypertension (ICH) or younger infant is challenging as they may onlyPseudotumor cerebri (PTC) is a syndrome have irritability or apathy [2]. Idiopathiccharacterised by high cerebrospinal fluid intracranial hypertension without papilledema ispressure without any underlying structural or well-described in adults [3] and is rarelysystemic cause [1]. The presenting symptoms of reported in the pediatric population [4]. PTC inidiopathic ICH are known to vary with age and 11 months old child without papilledema isthere are assertive reports of this entity in the highlighted in this case report.pediatric population. The older children usuallyhave associated headache, neck pain, diplopia,International Archives of Integrated Medicine, Vol. 2, Issue 1, January, 2015. Page 103Copy right © 2015, IAIM, All Rights Reserved.

Pseudotumor cerebri ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)Case report was vomiting. Breast feeding, oral feeds and fluids started subsequently. He did not have any11 months old male baby was seen in the neurological deficits and his fundus examinationcasualty late night with symptoms of lethargy, was normal and there was no papilledema. Herecurrent episodes of non projectile vomiting, had received Nalidixic acid (NA) suspension forabdominal pain, fowl smelling semi-loose stools the dysentery prior to the admission in ourwith blood streaks and mucous since the hospital. A diagnosis of PTC, secondary toprevious day. History revealed that the family Nalidixic acid, was considered, but decided tohad travelled for a pilgrimage and had food from rule out other causes of bulging anteriorhotels. He was treated for symptoms of the fontanel including meningitis. The laboratorydiarrhoeal episode and was on medications investigations revealed Hb (12 g/dl), PCVelsewhere. He became lethargic since the (33.7%), total count (9000/µl), neutrophilsprevious night with recurrent vomiting and had (48%), lymphocytes (46%), eosinophils (3.7%),low grade fever for the last 2 days. There was no monocytes (2.1%), basophils (0.2%), ESRassociated rash, ear discharge, seizures or any (31mm/hr), and platelets count (210000/µl)trauma. Birth history revealed that he was born were all found to be normal. Serum Na+ 133to 29 years old mother at 37 weeks gestation via mEq/l, K+ 4.4 mEq/l, Cl- 91 mEq/l, bicarbonatespontaneous vaginal delivery weighing 3.6 kg 26 mEq/l, glucose 144 mg/dl, total Ca++ 9.0with normal apgar score. His parents were of mg/dl and C Reactive protein was normal (<0.8non-consanguineously married. He was mg/dl). NA was not given further. He wasimmunized to date and had normal milestones started on oral Cefixime suspension 8of development. On general physical mg/kg/day in 2 divided doses along with Zincexamination, baby was tired, crying, at times sulphate solution 10 mg/day and oralwas very irritable. He had low grade fever rehydration solution. The baby’s clinical(99.20F), heart rate 102/minute, and respiratory condition was followed-up. The bulging anteriorrate 36/minute, weight 7.36 kg, anterior fontanel was present for 2 more days andfontanelle was bulging and prominent with became normal on the 4th day after admission.pulsations in the supine as well as in the sitting (Photo - 1C) The child was discharged withposition. (Photo - 1A, Photo - 1B) His oxygen advice for continuing breast feeding and tosaturation was 96%. All peripheral pulses were follow-up.equally felt. Blood pressure was 94/56 mm Hg inright upper limb. He was having moderate Discussiondehydration. His respiratory effort remainedgood and was very irritable at times PTC is a condition of ICH without localizing signsinconsolable even after breastfeeding. or neurological deficits except for papilledemaAbdominal examination did not reveal any mass and normal cerebrospinal fluid constituents within the abdomen and there was no normal intracranial structure [5]. The PTC mayhepatosplenomegaly. Cardiovascular system be primary PTC (idiopathic ICH) or arise from aexamination was normal. The child was detectable secondary cause. PTC in the primaryadmitted and his general condition improved group are generally associated with a normalwith symptomatic treatment given for fever with brain parenchyma and without ventriculoParacetamol drops and for vomiting with megaly, mass lesion, or underlying infection orDomperidone suspension. His dehydration was malignancy can also cause this type of PTC andcorrected with intravenous Ringer lactate as he also observed in adolescent obese children andInternational Archives of Integrated Medicine, Vol. 2, Issue 1, January, 2015. Page 104Copy right © 2015, IAIM, All Rights Reserved.

Pseudotumor cerebri ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)women [5]. The secondary PTC group causes are irritable child in the setting of PTC [2, 3]. Themultiple and includes causes such as cerebral diagnosis of PTC is based on high degree ofvenous abnormalities, cerebral venous sinus clinical suspicion but the tests that may be donethrombosis. Also a decreased CSF absorption include computed tomography (CT) scan of thefrom past intracranial infection or subarachnoid brain, ophthalmology examination, includinghemorrhage and endocrine disorders such as visual field testing, magnetic resonance imagingAddison disease, hypoparathyroidism, Cushing's (MRI) of the brain with MR venography anddisease and chronic kidney failure, anemia can Lumbar puncture for CSF analysis can be donecause PTC [2, 3]. There are many medications [1]. There are standard revised diagnosticwhich can cause PTC and they are birth control criteria such as modified Dandy criteria for thepills, cyclosporine, minocycline, NA, diagnosis of PTC as is mentioned in the literaturenitrofurantoin, phenytoin, sulfa drugs, review [5, 6]. According to these criteria, LPtamoxifen, tetracycline, vitamin A. Hormones must be included in the route to diagnose ofsuch as human growth hormone, thyroxine (in PTC. In this case, when the NA was withdrawn,children), leuprorelin acetate, levonorgestrel, the condition improved and thus ignored theanabolic steroids medical and starting or invasive procedure such as LP and other imagingwithdrawal from chronic corticosteroids can (e.g. CT, MRI). However, weight gain orcause PTC [6]. The pathogenesis of PTC is a exposures to a substance associated with thecascade involving cerebral edema, increased PTC are associated with relapse of PTC and therecerebral blood volume, and also decreased CSF will be recurrent papilledema [3].absorption [1, 7]. For ICH to occur there shouldbe a prolonged elevation of the intracranial In most patients with idiopathic PTC, withoutpressure, above 200 mm H2O. In most patients, papilledema, laboratory and neurologicit manifests as severe headache, papilledema, investigations are normal. The child in this casetransient visual disturbances, diplopia, tinnitus, had bulging anterior fontanel and signs of raisednausea, vomiting, and central nervous system ICH such as irritability and vomiting, but notand mental abnormalities, as well as associated with papilledema as mentioned indysfunctions of the cardio-respiratory systems other studies [3]. NA, a quinolone is still used in[7]. The presenting symptoms of benign ICH are the treatment of acute dysentery, and has beenknown to vary with age where by the older known to cause PTC in infants and youngchildren usually have associated headache, neck children. The recommended dose is 55pain, diplopia, or infrequent visual disturbances mg/kg/day in 3 divided doses [8]. In this case,and often experience intracranial noises. The the child reported received the drug anddiagnosis in younger infant is challenging as they developed PTC. Fortunately, no delay in medicalmay only have irritability or apathy [2, 7]. intervention excluded the possible serious side effects such as metabolic acidosis, associatedA good history taking regarding onset of the with the NA toxicity.signs and symptoms along with a thoroughclinical examination is necessary. It is pertinent The treatment of PTC involves initially theto look for signs of suspected raised ICH like removal and discontinuation of possiblebulging anterior fontanel in an infant, offending medication or hormonal preparation.neurological deficits, positive signs of meningeal The management for ICH is by medicalirritation and fundoscopy for not missing treatment, drug therapy, or surgicalpapilledema help clinch the diagnosis in an intervention. Fluid and salt restriction isInternational Archives of Integrated Medicine, Vol. 2, Issue 1, January, 2015. Page 105Copy right © 2015, IAIM, All Rights Reserved.

Pseudotumor cerebri ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)routinely advised. A combined therapy withacetazolamide and furosemide is recommended Referencesas an effective first-line method of treatingraised intracranial pressure in children with PTC 1. Moffat FL. Pseudotumor cerebri. Can J[9]. It is advised not to use corticosteroids as thefirst choice for treatment and the LP and diuretic Neurol Sci, 1978; 5: 431-6.therapy should precede its administration. An LPcan also help relieve CSF pressure in the brain 2. Weig SG. Asymptomatic idiopathicand prevent vision problems. intracranial hypertension in youngCSF shunting procedures namely(lumboperitoneal or ventriculoperitoneal shunt) children. J Child Neurol, 2002; 17: 239-may be employed, if medical treatment fails.Papilledema should not be missed. When 41.chronic papilledema threatens visual functionoptic nerve sheath decompression may also be 3. Marcelis J, Silberstein SD. Idiopathicdone to prevent vision loss [3]. Weight loss isadvised in adults and adolescents with obesity in intracranial hypertension withoutthe primary PTC. PTC disappears on its ownwithin 6 months in some subset of patients. It is papilledema. Arch Neurol, 1991; 48:found that even with immediate intervention,visual loss can occur [10]. Transient visual 392-9.abnormalities were very common in a subset ofpatients with absent papilledema in PTC [11] 4. Beri S, Gosalakkal JA, Hussain N, Balkyand so vision has to be monitored on follow-up.The child does not have any deficits. AP, Parepalli S. Idiopathic intracranialThis case study concluded that awareness of hypertension without papilledema.causes of PTC among the pediatricians andtreating physicians is very important. Even Pediatr Neurol, 2010; 42: 56-8.though there is a risk of vision in a minority ofcases, the prognosis in PTC is excellent. PTC has 5. Friedman DI, Liu GT, Digre KB. Revisedgood prognosis, if diagnosed early and correctlywith the simple non invasive tools such as diagnostic criteria for the pseudotumorproper history taking and clinical examination asthe diagnosis is mainly of exclusion of causes. cerebri syndrome in adults and children.Acknowledgement Neurology, 2013; 81(13): 1159-65.The author acknowledges the help of Dr Ajith 6. Liguori C, Romigi A, AlbaneseTA, Professor of Biochemistry, Amala Institute ofMedical Sciences, Amala Nagar, Thrissur, Kerala M, Marciani MG, Placidi F, Friedmanduring the preparation of the manuscript. D, Digre K, Liu G. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology, 2014; 82(19): 1752-3. 7. Kabat AG. Intracranial hypertension. Optom Clin, 1996; 5: 153-79. 8. Riyaz A, Aboobacker CM, Sreelatha PR. Nalidixic acid induced pseudotumour cerebri in children. J Indian Med Assoc, 1998; 96: 308-314. 9. Schoeman JF. Childhood pseudotumor cerebri: Clinical and intracranial pressure response to acetazolamide and furosemide treatment in a case series. J Child Neurol, 1994; 9: 130-4. 10. Kesler A, Gadoth N. Pseudotumor cerebri (PTC--an update). Harefuah, 2002; 141: 297-300, 312. 11. Amacher AL, Spence JD. Spectrum of benign intracranial hypertension in children and adolescents. Childs Nerv Syst, 1985; 1: 81-6.International Archives of Integrated Medicine, Vol. 2, Issue 1, January, 2015. Page 106Copy right © 2015, IAIM, All Rights Reserved.

Pseudotumor cerebri ISSN: 2394-0026 (P) ISSN: 2394-0034 (O)Photo – 1: Child with the bulging anterior fontanel in the A) supine; B) sitting position and C)Anterior fontanel returned to be normal on 3rd hospital day.Source of support: Nil Conflict of interest: None declared.International Archives of Integrated Medicine, Vol. 2, Issue 1, January, 2015. Page 107Copy right © 2015, IAIM, All Rights Reserved.