Anil Israni

Finger drop sign: Rare presentation of a common disorder.

BACKGROUND Guillain Barre syndrome (GBS) commonly presents with limb weakness and occasional cranial nerve, respiratory or autonomic involvement. Isolated or predominant bilateral finger drop as presenting feature has never been reported in the pediatric age group. CASE A 9-year-old boy presented with deformity of both hands for 7 days and leg pain with difficulty in getting up from floor for 3 days. On examination he had bilateral clawing with subtle hip flexor weakness and hyporeflexia. His nerve conduction study revealed motor axonal neuropathy. His serum lead levels and autoimmune markers were within normal limits. His cerebrospinal fluid examination revealed albuminocytological dissociation. He was diagnosed as GBS and was given intravenous immunoglobulin. He improved completely over next 8 weeks. CONCLUSIONS GBS is one of the commonest causes of acquired neuropathy in the tropics. In resource limited setting, where electrophysiological facilities may not be available, identification of finger drop sign may help in correct management.

Tubercular meningitis in children: Clinical, pathological, and radiological profile and factors associated with mortality

Context: Childhood tuberculosis is a major public health problem in developing countries with tubercular meningitis being a serious complication with high mortality and morbidity. Aim: To study the clinicopathological as well as radiological profile of childhood tuberculous meningitis (TBM) cases. Settings and Design: Prospective, observational study including children <14 years of age with TBM admitted in a tertiary care hospital from Western India. Subjects and Methods: TBM was diagnosed based on predefined criteria. Glassgow coma scale (GCS) and intracranial pressure (ICP) was recorded. Staging was done as per British Medical Council Staging System. Mantoux test, chest X-ray, cerebrospinal fluid (CSF) examination, neuroimaging, and other investigations were done to confirm TB. Statistical Analysis Used: STATA software (version 9.0) was used for data analysis. Various risk factors were determined using Chi-square tests, and a P< 0.05 was considered significant. Results: Forty-seven children were included, of which 11 (24.3%) died. Fever was the most common presenting symptom, and meningismus was the most common sign. Twenty-nine (62%) children presented with Stage III disease. Stage III disease, low GCS, and raised ICP were predictors of mortality. Findings on neuroimaging or CSF examination did not predict mortality. Conclusions: Childhood TBM presents with nonspecific clinical features. Stage III disease, low GCS, lack of Bacillus Calmette–Gu͹rin vaccination at birth and raised ICP seem to the most important adverse prognostic factors.

Recurrent bacterial meningitis—devil is in the details

Dear Sir, A 6-year-old girl presented with complaints of fever and vomiting for 2 days, headache for 1 day, and one episode of generalized tonic-clonic seizure followed by altered sensorium for the last 12 h. Her past history was remarkable for similar complaints with three such episodes in the last 3 years. CSF (cerebrospinal fluid) examination in these episodes was consistent with bacterial meningitis; pneumococcus was isolated in the CSF specimen in one of the episodes. She was treated with IVantibiotics adequately in each episode; the last episode being 6 months back. There was no history of recurrent multisite infections. There was no history of intake of immunosuppressive drugs. In the current admission, she had signs of meningeal irritation with marked neck rigidity and positive Kernig’s sign. Her fundus examination was normal. There were no cranial nerve deficits and her motor system examination revealed no deficits. CSF examination showed neutrophilic pleocytosis (140 cells/mm; 70% polymorphs), raised protein (138 mg/dl) and hypoglycorrhachia (39 mg/dl; corresponding blood glucose 82 mg/dl); Gram stain and bacterial culture were negative. Her blood culture did not isolate any pathogen. She was managed with broad-spectrum IV antibiotics and other supportive measures. In view of recurrent meningitis, clinical history and medical records were reviewed. Repeated questioning revealed a history of frequent clear discharge from right nostril and on further probing there was a history of trauma 3 years ago. She had a fall from height (first floor) followed by loss of consciousness, but there was no history of seizure or bleeding from nose, ear, or mouth. She was taken to an orthopedic surgeon to exclude any limb or cervical spine fracture, but no further records were available. During subsequent hospitalizations with meningitis, immunodeficiencies (primary and acquired) were ruled out with appropriate investigations. In the current presentation, in light of history of trauma and multiple episodes of bacterial meningitis, an HR (high resolution) CT (computed tomography) scan with 3D bony reconstruction of skull base was done; this showed a defect in the cribriform plate on the right side (Image 1a). CT cisternogram confirmed extravasation of contrast into ethmoid air cells through a focal defect at the cribriform plate (Image 1b). Trans-nasal endoscopic repair of the anterior skull base defect was done using by underlay technique. Postoperatively there was no CSF rhinorrhea and the child remains asymptomatic for the past 2 years on follow-up. There is no consensus definition of recurrent bacterial meningitis. It is generally accepted as two or more episodes of meningitis caused by a different bacterial organism or, alternatively, a second or further episode caused by the same organism with more than 3 weeks interval after the completion of therapy for the initial episode [1]. The exact incidence of recurrent bacterial meningitis in the pediatric population is not known, but Drummond et al. [2] in their single-center experience reported that only 1.3% of children admitted with meningitis had at least one previous episode of bacterial meningitis. Both congenital and acquired conditions can lead to recurrent bacterial meningitis in children. They can further be * Anirban Mandal anirban.nrs@gmail.com

De Novo Robertsonian Translocation t(21; 21) in a Child with Down Syndrome

The phenotypic expression in DS is determined by the type of underlying cytogenetic abnormality. Almost 90-95% cases of DS are due to pure trisomy of the 21st chromosome; 6-7% is the result of mosaicism and in only 3-5% of cases it results from Robertsonian translocation (ROB). About 1/3rd cases of unbalanced Robertsonian translocation causing DS are inherited. We report a 1-year-old-boy with DS secondary to rea(21;21) ROB.

Partial oculomotor nerve palsy in a 7-year-old child.

Oculomotor nerve palsy can be due to varied causes that include diabetic neuropathy, myasthenia gravis, brainstem infarction, demyelinating conditions, and cerebral aneurysms. Among the aneurysmal causes of oculomotor nerve palsy, aneurysm of the posterior communicating artery has been observed to be the most common. Pupillary dysfunction is considered to be an important feature of aneurysmal oculomotor nerve paresis. A case of a 7-year-old boy with partial oculomotor nerve palsy with pupillary sparing is being reported here, the etiology of which is tortuous and ectatic distal internal carotid artery. This is a rare cause of oculomotor nerve paresis and to the best of our knowledge has not yet been reported in children. Ischemia rather than compression seems to be the most plausible cause in this case.