Paediatric Bickerstaff brainstem encephalitis: A rare case report

Bickerstaff Brainstem Encephalitis, a rare variant of Guillain- Barre Syndrome has an estimated prevalence of eight per 100 million individuals. It presents with the classic triad of ataxia, ophthalmoplegia and altered consciousness. We report the case of a 10-year-old child who presented with low grade fever, hypersomnia and inability to walk. Central nervous system examination revealed ophthalmoplegia and multiple cranial nerve palsies. However, CSF examination showed lack of albuminocytological dissociation with no previous history of respiratory or gastrointestinal tract infection. Unavailability of Anti-GQ1b antibodies led to a diagnosis based on suggestive clinical features, abnormal MRI signals and prompt response to corticosteroid administration. Intravenous Methyl Prednisolone in a dose of 30mg/kg/day was administered for 10 days followed by oral Prednisolone 2 mg/kg/day. After complete recovery the patient was discharged, Prednisolone was tapered gradually and eventually discontinued after four months.