A bstract Introduction: Demyelinating disorders of central nervous system are rare childhood disorders that cause significant physical and cognitive disabilities. Early diagnosis and appropriate treatment determines prognosis and outcome. Objective: The objective of this work was to study clinical profile, investigative and radiological features, treatment, and outcome of children with demyelinating disorders. Materials and Methods: A retrospective chart review of 32 children with demyelinating disorders admitted in a tertiary-care pediatric hospital from Bangalore between November 2013 and October 2017 was conducted. Sociodemographic data, clinical features, cerebrospinal fluid (CSF) findings, radiological features, treatment received, and outcome were collected and results were analyzed. Results: Among 32 patients of demyelinating disorders, majority were acute disseminated encephalomyelitis (ADEM) (15, 46.9%), followed by transverse myelitis (9, 28.1%), multiple sclerosis (MS) (3, 9.4%), optic neuritis (3, 9.4%), and neuromyelitis optica (NMO) (2, 6.2%). Mean age of presentation was 7.8 years. Among 32 patients, 15 were male (47%). In patients with ADEM, the mean age of presentation was 5.5 years. Two patients had tumefactive ADEM and 10 recovered after steroid therapy. In patients with MS, the mean age of presentation was 10.6 years; one patient died and the condition of two patients improved. In patients with transverse myelitis, the mean age of presentation was 8.9 years and seven patients recovered. In a patient with NMO, CSF was positive for antibody to aquaporin-4. The child improved with steroids and intravenous immunoglobulin. Three patients with optic neuritis were treated with steroids and they recovered. Conclusion: Even though demyelinating disorders are rare in pediatric age group, one should be aware of this entity as early diagnosis and treatment improves outcome. Most common among them is ADEM.

Idiopathic intracranial hypertension is rare in children and associated with high cerebrospinal fluid pressure, with no evidence of intracranial malformation. The etiology is unknown but it is associated with endocrine disorders, drugs, toxins and viral infections. Investigations such as lumbar puncture and imaging are usually normal. We report a child who presented with features of elevated intracranial pressure following measles vaccination. His investigations were all found to be normal and was treated with acetazolamide following which he recovered.

We report a case of Kearns-Sayre syndrome in 10-year-old boy, who presented with epilepsia partialis continua. Laboratory investigations revealed an elevated creatine kinase, serum lactate and cerebrospinal fluid protein. The subject was treated with multiple antiepileptic medications and megavitamins and the seizures decreased over a period of 2 wk.

Agenesis of corpus callosum is a rare malformation of the brain. In Aicardi syndrome, it is associated with retinal lacunae and infantile spasms. Once Aicardi syndrome is diagnosed, one should look for other central nervous system malformations. We report a child who presented with developmental delay, chorioretinal lacunae and infantile spasms, due to Aicardi syndrome, associated with a type 2b interhemispheric cyst diagnosed on magnetic resonance imaging.