Introduction: Autoimmune encephalitis is a spectrum of disorders manifested by epileptiform encephalopathy, neuropsychiatric manifestations and extrapyramidal features. Anti NMDA receptor and Basal ganglia encephalitis are two such entities encountered in the pediatric age group.¹

Material and Methods: Fourteen cases of proven autoimmune encephalitis (upto 18 years of age) presented to a tertiary care hospital in north India between January 2010 and August 2013.

Results: In the 11 patients with anti NMDA receptor encephalitis (average age 9 years, male female ratio 1:1.2), the common modes of presentation were  progressive extrapyramidal syndrome and epileptiform encephalopathy. Sixty four percentage ( 7/11) showed significant response to combination therapy of steroids and intravenous immunoglobulin. The 3 cases with basal ganglia encephalitis (all males, aged 8, 6 and 10 years) presented with acute onset parkinsonian features, chronic progressive generalized dystonia and acute onset motor tics respectively. The first 2 cases had abnormal brain MRI in the form of bilateral striatal signal changes with additional cerebellar and occipital changes in the first, the third patient’s MRI was normal. The first and third case showed near complete response to steroids and intravenous immunoglobulin. The second case was already 2 years into the illness and had irreversible bilateral striatal cystic changes on MRI with no significant response to immunotherapy.

Conclusion: In upto two-third patients with encephalitis like presentation, a specific infectious etiology is not found.² Autoimmune encephalitis should always be considered if there are specific clinical pointers in view of possible favorable response to early immunotherapy.