Extended clinical spectrum of Anti NMDA receptor encephalitis in children: A case series

Publication date: Available online 5 April 2017
Source:Pediatric Neurology
Author(s): Ajay Goenka, Vivek Jain, Hiroki Nariai, Alfred Spiro, Mitchell Steinschneider
ObjectiveTo emphasize the wide spectrum of clinical manifestations in children with Anti NMDAR encephalitis from two different health care settings.MethodsWe report our experience with thirteen patients (median age: 7 years, range: 5 months - 19 years) presenting to tertiary referral centers in India and USA.ResultsInitial manifestations were neurological (seizures or movement disorders) in 8 patients, and psychiatric (e.g. emotional lability, hallucination) in 5 patients. Symptoms during the clinical course included seizures in 10 patients, movement disorders (dyskinesia and choreiform movements) in 11 patients and behavioral changes (aggressiveness, insomnia) in 10 patients. Concomitant infections (HSV1, tuberculous meningitis and influenza A) were present in 3 patients. Analysis of the CSF in all but the two cases preceded by infection (HSV encephalitis and tuberculous meningitis) was unremarkable. Treatment included IVIG/Methylprednisolone (11 patients), Rituximab (8 patients), plasmapheresis (2 patients) and Cyclophosphamide (2 patients). Six patients recovered completely. Two had mild while 4 had severe residual neurological deficits. Two patients had profound autonomic instability, which was the cause of death for one of them. Two patients had a relapse at 2 months and 6 months after the initial recovery.ConclusionHerein we report on the differences and similarities of clinical presentation, test results, and response to treatment of pediatric patient with Anti NMDAR encephalitis from India and United States. Included is a description of one of the youngest patients with Anti NMDAR encephalitis (5 months) and the first case to be reported in association with tuberculous meningitis.



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