J Pediatr Neurol 2013; 11(01): 035-038
DOI: 10.3233/JPN-120595
Georg Thieme Verlag KG Stuttgart – New York

Anti-NMDA receptor encephalitis subacute cerebellitis remarkably responsive to rituximab

Shilpi Kumar
a  Department of Neurology, University of North Carolina, Chapel Hill, NC, USA
,
Zheng Fan
a  Department of Neurology, University of North Carolina, Chapel Hill, NC, USA
,
Robert S. Greenwood
a  Department of Neurology, University of North Carolina, Chapel Hill, NC, USA
› Author Affiliations

Subject Editor:
Further Information

Publication History

12 May 2012

18 June 2012

Publication Date:
30 July 2015 (online)

Abstract

We report a case of a child presenting with sub-acute cerebellar ataxia that was associated with anti- N-methyl-D- aspartate (NMDA) receptor antibodies who dramatically responded to early aggressive immunomodulating therapy but not to conventional therapy. A three-year-old boy with history of prematurity and speech delays presented with subacute onset ataxia followed by progression to choreoathetosis, dysphagia, oro-facial dyskinesia, insomnia, aggressive behavior and loss of speech. An extensive work up for causes of ataxia and occult malignancy was negative except for presence of oligoclonal bands and anti-NMDA receptor antibodies in the cerebrospinal fluid. The patient did not improve despite treatment with steroids and intravenous immunoglobulin. His first improvement occurred within 48 hr of rituximab infusion and with continued rituximab he returned to his neurological baseline. This anti-NMDA receptor encephalitis case presented as cerebellar ataxia refractory to conventional treatment and responded to rituximab.