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.
