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Encephalitis associated with antibodies against the NMDA receptor (anti-NMDA-R) is characterized by prominent psychiatric symptoms, dyskinesias, seizures, autonomic instability, and central hypoventilation.1 We report a 20-month-old boy with oral-buccal dyskinesia, choreoathetosis, seizures, and encephalopathy, consistent with anti-NMDA-R encephalitis.
Level of evidence.
This report is Class IV evidence and is a single observational study without controls.
Case report.
A 20-month-old boy had a pruritic maculopapular rash over his face, trunk, and arms for 2 weeks without preceding fever or illness. A week later, he developed involuntary tongue thrusting and right hand twitching. He spoke less and became less interactive. On admission, his temperature was 39.2oC. Examination showed oral-buccal dyskinesia and choreoathetosis, particularly the upper extremities (video on the Neurology® Web site at www.neurology.org). Complete blood count showed a white cell count of 15.6 × 109/L with neutrophilic predominance. CSF revealed slightly elevated total nucleated cells of 5/μL with lymphocytic predominance (65%), normal protein (24 mg/dL), glucose (59 mg/dL), and lactate (1 mmol/L) concentrations. Nasogastric tube was placed owing to impaired oropharyngeal coordination. Prolonged video EEG recorded several generalized seizures from sleep. There was no electrographic correlate with the involuntary movements, which persisted during wakefulness and sleep. Brain MRI and magnetic resonance spectroscopy on day 2 were normal.
Bacterial cultures in the blood, urine, and CSF were negative. Serologies and PCRs in the serum and CSF were negative for infectious etiology …
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- RESPONSE TO IMMUNOTHERAPY IN A 20-MONTH-OLD BOY WITH ANTI-NMDA RECEPTOR ENCEPHALITIS
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