Last modified: 2014-04-03
Abstract
Objective: To report the clinical features of anti NMDA receptor encephalitis in 3 Filipino adolescents.
Background: Anti-N-Methyl-D-Aspartate (NMDA) Receptor Encephalitis represents a new category of immune-mediated disorders that typically present with fever, neuropsychiatric manifestations, movement disorder, aphasia and dysautonomia. It has been reported to commonly occur in young women with teratomas but has also been diagnosed in men and children without tumors.
The Cases: We report 3 previously healthy adolescents with serum and cerebrospinal fluid that tested positive for anti-NMDA receptor antibodies. The first case is a 14 year old female who presented with fever, seizures, disorientation, aphasia, hemiparesis, unresponsiveness, dystonia and choreoatheosis. The second case is a 14 year old male who presented with fever, prominent psychiatric symptoms, persistent hypertension, orofacial dyskinesias at the onset of illness and then later developed global aphasia, catatonia, ileus , urinary and bowel incontinence. The 3rd case is a 15 year old male who presented with a 3 month history of bizarre behavior, followed by neck and truncal dystonia and orofacial dyskinesias. Work up for associated tumors was negative in all three patients. Immunotherapy was given to all three patients, IVIG in one and intravenous methylprednisolone in the other two. The dyskinesias resolved 5 to 7 days after treatment.
Conclusion: Abnormal posture and movements are important features of anti- NMDA receptor encephalitis. Its presence in a child or adolescent manifesting with behavioural changes, neuropsychiatric manifestations, seizures, and autonomic instability should alert the clinician to this condition.