Author Of 1 Presentation
P0786 - Anti-NMDA receptor encephalitis presenting as focal temporal lobe seizures with mixed transcortical aphasia. (ID 1940)
Abstract
Background
Anti-NMDA receptor antibody associated encephalitis is an autoimmune encephalitis due to antibodies against neuronal surface antigens mediating glutamate neurotransmission. Changes in consciousness, seizures, cognitive, speech and behavioral abnormalities, movement disorders and autonomic dysfunction can be presenting features. Initial CSF analysis can reveal lymphocytosis vs pleocytosis and oligoclonal bands can be present.
Objectives
This is a case report of anti-NMDA receptor encephalitis presenting as focal temporal lobe seizures and mixed transcortical aphasia.
Methods
Case Report. A previously healthy 24- year-old woman presented with reduced speech over the past four days and report of brief episode (<30s) of right eye deviation and right upper extremity jerking. Neurological exam was intact except for language dysfunction. She was non-fluent, aprosodic with limited vocabulary. Naming and comprehension were impaired. Repetition was intact. She was administered loading dose of lacosamide with subsequent additional load of levetiracetam. Electroencephalogram post first anti-epileptic showed focal slow wave rhythmic/semi-rhythmic delta and theta activity interspersed with frequent epileptiform discharges in left anterior temporal and frontal central lobes. MR brain contrasted imaging on day 2 of presentation was negative, however when repeated on day 7 showed T2 FLAIR hyper intensity in lateral aspect of left temporal lobe. CSF analysis showed lymphocytic pleocytosis (WBC 35 cells, lymphocytes 98/100) and subsequently anti NMDA receptor positivity (high).
Results
Initiation of solumedrol 1000 mg IV for five days, followed by intravenous immunoglobulin therapy and continued anti-epileptic medication resulted in improvement of speech and resolution of epileptiform activity on EEG, however with some remnant of polymorphic delta waves and rare sharps. Acyclovir coverage was discontinued once HSV PCR resulted as negative. Screening for malignancy was negative.
Conclusions
We present a patient with mixed transcortical aphasia with EEG correlate representing focal temporal lobe epileptiform discharges as the initial presentation of anti-NMDA receptor antibody mediated encephalitis. To our knowledge, mixed transcortical aphasia has not been reported before in association with NMDA receptor antibody mediated encephalitis. We review the language dysfunction associated with the disorder, recommending low threshold for suspicion of auto-immune encephalopathies for new onset seizures or speech changes in young patient. Both immunosuppressive and anti-epileptic therapy aided in the improvement of patient’s deficits. Tumor detection can be absent in less than half the patients, but continued surveillance is recommended.