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Abstract Details

Hemiparesis and Demyelination as Presentation of NMDA Receptor Antibody Encephalitis
MS and Related Diseases
P02 - (-)
111
BACKGROUND: NMDA receptor antibody encephalitis is characterized by neuropsychiatric symptoms, seizures, autonomic changes and abnormal movements, together with laboratory evidence of the auto-antibodies.
DESIGN/METHODS: Case report.
RESULTS: A 22 year-old woman presented with personality changes, 65 lb weight loss and amenorrhea for 6 months. Examination showed decreased attention, short-term memory and judgment. She had mild upper motor neuron left facial paresis, 4/5 strength in her left arm and positive left Babinski sign. Brain MRI showed bitemporal T2- FLAIR hyperintensities involving both gray and white matter with mass effect on the frontal horn of the right lateral ventricle. Cerebrospinal fluid had 68mg/dl protein, 4 leukocytes/mm3 and normal glucose; CSF Herpes simplex PCR and serum Mayo clinic paraneoplastic panel were negative. EEG showed background slowing. A right temporal lobe biopsy showed perivascular lymphocytic infiltrates with CD3, rare CD20, and infiltrating CD68-immunopositive cells. The most striking feature was the presence of scanty residual axons in zones lacking any myelin suggesting demyelinating disease. Whole body CT and pelvic ultrasound were normal. Testing for serum NMDA receptor antibodies was positive. IV steroids for 5 days gave no improvement, so plasma exchange(5 sessions) was done after which her memory and strength improved. She relapsed 2 months later and plasma exchange was repeated.
CONCLUSIONS: NMDA antibody receptor encephalitis is usually a paraneoplastic syndrome associated with ovarian teratoma or dermoid cyst. To our knowledge, a presentation with hemiparesis; and gray and white matter involvement with mass effect mimicking a tumor has not previously been reported. The biopsy results were confusing by showing demyelination and diagnosis of atypical MS was initially considered. In conclusion, NMDA receptor encephalitis should be considered in cases with bitemporal mass lesions presented with hemiparesis. A biopsy showing demyelination does not rule out the diagnosis.
Authors/Disclosures
Pradeep C. Bollu, MD
PRESENTER 		Dr. Bollu has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Abbvie.
Anantha Vellipuram, MD Dr. Vellipuram has nothing to disclose.
Divyajot Sandhu, MD, MBBS (Sanford Medical Center) Dr. Sandhu has nothing to disclose.
No disclosure on file
Miguel Chuquilin Arista, MD, FAAN Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Item writer with 好色先生. Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Item Writer with 好色先生. Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Item Writer with 好色先生. Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Item reviewer with National Board of Medical Examiners. Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Southeast regional advisory board with Argenx. Dr. Chuquilin Arista has received personal compensation in the range of $500-$4,999 for serving as a Focus group with Alexion. Dr. Chuquilin Arista has received personal compensation in the range of $0-$499 for serving as a Speaker with Periodic Paralysis Association.
No disclosure on file