Abstract Details

Title Title: Faciobrachial Dystonic Seizures in a patient with NMDAR (N Methyl D aspartate receptor) Encephalitis

Topic Autoimmune Neurology

Presentation(s) P10 - Poster Session 10 (8:00 AM-9:00 AM)

Poster/Presentation
Number 9-004

Background FBD seizures are well recognized in leucine-rich glioma inactivated-1 antibodies (LG1) or voltage-gated potassium channel (VGKC) antibody encephalitis. NMDA encephalitis presenting with FBD seizures is rare.

Objective To report a case of Fascio brachial dystonic seizures (FBDS) in a patient with NMDAR encephalitis.

Design/Methods A previously healthy 48-year-old right-handed Hispanic female presented from a psychiatric facility with new-onset behavioral problems and abnormal facial movements for five days. Electroencephalogram (EEG) on admission showed frequent bilateral independent high-voltage [100-150 microvolts] frontal predominant rhythmic delta activity (RDA). Magnetic resonance imaging of the brain showed several T2 flair hyperintensities involving the bifrontal and biparietal areas with no enhancing lesions. The seizure semiology involved ipsilateral facial twitching and shoulder twitching lasting 2-3 minutes. FBD seizures typically involve the face (76%), to lesser extent arms and legs (34 %). . Seizures persisted on high doses of Levetiracetam, Lacosamide and Phenytoin. Autoimmune antibody panel was negative in serum but positive for high titers of NMDAR antibodies in CSF. Malignancy workup was negative. There was no clinical improvement with high-dose steroids. Patient was a Jehovah’s witness and Intravenous Immunoglobulins, or Plasmapheresis could not be done. She was subsequently started on Rituximab therapy to which she responded well.

Results The FBD seizures resolved after the second dose of rituximab along with improvement in mental status.

Conclusions FBD seizures can occur in patients with NMDAR encephalitis with no clear EEG correlate. FBD seizures can be AED refractory but immunotherapy responsive.

Authors/Disclosures
Naga Krishnakanth Madireddy, MD (Baptist Health Louisville) PRESENTER	Dr. Madireddy has nothing to disclose.
Shivani Naik, MD (Cedar Sinai Medical Center)	Dr. Naik has nothing to disclose.
Zarmina Mufti, MD	Dr. Mufti has nothing to disclose.
Vishwanath Sagi, MD, FAAN (University of Louisville, Department of Neurology)	Dr. Sagi has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for SK Life Sciences. Dr. Sagi has a non-compensated relationship as a President with Commonwealth Neurological Society that is relevant to AAN interests or activities.