Abstract Details

Title A Young Woman with Palatal Myoclonus, Memory loss and Seizures

Topic Autoimmune Neurology

Presentation(s) P2 - Poster Session 2 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-050

Objective We report a rare case of palatal myoclonus in a 32 year old woman with no significant medical history, diagnosed with GAD-65 autoimmune encephalitis, with an infrequently described presentation of seizures, palatal myoclonus and memory loss.

Background There are several known associated antibody related epilepsy syndromes that often present with seizure. We present a case of GAD-65 associated autoimmune enephalitis, with a rare but formerly described presentation of pharmacoresistant epilepsy and palatal myoclonus. Palatal myoclonus has also been described with lesions involving the triangle of Mollaret as well as hypertrophy of the inferior olive, although often will appear normal on brain imaging in the absence of ischemia or other injury.

Design/Methods The patient described presented with nocturnal seizures, that rapidly increased in duration and frequency over the coming weeks. She further developed onset of palatal myoclonus and progressive dysarthria, dysphagia, and short term memory loss. She was treated for GAD-65 associated autoimmune encephalitis with IVIG and steroids. Her palatal myoclonus responded well to benzodiazepine and beta blocker therapy. She was also screened for underlying malignancy and found to have increased uptake on PET imaging in the R inguinal lymph nodes, which was scheduled for biopsy.

Results Testing initially including a bedside EEG and brain MRI which were normal. Serum autoimmune workup revealed mildly elevated anti-TPO antibody levels (12) and high Anti-GAD 65 levels (365). She had MOCA testing scored at 25/30. She was also noted to have mild splenomegaly with increased moderate to intensely FDG avid R inguinal lymph nodes which was scheduled for biopsy.

Conclusions The triad of seizures, palatal myoclonus, and memory deficits is a rare presentation of GAD-65 autoimmune encephalitis but has been described. Palatal myoclonus with normal brain imaging should raise suspicion for the possibility of underlying autoimmune or paraneoplastic disease.

Authors/Disclosures
Shawn Allen, MD PRESENTER	Dr. Allen has nothing to disclose.
Mohammad A. Kabir, MD (Hamilton Physician Group)	Dr. Kabir has received personal compensation for serving as an employee of Hamilton Physician Group. Dr. Kabir has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Inlightened Inc. . Dr. Kabir has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB Pharma. Dr. Kabir has received personal compensation in the range of $0-$499 for serving as a Consultant for Livanova Inc. Dr. Kabir has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Eisai. Dr. Kabir has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Jazz Pharmaceuticals. Dr. Kabir has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Jazz Pharma. Dr. Kabir has stock in ArgenX. Dr. Kabir has stock in Lilly. Dr. Kabir has stock in Pfizer.
Thanujaa Subramaniam, MD (Brown Neurology)	Dr. Subramaniam has nothing to disclose.
Aaron F. Struck, MD (Washington University in St. Louis)	The institution of Dr. Struck has received research support from Ceribell.

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