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Abstract Details

Paroxysmal Dysarthria-Ataxia Following a Brainstem Relapse in a Patient with Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease
Autoimmune Neurology
P3 - Poster Session 3 (12:00 PM-1:00 PM)
057
Describe a rare manifestation of MOGAD and the empiric treatment with anti-seizure medications.
Paroxysmal dysarthria-ataxia has been well-described in previous literature, traditionally seen in patients with multiple sclerosis (MS).  The suspected etiology in MS involves ephaptic transmission in partially demyelinated axons. It has been treated with antiepileptic drugs in several case reports and series.  We report a case of a patient with myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD) who developed paroxysmal dysarthria-ataxia following a brainstem relapse and was successfully treated with oxcarbazepine.
Case Report
A 52-year-old female previously diagnosed with MOGAD with positive serologic testing presented to the inpatient service with diplopia and gait impairment progressively worsening over two weeks. She had previously been on outpatient rituximab treatment every 6 months, but was lost to follow up. She first presented to another hospital and treated with high dose glucocorticoids. She showed modest improvement, but her symptoms returned and worsened, prompting her to present to our facility.  On exam, she had bilateral horizontal nystagmus, a left CN VI palsy, dysmetria and gait ataxia. Brain MRI with and without contrast showed bilateral T2/FLAIR changes of the cerebral peduncles and post-contrast enhancement. Due to her lack of response to steroids, the patient was treated with five cycles of plasmapheresis, which led to significant improvement. A few days after discharge, she complained of multiple events described as slurred speech with associated limb and gait ataxia. These events lasted approximately 15-30 seconds each, resolved spontaneously, and occurred 10-20 time a day at random intervals without known triggers. She was treated with oxcarbazepine for suspected paroxysmal dysarthria-ataxia, with immediate and full remission of the events.
Paroxysmal dysarthria-ataxia is a potential complication of brainstem relapse in patients with MOGAD, and empiric management with anti-seizure medications should be considered when symptoms are present.
Authors/Disclosures
Richard Sliva, MD
PRESENTER
No disclosure on file
Vinaykumar Tallavajhala, MD (USA Health University Hospital) Dr. Tallavajhala has nothing to disclose.
William A. Kilgo, MD, FAAN Dr. Kilgo has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech. Dr. Kilgo has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Kilgo has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech. Dr. Kilgo has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen.