Abstract Details

Title Recurrent Bickerstaff Encephalitis: Clinical Manifestation and Potential Treatment Options

Topic Autoimmune Neurology

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

Poster/Presentation
Number 6-020

Background

Bickerstaff encephalitis is a rare autoimmune disease involving the peripheral and central nervous system, most commonly presenting as a monophasic condition with ophthalmoplegia, ataxia and altered sensorium.

Objective NA

Design/Methods

We present a rare case of Bickerstaff encephalitis with recurrent episodes along with notable EMG findings and possible therapeutic options to prevent recurrence.

Results A 47-year-old woman presented with four days of progressive bilateral ptosis and dysphagia. Her exam was notable for anosognosia, oculobulbar weakness, hyperreflexia, and ophthalmoplegia. Her hospital course was complicated by respiratory failure and autonomic instability. NCS revealed absent F-waves in the left median, ulnar, and radial nerves. MRI, lumbar puncture, malignancy screenings, autoimmune and paraneoplastic panels were all unrevealing. She was found to be ganglioside antibody negative. The patient was treated with IVIg for possible Bickerstaff encephalitis with no improvement. Nine days after completing her IVIg course, she was treated with PLEX followed by rapid recovery. She has since re-presented with dysphagia, ptosis, diplopia and ophthalmoplegia, and has been treated successfully with steroids and PLEX. To prevent further episodes, she was started on immune-modulating therapy, and had been in remission for over one year. Recently, she accidentally discontinued her immune-modulating therapy resulting in a recurrence, which was treated with another session of PLEX.

Conclusions Additional studies are needed to guide treatment of Bickerstaff encephalitis and identify which patients are likely to benefit from PLEX over IVIG. Since this disease can lead to life threatening respiratory failure and autonomic instability, immune-modulating therapy might be a reasonable preventive option in rare cases of recurrent Bickerstaff Encephalitis. Further investigation is necessary regarding effective management of this rare clinical syndrome.

Authors/Disclosures
Ketevan Amirkhanashvili, MD (Montefiore Medical Center) PRESENTER	Dr. Amirkhanashvili has nothing to disclose.
Kelvin K. Chang	Mr. Chang has nothing to disclose.
Jessalyn Shen, MD (Montefiore Medical Center)	Dr. Shen has nothing to disclose.
Niki Holtzman-Hayes, MD (Montefiore Medical Center)	Dr. Holtzman-Hayes has nothing to disclose.
Garrett M. Friedman, MD (self)	Dr. Friedman has nothing to disclose.
Lauren Gluck, MD (Montefiore Medical Center)	Dr. Gluck has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen.
Mark Milstein, MD, FAAN (Montefiore Medical Center)	Dr. Milstein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbvie. Dr. Milstein has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for various law firms. Dr. Milstein has received publishing royalties from a publication relating to health care. Dr. Milstein has a non-compensated relationship as a Board of Directors with New York County Medical Society that is relevant to AAN interests or activities.