Recurrent Bickerstaff Encephalitis: Clinical Manifestation and Potential Treatment Options
Ketevan Amirkhanashvili1, Kelvin Chang2, Jessalyn Shen1, Niki Holtzman-Hayes1, Garrett Friedman3, Lauren Gluck1, Mark Milstein1
1Montefiore Medical Center, 2Brown University, 3Boston University
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.
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.