Abstract Details

Title Neuromyelitis Optica Spectrum Disorder Presenting as Symmetric, Bilateral Cranial Nerve Palsies

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 15-008

Objective NA

Background Neuromyelitis Optica Spectrum Disorder (NMOSD) is an autoimmune antibody mediated astrocytopathy characterized by frequent relapses and accumulation of disability. Several core clinical syndromes have been identified including bilateral optic neuritis, longitudinally extensive transverse myelitis, and brainstem syndromes. We describe a case of AQP-4 positive NMOSD presenting with symmetric, bilateral CN IX, X, and XII palsies.

Design/Methods A 19-year-old African American female presented with several days of nausea, vomiting, and progressive dysarthria and dysphagia. She was evaluated twice in the preceding week, with initial symptoms attributed to a gastrointestinal illness. Her symptoms progressed precipitously, prompting re-evaluation for hypersalivation and dysarthria with shortness of breath. Neurologic examination depicted bilateral CN IX, X, and XII palsies. She was admitted for further evaluation, where a contrasted MRI of the brain and cervical spinal cord demonstrated T2 hyperintensity at the dorsal cervicomedullary junction with homogenous contrast enhancement. CSF was notable for lymphocytic pleocytosis and mildly elevated IgG index (0.66). She required intubation for airway protection due to excessive secretions. Serum AQP-4 antibody resulted on day three of admission (positive titer 1:80). She underwent a five-day course of pulse intravenous methylprednisolone (IVMP), followed by six sessions of plasma exchange (PLEX). While repeat MRI on hospital day 20 demonstrated resolution of contrast enhancement, the patient was unable to be weaned from the ventilator. She ultimately underwent tracheostomy and gastric feeding tube placement prior to discharge.

Results NA

Conclusions We present a case of symmetric, bilateral CN IX, X, and XII palsies causing severe isolated dysarthria and dysphagia as the presenting symptom of AQP-4 positive NMOSD. While the presence of multiple cranial nerve palsies in neurologic conditions is not uncommon, symmetric involvement in demyelinating diseases is not well reported. NMOSD should remain high on the differential for patients presenting with lower brainstem cranial nerve palsies to guide early, aggressive treatment.

Authors/Disclosures
Alise K. Carlson, MD (Cleveland Clinic) PRESENTER	Dr. Carlson has received research support from Biogen (fellowship grant 16696-P-FEL).
Ahmad Mahadeen, MD (University of Mississippi Medical Center)	Dr. Mahadeen has nothing to disclose.
Catherine Hassett, MD	Dr. Hassett has nothing to disclose.
A. Blake Buletko, MD (Cleveland Clinic)	Dr. Buletko has nothing to disclose.