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Abstract Details

An Unusual Manifestation of Wegener's Granulomatosis Presenting with Multiple Cranial Neuropathies
Neurotoxicology
P06 - (-)
226
BACKGROUND: Systemic granulomatoses may present with neurological manifestations secondary to vasculitis involving intracranial vessels or vasa nervorum along the peripheral nerves. However, they may also present as a pseudo-neurological syndrome mimicking cranial neuropathy secondary to intrinsic compression from skull bone inflammation.
DESIGN/METHODS: Case report and literature review.
RESULTS: A 64-year-old-female with a history of hypothyroidism presented with sudden onset right-sided facial weakness, ataxia and dysarthria. She had a progressively worsening bilateral hearing loss for past three months and had undergone tympanostomy for suspected otitis-media. Her MRI-brain with contrast showed extensive opacification of bilateral mastoid air cells and middle-ear cavities, consistent with otomastoiditis, but no abnormal enhancement intracranially and no evidence of stroke. Her CSF was benign. A cerebral angiogram did not reveal pattern of vasculitis. She also developed night sweats and dry cough during her hospital stay. A CT-chest showed symmetric bilateral hilar and mediastinal lymphadenopathy, and a working diagnosis of sarcoidosis was made. Endobronchial biopsy showed lymphoplasmacytic and neutrophilic infiltrates, with microabscesses and granulomatous inflammation with focal necrosis. This biopsy was consistent with WG. Her serological studies showed C-ANCA positivity as opposed to classic P-ANCA associated with WG. It was determined that the most likely cause of her facial paralysis and dysarthria was a result of extrinsic compression of facial nerve from mastoiditis-related edema during its course through the middle-ear cavity. Her ataxia was attributed to vestibular inflammation. She was started on cyclophosphamide and oral prednisone and had an interval improvement in her ataxia, facial weakness and pulmonary symptoms on three months follow-up. However, her hearing loss persisted and she eventually required cochlear implants bilaterally.
CONCLUSIONS: Wegener's Granulomatosis can present with cranial neuropathies such as facial paralysis secondary to intrinsic compression from inflammation in the middle ear cavity.
Authors/Disclosures
Ahmed Itrat, MD (Cleveland Clinic)
PRESENTER 		Dr. Itrat has nothing to disclose.
David Polston, MD (Cleveland Clinic) No disclosure on file
Bianca Weinstock-Guttman, MD (Department of Neurology, University At Buffalo) Dr. Weinstock-Guttman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Weinstock-Guttman has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech. Dr. Weinstock-Guttman has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis . Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbvie. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genzyme &Sanofi. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen . Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bayer. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Weinstock-Guttman has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Weinstock-Guttman has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen. Dr. Weinstock-Guttman has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Janssen. Dr. Weinstock-Guttman has received personal compensation in the range of $0-$499 for serving as a Reviewer with NIH.