Abstract Details

Title A unique neurological presentation of Granulomatosis with polyangiitis (Wegener's granulomatosis) with polyneuropathy, myopathy and small vessel stroke.

Topic Autoimmune Neurology

Presentation(s) P5 - Poster Session 5 (8:00 AM-9:00 AM)

Poster/Presentation
Number 15-008

Objective Granulomatosis with polyangiitis (Wegener's granulomatosis) is a necrotizing, granulomatous vasculitis of the medium and small arteries, mainly affecting the respiratory tract and kidneys however can affect the Central and Peripheral nervous systems. Here, we report a rare and complicated neurological presentation of Granulomatosis with polyangiitis presenting with polyneuropathy, Polymyalgia Rheumatica (PMR) and small vessel stroke.

Background 60 y/o woman with h/o peripheral vascular disease, hypertension, osteoarthritis presented with a 4-month history of progressive neck and back pain, and dysesthesias of all four extremities. Initial neurological exam revealed normal motor strength in all muscle groups, acral sensory loss to all modalities and decreased reflexes, consistent with neuropathy. She had an episode of transient diplopia 6 weeks after presentation, with negative brain MRI. She was later hospitalized for diplopia, imbalance, bilateral ptosis, and ophthalmoplegia. The exam was significant for splinter hemorrhages on several nail beds, diffuse myofascial tenderness. Neurological exam showed mild ptosis on the right, right sided Internuclear Ophthalmoplegia (INO), weakness in proximal muscles, acral sensory loss, diffuse hyporeflexia, and bilateral dysmetria.

Design/Methods NA

Results Blood workup showed elevated ESR (81), CRP (61), positive RF and c-ANCA (1:80), with normal Vitamin-B1, B6, mildly low B12 and D, negative ANA, Sjogren's, APLA, AchR ab, MuSK, TPO, SPEP, and UPEP. CSF was normal. EMG showed polyneuropathy with mixed axonal and demyelinating features. MRI brain showed restricted diffusion in dorsal median midbrain, in a V-shaped morphology, as has been reported in Wernikink Commissure Syndrome involving the inferior paramedian mesencephalic arteries. CT chest showed multiple pulmonary nodules.

Conclusions Overall our patient’s clinical syndrome is consistent with a small vessel vasculitis, affecting both central and peripheral nervous systems, resulting in peripheral neuropathy, PMR vs myopathy, and a small vessel stroke. She was treated with high dose IV methylprednisolone followed by oral steroid taper and Rituximab with clinical improvement.

Authors/Disclosures
Azima Shaji, MD, MBBS (University of Texas, Health Science Center, Houston) PRESENTER	Dr. Shaji has nothing to disclose.
Sruthi Devarinti, DO (St.Luke Neurology)	Dr. Devarinti has nothing to disclose.
Richard Leung, MD (Northwell Health)	No disclosure on file
Divisha Raheja, MD, F�鶹��ýӳ�� (St. Lukes Neurology Associates)	Dr. Raheja has nothing to disclose.

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