Abstract Details

Title A?-Related Angiitis Presenting as Encephalopathy and Anterior Uveitis

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P07 - (-)

Poster/Presentation
Number 228

Objective

Background BACKGROUND: A?-related angiitis is a recently recognized subset of central nervous system vasculitis. ABRA has a similar presentation to primary angiitis of the central nervous system (PACNS) but differs pathologically by the presence of abundant A? within blood vessels. Previously reported cases of ABRA have described a disease confined to the central nervous system.

Design/Methods DESIGN/METHODS: Case Report at a Tertiary Care Center.

Results RESULTS: A 59-year-old gentleman presented to an outside facility with headaches, fevers, chills and night sweats. His initial evaluation revealed diffuse lymphadenopathy, pulmonary ground-glass opacities, normal spinal fluid exam and lymph node biopsy. He was initially treated empirically for pneumonia, although no organism was isolated. He subsequently developed bilateral uveitis followed by the subacute onset of encephalopathy. He was referred to our institution 10 weeks after symptom onset with progressively worsening cognition. His neurological examination revealed encephalopathy, papilledema and mild ataxia. Extensive laboratory work-up was negative. Subsequent analysis of cerebrospinal fluid now revealed a lymphocytic pleocytosis and markedly elevated protein. MRI demonstrated diffuse leptomeningeal enhancement. MR angiography was unremarkable. He subsequently underwent brain biopsy that was diagnostic for A?-related angiitis. He was initiated on high-dose prednisone and a plan to initiate mycophenolate mofetil after allowing time for recovery from the biopsy.

Conclusions CONCLUSIONS: A Beta-related angiitis is an uncommon cause of central nervous system vasculitis, and may have variable clinical presentations making the diagnosis difficult. We demonstrate a case of A?-related angiitis accompanied by systemic symptoms, raising the possibility of induction of ABRA due to systemic inflammation or infection. Possible characterization of an undescribed uveomeningeal syndrome secondary to ABRA is another consideration.

Authors/Disclosures
Sherri A. Braksick, MD, FAAN (Mayo Clinic) PRESENTER	Dr. Braksick has nothing to disclose.
Nicholas Child, MD (Auckland City Hospital)	Dr. Child has nothing to disclose.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.
Orhun H. Kantarci, MD	Dr. Kantarci has nothing to disclose.
Krzysztof W. Selmaj (University of Warmia and Mazury)	Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astra. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BMS. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Astra. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS. Krzysztof W. Selmaj has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merck.

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