Abstract Details

Title Primary Central Nervous System Vasculitis Triggered by Cytomegalovirus Encephalitis

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P01 - (-)

Poster/Presentation
Number 231

Objective

Background BACKGROUND: Vasculitis of the central nervous system refers to inflammation of the vessel walls restricted to the brain and the spinal cord.

Design/Methods DESIGN/METHODS: We present the case of a 67 year old lady who initially presented with fatigue, drowsiness and confusion. Brain MRI WWO contrast showed an old right parietal stroke, MRA showed "narrowing" of the main trunk of the left middle cerebral artery (MCA) and the cerebrospinal fluid (CSF) was positive for cytomegalovirus (CMV). After antiviral treatment and high doses of steroids, the patient clinically improved. Over the next 6 months, she had 3 recurrent events with confusion, aphasia and weakness of the right upper extremity that partially responded to steroids.

Results RESULTS: Multiple Brain MRIs, and repeat CSF analysis including CMV were normal. Repeat brain MRA did not show the previous "narrowing" of the MCA. Cerebral angiogram, paraneoplastic and autoimmune antibodies were negative. Patient was started on high doses of prednisone and gamma immunoglobulin with improvement. A follow up Brain MRI showed multiple small lacunar acute infarcts in the left corona radiata and subcortical white matter. The patient underwent meningeal and brain biopsy and pathology was suggestive of non-necrotizing vasculitis. No evidence was found for CMV on immunostaining. Cyclophosphamide was started with control of the disease.

Conclusions CONCLUSIONS: Our case is the first reported case in the literature of proven CMV encephalitis triggering a subsequent non-infectious CNS vasculitis probably secondary to an autoimmune response. The absence of findings in the initial MRIs of the brain in spite of an intermittently symptomatic patient and the subsequent development of ischemic infarcts supports the theory of intermittent focal hypoperfusion, finally leading to infarction. The high clinical suspicion even with lack of clear evidence of the disease process and empirical treatment led to a successful clinical recovery.

Authors/Disclosures
Nancy D. Rosales, MD (Wake Forest Baptist Health) PRESENTER	No disclosure on file
Camilo I. Garcia-Gracia, MD	Dr. Garcia-Gracia has received personal compensation for serving as an employee of jazz pharmaceutical. Dr. Garcia-Gracia has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Jazz pharmaceutical.
Efrain D. Salgado, MD (Cleveland Clinic Florida)	Dr. Salgado has nothing to disclose.
Virgilio D. Salanga, MD, FAAN (Cleveland Clinic - Florida)	No disclosure on file
Massimo Filippi, MD, FAAN (Ospedale San Raffaele, Neuroimaging Research Unit)	Dr. Filippi has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion, Almirall, Biogen, Merck, Novartis, Roche, Sanofi. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion, Biogen, Bristol-Myers Squibb, Merck, Novartis, Roche, Sanofi, Sanofi-Aventis, Sanofi-Genzyme, Takeda. Dr. Filippi has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bayer, Biogen, Celgene, Chiesi Italia SpA, Eli Lilly, Genzyme, Janssen, Merck-Serono, Neopharmed Gentili, Novartis, Novo Nordisk, Roche, Sanofi, Takeda, and TEVA. Dr. Filippi has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. The institution of Dr. Filippi has received research support from Biogen Idec, Merck-Serono, Novartis, Roche, the Italian Ministry of Health, the Italian Ministry of University and Research, and Fondazione Italiana Sclerosi Multipla.

��ɫ��

��ɫ��