Abstract Details

Title Progressive Ischemic Strokes With Malignant Cerebral Edema In a Patient With Concomitant Varicella Zoster Virus(VZV)Vasculopathy And Giant Cell Arteritis(GCA)

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P16 - Poster Session 16 (5:30 PM-6:30 PM)

Poster/Presentation
Number 4-018

Objective

We describe a case of fulminant VZV vasculopathy presenting with recurrent strokes leading to death despite aggressive antiviral therapy, who was found to have concomitant GCA on autopsy.

Background VZV antigens are detected in temporal artery biopsies in 74% patients with GCA. However, concomitant VZV vasculopathy and GCA is rare and not been reported. While VZV vasculopathy is treated with antiviral agents, GCA requires long-term steroids to prevent progressive strokes which in turn may lead to viral reactivation.

Design/Methods Case report.

Results 64-year-old Caucasian male with multiple myeloma (in remission) presented with headache, recurrent left hemiparesis and seizures. MRI showed moderate right ACA-territory infarction. Angiogram revealed near-complete occlusion of right ACA and irregularity in the right MCA branches. CSF analysis revealed lymphocytic pleocytosis and a positive VZV PCR. After a course of intravenous acyclovir and high-dose prednisone, patient recovered well with independent ambulation at the time of discharge. 2 days later, he returned with left hemiplegia. MRI showed worsening right ACA-territory infarction extending into the anterior MCA-territory with sub-falcine herniation. Repeat angiogram revealed near-complete right supraclinoid ICA, MCA and complete ACA occlusion, suggesting vasculitis. Acyclovir was continued and high-dose methylprednisolone initiated. On day 4, patient became comatose and CT revealed significant right hemispheric cerebral edema with 2.1cm midline shift and trans-tentorial herniation. Due to significant neurological impairment, hemicraniectomy was not offered, and patient was transitioned to comfort care. Autopsy revealed complete obliteration of the right ICA and its branches with transmural inflammation, multinucleated giant cells and granulomatous changes consistent with GCA. The internal elastic lamina was positive for VZV antigens consistent with active VZV infection.

Conclusions

Concomitant GCA and VZV vasculopathy poses a therapeutic dilemma with no literature to guide therapy. These patients may require long term steroids and prolonged antiviral therapy to prevent progression. Prospective studies are needed to support these recommendation.

Authors/Disclosures
Shilpa Haldal, MD PRESENTER	Dr. Haldal has nothing to disclose.
Rohan Sharma, MD	Dr. Sharma has nothing to disclose.
Mitesh P. Lotia, MD (AdventHealth Neuroscience Institute)	Dr. Lotia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Lotia has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Orphalan. Dr. Lotia has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Abbott.
Vishank A. Shah, MD (Johns Hopkins University)	Dr. Shah has nothing to disclose.
Krishna Nalleballe, MD, F�鶹��ýӳ��	Dr. Nalleballe has nothing to disclose.

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