A 74 year old man with a rash, facial droop and multifocal intracranial stenosis
Yasmin Aghajan1, Philippe-Antoine Bilodeau2, Saef Izzy3
1Neurocritical Care, 2Neurology, Brigham and Women's Hospital, 3Brigham and Women's Hospital, Harvard Medical School
Objective:

NA


Background:
Although atherosclerosis is the most common cause of intracranial stenosis, the differential diagnosis includes infectious and inflammatory causes. We report a case of a Varicella Zoster Virus (VZV) induced vasculitis. 
Design/Methods:
The study is a case report.
Results:

A 74 year old man presented with an episode of loss of consciousness. CT angiography showed stenotic distal internal carotid arteries (ICA), severe stenosis of the left M1 segment, and moderate stenosis of the right M1 segment of the middle cerebral arteries (MCA) (Figure 1A-B). CT perfusion showed delayed perfusion to the left MCA territory (Figure 1C). MRI brain revealed a punctate right frontal infarct (Figure 2A). He was transferred to our hospital for superficial temporal artery - middle cerebral artery (STA-MCA) bypass. However, neurologic exam was notable for right facial droop involving right forehead and incomplete eye closure. MRI of the brain with vessel wall imaging showed thin enhancement of the bilateral ophthalmic and supraclinoid ICA’s and left M1 enhancement (Figure 2B-C). A rash was found behind the right ear with crusted vesicles as well as bilateral cranial nerve 6 palsies. Cerebrospinal fluid had 47 nucleated cells/uL with 37 red blood cells/uL. Glucose was 75 mg/dL and total protein was 57 mg/dL. CSF VZV IgG returned positive at 819.0 and CSF VZV IgM was negative at 0.25. Thus, the diagnosis was VZV vasculitis. He was treated with intravenous acyclovir and transitioned to oral valacyclovir for a 14 day course.

Conclusions:
Even though intracranial atherosclerotic disease is the most common cause of vasculopathy, infectious or inflammatory vasculitis should be considered on the differential. Before considering bypass surgery or other invasive neurosurgical procedures, ensure reversible causes of vasculopathy have been ruled out. The presence of cranial neuropathies, rash, and/or elevated inflammatory markers should be red flags for vasculitis in patients presenting with stroke.
10.1212/WNL.0000000000201823