Rapidly Progressive Multifocal Intracranial Vasculopathy Mimicking Atherosclerosis: A Diagnostic and Therapeutic Challenge
Background:
Rapidly evolving intracranial arterial stenoses are uncommon and pose a major diagnostic challenge. Distinguishing between infectious, inflammatory, and atherosclerotic causes is critical, as early recognition can prevent recurrent strokes.
Design/Methods:
An 80-year-old fully independent male with a recent history of herpes simplex virus ophthalmicus presented to the emergency department with one week of intermittent colourful floaters and visual hallucinations in his left visual field. Neurological exam revealed a left homonymous hemianopia. CT angiography (CTA) demonstrated high-grade right posterior cerebral artery (PCA) stenosis and MRI showed acute infarcts in the right temporal lobe, thalamus and corona radiata for which he was discharged on dual antiplatelet therapy (DAPT). Two months later, he re-presented with similar transient visual complaints thought to represent focal seizures and was started on Levetiracetam. CTA revealed persistent right PCA and new right anterior cerebral artery stenosis without new infarction on MRI. One month later, he presented with encephalopathy and was found to have an acute right occipital infarct. He was continued on DAPT and admitted for EEG which ruled out epileptiform activity. During this admission, he acutely developed left hemiparesis and dysarthria. CTA revealed a new right middle cerebral artery (MCA) occlusion which was previously normal on prior imaging. Digital subtraction angiography confirmed severe right M1 stenosis with a small thrombus, which was aspirated and stented. Cerebrospinal fluid analysis showed lymphocytic pleocytosis with otherwise negative infectious and autoimmune workup.
Results:
Empiric acyclovir for possible varicella-zoster virus (VZV) vasculopathy was initiated, resulting in clinical stabilization with no new neurological symptoms or recurrent strokes at 1-year follow-up.
Conclusions:
This case emphasizes that rapidly progressive intracranial stenoses in different vascular territories should raise suspicion for infectious or inflammatory vasculopathy. Negative PCR results do not exclude VZV vasculitis, as antibody testing (IgG and IGM) is more sensitive and empiric antiviral therapy may be warranted.
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