Role of high-resolution magnetic resonance imaging in the diagnosis of primary angiitis of the central nervous system.

Abstract

BACKGROUND: Primary angiitis of the central nervous system (PACNS) is a rare

disorder and is often difficult to diagnose due to the lack of a confirmatory

test. PACNS can generally be diagnosed based on typical angiographic findings. We

describe herein a patient diagnosed with PACNS despite the presence of normal

findings on conventional angiography. CASE REPORT: A 44-year-old man with a

recent history of ischemic stroke in the right posterior cerebral artery

territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an

acute infarction within the left posterior inferior cerebellar artery. His

medical history was unremarkable except for hyperlipidemia; the initial

examination revealed mild gait imbalance. During the 10 days of hospital

admission, the patient experienced four recurrent ischemic strokes within the

posterior circulation territory (occipital lobe, pons, and cerebellum). He was

diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery

exhibited no demonstrable luminal stenosis, but there were direct imaging signs

of central nervous system angiitis including wall thickening and contrast

enhancement. High-dose intravenous steroid therapy followed by oral prednisolone

was administered. There was no further stroke recurrence and follow-up imaging of

the arterial walls showed normalization of their characteristics. CONCLUSIONS:

The present case emphasizes the importance of wall imaging in the diagnosis and

treatment of PACNS.