J Korean Neurol Assoc.  2013 Nov;31(4):262-265.

Primary Angiitis of the Central Nervous System Diagnosed by High-Resolution Vessel Wall MRI

Affiliations
  • 1Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. sukjae.kim@gmail.com
  • 2Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Abstract

Primary angiitis of the central nervous system (PACNS) is a poorly understood form of vascular inflammatory disease that is restricted to the brain and spinal cord. A 38-year-old woman presented with severe headache and transient aphasia. Her cerebrospinal fluid exhibited aseptic meningitis, and high-resolution vessel-wall MRI (HRVW-MRI) revealed narrowing of multiple intracranial vessels with concentric wall thickening and diffuse enhancement, suggestive of PACNS. High-dose steroid and azathioprine therapy resulted in a significant improvement in vessel wall thickening, and enhancement was observed on the follow-up HRVW-MRI.

Keyword

PACNS; Vessel-wall MRI; Therapeutic monitoring

MeSH Terms

Adult
Aphasia
Azathioprine
Brain
Central Nervous System*
Cerebrospinal Fluid
Female
Follow-Up Studies
Headache
Humans
Magnetic Resonance Imaging*
Meningitis, Aseptic
Spinal Cord
Vasculitis*
Vasculitis, Central Nervous System
Azathioprine
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