Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Primary central nervous system vasculitis: a differential diagnosis of longitudinally extensive spinal cord lesion

Haruto Shibata, M.D.1), Yuto Uchida, M.D., Ph.D.1)2), Shin Kobayashi, M.D.3), Koji Takada, M.D., Ph.D.1), Yukihiro Ikegami, M.D., Ph.D.4) and Noriyuki Matsukawa, M.D., Ph.D.2)

1) Department of Neurology, Toyokawa City Hospital
2) Department of Neurology, Nagoya City University Graduate School of Medicine
3) Department of Radiology, Toyokawa City Hospital
4) Emergency Department, Toyokawa City Hospital

A 63-year-old man was admitted to our hospital with a 2-month history of anxiety. He presented with cognitive impairment and muscle weakness. On MRI, T2-weighted images showed longitudinally extensive spinal cord lesion (LESCL) from C2 to T6 and gadolinium-enhanced T1-weighted images showed fan-shaped multiple linear enhancements converging to the lateral ventricles. He was diagnosed as primary central nervous system vasculitis (PCNSV) by brain biopsy. After using high dose corticosteroids, cognitive impairment and muscle weakness were dramatically improved. In patients with cognitive impairment, PCNSV should be included in the differential diagnosis of LESCL.
Full Text of this Article in Japanese PDF (2133K)

(CLINICA NEUROL, 60: 857|860, 2020)
key words: primary central nervous system vasculitis (PCNSV), dementia, brain biopsy, longitudinally extensive spinal cord lesion

(Received: 28-Mar-20)