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

Abstract

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.