脊髄長大病変を認め，特徴的な頭部MRI画像を呈した肉芽腫性原発性中枢神経系血管炎の1例

Longitudinally Extensive Transverse Myelitis Caused by Granulomatous Primary Central Nervous System Vasculitis with Characteristic Brain Magnetic Resonance Image: A Case Report

もの忘れと歩行障害を主訴として来院し，神経学的に緩徐進行性の認知機能障害と両下肢の痙縮を呈した47歳女性を経験した。頭部MRIのT2強調画像では大脳深部白質にびまん性に高信号を，造影T1強調画像では側脳室から放射状に伸びる多発線状造影効果を認めた。脊髄MRIでは，脊髄の腫脹とC7椎体レベルからTh12椎体レベルにわたる長大病変を認めた。頭部造影MRIの特徴的な画像から原発性中枢神経系血管炎（PCNSV）を疑い，脳生検を施行し，肉芽腫性PCNSVと診断した。脊髄の長大病変をきたす疾患として，PCNSVを鑑別診断に挙げる必要がある。

Abstract

We report a case of primary central nervous system vasculitis (PCNSV) with longitudinally extensive transverse myelitis. A 47-year-old woman presented with malaise, progressive cognitive impairment, and lower limb spasticity. Diffuse hyperintense areas in the deep cerebral white matter on the diffusion-weighted image and T2-weighted images were observed during brain magnetic resonance imaging. Gadolinium-enhanced T1-weighted images showed multiple linear enhancements. A sagittal T2-weighted image displayed a longitudinal extensive lesion of transverse myelitis in the spinal column from the upper cervical (C7) to the thoracic region (Th12). On brain biopsy, the patient was diagnosed as having granulomatous primary angiitis of the central nervous system (PCNSV). This case suggests that PCNSV could show longitudinally extensive transverse myelitis.

(Received 14 January, 2021; Accepted 18 February, 2021; Published 1 August, 2021)