BRAIN and NERVE－神経研究の進歩73巻8号

2021年08月発行

症例報告

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

著者： 伏屋公晴¹ 吉倉延亮¹ 加藤雅彦¹ 林祐一¹ 木村暁夫¹ 酒々井夏子² 下畑享良¹

所属機関： ¹岐阜大学大学院医学系研究科脳神経内科学分野 ²岐阜大学医学部附属病院病理診断科

ページ範囲：P.931 - P.937

文献概要

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。

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