側脳室に発生し神経内視鏡にて診断し得た孤発性神経サルコイドーシスの1例

A Case of Intraventricular Isolated Neurosarcoidosis Diagnosed by Neuroendoscopic Biopsy

Ⅰ．はじめに

　サルコイドーシス（以下，サ症）は非乾酪性類上皮細胞肉芽腫が肺，リンパ節，皮膚，眼などあらゆる臓器に生じて起こる病態である．その中でも，孤発性神経サ症は，全身性サ症と異なり，中枢神経系にのみ病変を有するサ症の亜型である．その頻度はサ症の中でも約1%と少なく，結核性肉芽腫の鑑別に苦慮する例も少なくない^5,7,8)．今回神経内視鏡による生検術により診断し得た孤発性神経サ症の1例を経験したので，文献的検索をふまえて報告する．

　A 76-year-old woman was admitted to our hospital because of dementia, nausea, and speech disturbances. Computed tomography（CT）of her brain showed hydrocephalus and an intra-ventricular mass with a left temporo-parieto-occipital low density area. She underwent emergency ventricular drainage. Thereafter, she was referred to our department：neurosurgery. Gadolinium-enhanced magnetic resonance imaging of the brain showed homonymous enhancement in the left lateral, third, and fourth ventricles. CT of her chest and abdomen showed no abnormal findings. Initially, we performed a neuro-endoscopic biopsy and made a histopathological diagnosis of noncaseating granuloma. However, because we did not detect pulmonary or ocular lesions, we eventually made a diagnosis of isolated neurosarcoidosis. She received a ventricular-peritoneal shunt and steroid pulse therapy, and recovered from all her symptoms.

　Neurosurgeons should be aware of the possibility of hydrocephalus mimicking an intraventricular tumor and caused by isolated neurosarcoidosis. In this article, we provide a case description and review of the literature.