特発性正常圧水頭症に類似した症状・画像所見で初発した神経サルコイドーシスの1例

Hydrocephalus Mimicking Idiopathic Normal Pressure Hydrocephalus as the First Manifestation of Neurosarcoidosis

症例は61歳女性。歩行障害，尿失禁と認知機能低下で発症，頭部MRIで特発性正常圧水頭症に類似した所見を認めた。脳室腹腔シャント施行後，症状が改善した。縦隔リンパ節生検を行い，神経サルコイドーシスによる水頭症と診断した。特発性正常圧水頭症に類似した症状・画像所見で発症した神経サルコイドーシスの報告はこれまでになく，文献的考察を加えて報告する。

Abstract

A 61-year-old woman presented with a 10-month history of gait disturbance and a 7-month history of urinary incontinence. The Hasegawa dementia scale-revised score indicated cognitive impairment. Brain magnetic resonance imaging (MRI) indicated hydrocephalus with disproportionately enlarged subarachnoid space. This is usually considered a characteristic finding in idiopathic normal pressure hydrocephalus (iNPH). Ventriculo-peritoneal shunting improved the patient's symptoms. Neurosarcoidosis was suspected as a cause of the hydrocephalus because of the abnormalities in the cerebrospinal fluid and the abnormal enhancement of the cauda equina, the leptomeninges of the brainstem, and the spinal cord, as seen on MRI with gadolinium enhancement. A biopsy from the mediastinum lymph nodes confirmed the histological diagnosis of sarcoidosis. Physicians should consider the possibility of neurosarcoidosis in patients presenting with hydrocephalus, even in cases where clinical and radiological data are characteristic of iNPH.

(Received April 13, 2016; Accepted July 5, 2016; Published December 1, 2016)