ラムゼイ ハント症候群から播種性帯状疱疹に進展し，髄膜脳炎・脳梗塞を併発した90歳男性の剖検例

An Autopsy Case of Meningoencephalitis and Cerebral Infarction that Developed with Ramsay Hunt Syndrome and Disseminated Herpes Zoster

症例は90歳男性。2014年3月右耳痛が出現。発熱，右外耳道に発赤・腫脹を伴う複数の小丘疹を認めた。脳神経では右末梢性顔面神経麻痺を認め，項部硬直とケルニッヒ徴候を認めた。脳脊髄液検査では細胞数572個/μL（単核球212個），蛋白631mg/dLと増加を認め水痘-帯状疱疹ウイルスPCRが陽性であり，頭部MRI FLAIR画像にて左橋底部および左側頭葉先端部に高信号を認めた。水痘-帯状疱疹ウイルス性髄膜脳炎と診断し，アシクロビル投与を開始したところ，全身に皮疹が拡大し播種性帯状疱疹に進展したが，次第に意識レベル・皮疹の改善を認めた。4月中旬に突然の嘔吐，黒色便および貧血を認め，死亡した。剖検では，左側頭葉および島を中心として脳表および血管周囲腔に沿った小血管主体のリンパ球浸潤を認めた。帯状疱疹ウイルス感染を示唆する血管炎を病理学的に確認できた点で貴重な症例と考えられた。

Abstract

We report here the clinical presentation and subsequent autopsy of a 90-year-old man who developed small papules with pain and swelling in his right ear. On admission, he exhibited right facial nerve paralysis, neck stiffness and Kernig's sign. The cell count was elevated and the varicella-zoster virus-PCR was positive in the CSF. Brain magnetic resonance imaging showed hyperintense lesions in the left pons and left temporal lobe, in FLAIR images. We diagnosed the patient with Ramsay Hunt syndrome and meningoencephalitis due to varicella-zoster virus. Although the symptoms of meningitis improved following treatment with intravenous acyclovir (750 mg/day initially, raised to 1,125 mg/day), 16 days after admission, he died suddenly due to gastrointestinal hemorrhage. The autopsy findings included lymphocytic infiltration of the leptomeninges and perivascular space of the cerebrum, and slight parenchyma in the left temporal lobe and insula, as the main histological features. Encephalitis due to varicella zoster virus has been recognized as a vasculopathy affecting large and small vessels. Pathological confirmation is rare in varicella zoster virus meningoencephalitis.