臨床神経学

<シンポジウム20―4>難治性神経感染症update

進行性多巣性白質脳症

水澤 英洋1), 岸田 修二2), 西條 政幸3), 雪下 基弘4), 宍戸―原 由紀子5), 澤 洋文6), 長嶋 和郎7), 奴久妻 聡一8), 山田 正仁9)

1)東京医科歯科大学大学院脳神経病態学〔〒113―8519 東京都文京区湯島1―5―45〕
2)都立駒込病院神経内科
3)国立感染症研究所ウイルス第一部第三室
4)佐賀大学医学部内科(神経内科)
5)杏林大学医学部病理学教室
6)北大人獣共通感染症分子病態・診断部門
7)札幌東徳州会病院病理部
8)神戸市環境保健研究者微生物部
9)金沢大学大学院脳老化・神経病態学

Progressive multifocal leukoencephalopathy (PML) is caused by reactivation of latently infected JCV when hosts' immune system is impaired by HIV infection, hematologic diseases, collagen diseases, immunemodulatory therapy and so on. PML was rare but HIV infection and Natalizumab have made it much more common while the prognosis is much better than other PML. PML patients present with various signs and symptoms including hemiparesis, dementia, aphasia, visual disturbance, cranial nerve paresis, cerebellar signs and bladder bowel disturbance. Brain MRI reveals characteristic demyelinating lesions in the CNS white matter and CSF mild increase of protein with or without mild mononuclear pleocytosis. Detection of JCV genome from CSF is crucial for the clinical diagnosis of PML. PML was once thought to be fatal but some HIV infected PML patients showed halting progression or even recovery after introduction of HAART. In addition, anti-malarial drug mefloquine was found to be effective. Recovery of immunity may provoke some inflammatory responses known as immune reconstruction inflammatory syndrome (IRIS) which requires high dose corticosteroid. In Japan, we are providing free test of CSF-JCV genome and organized a unique system for surveillance and clinical research of PML. Using this system we hope to improve diagnosis and therapy of PML in Japan.
Full Text of this Article in Japanese PDF (803K)

(臨床神経, 51:1051−1057, 2011)
key words:進行性核上性麻痺,JCウイルス,ヒト免疫不全ウイルス,ナタリツマブ,メフロキン

(受付日:2011年5月20日)