中国海洛因海绵状白质脑病的临床和病理特点

Clinical and pathological characteristics of heroin spongiform leukoencephalopathy in China

目的 总结海洛因海绵状白质脑病 (HSLE)的临床、影像和病理特征。方法 2 8例患者的临床、CT和MRI分析 ,2例脑部解剖和 8例脑活检和电镜观察。结果 HSLE的临床、影像和病理特点 :(1)有烫吸海洛因病史 ;(2 ) 2 8例患者都有戒毒史 ,17例在戒毒过程中发病 ,8例戒毒后 1～ 2个月发病 ,2例在戒毒后 4个月发病 ,1例吸入大量海洛因后发病 ,戒毒后症状加重 ;(3)多数患者急性起病 ,多以小脑受损为首发症状 ;(4)锥体束受损常见 ,感觉正常 ;(5 )脑脊液正常 ;(6 )头部CT和MRI示大脑、小脑白质广泛对称性受累 ;(7)主要病理改变为脑白质海绵状空泡变性。结论 中国大陆首次发现HSLE。有海洛因烫吸史者出现小脑症状时 ,要考虑到HSLE。

Objective To observe the clinical, image and pathological characteristics of Heroin Spongiform Leukoencephalopathy(HSLE). Methods Clinical, CT and MRI analysis of 28 cases, brain autopsies of 2 cases and brain biopsies of 8 cases with HE, Loyez and Congo Red staining and observation through electron microscope. Results The clinic, image and pathologic characteristics of HSLE: (1) the history of inhalation of heated heroin vapor; (2)among 28 cases with HSLE,recurrence of HSLE was found in 17 cases during the abstinence, in 8 cases 1 2 month after abstinence,in 2 cases 4 month after abstinence and in 1 case after inhaling large amount of heroin; (3)the majority of cases were described with the acute onset characterized by cerebellar signs; (4)pyramidal tract lesion was frequently involved, but sensory system usually remained normal; (5) CSF test was normal; (6) CT and MRI revealed extensive symmetric white matter lesions in cerebra and cerebellum; (7)spongiform vacuoles degeneration of white matter was the main morphological change. Conclusions Spongiform leukoencephalopathy may be considered if a patient who had a history of inhaling heated heroin vapor showed acute cerebellar signs. Spongiform vacuoles degeneration of cerebral white matter was the main pathological change.