Gessain A, Gout O
Laboratory of Tumor Cell Biology, National Cancer Institute, National Institues of Health, Bethesda, MD 20892.
Ann Intern Med. 1992 Dec 1;117(11):933-46. doi: 10.7326/0003-4819-117-11-933.
To review the clinical, epidemiologic, immunologic, and virologic aspects of the chronic myelopathy associated with human T-cell leukemia/lymphoma virus type I (HTLV-I), currently called tropical spastic paraparesis/HTLV-I-associated myelopathy (TSP/HAM).
Studies done after 1985, when TSP/HAM was first recognized, were identified by a computer search using MEDLARS II and CANCERLIT. Additional information was acquired from personal files and bibliographies of existing literature.
A total of 400 articles, 90 book chapters, and 150 abstracts from meetings covering all aspects of HTLV-I and neurologic diseases were critically analyzed, and information from 250 publications was included.
TSP/HAM is present in most HTLV-I endemic areas, with a prevalence ranging from 5.1 to 128 per 100,000 inhabitants. Up to 20% of patients develop TSP/HAM after transfusion of HTLV-I contaminated blood. Pathologic characteristics indicate a chronic meningomyelitis. The clinical features consist of a chronic progressive spastic paraparesis or paraplegia, sphincter disturbances, and minimal sensory loss. Supraspinal and peripheral nerve involvement is sometimes observed. High titers of HTLV-I-specific antibodies are present in the serum and cerebrospinal fluid. The high level of humoral and cellular immunologic response and the association of TSP/HAM with other immunologic diseases suggest an immune-mediated process. Corticosteroids and immunosuppressor treatment usually result in only short-term improvement.
TSP/HAM is a common neurologic disease in many parts of the world. All patients with chronic progressive myelopathies should be tested for serum and cerebrospinal fluid HTLV-I-specific antibodies. Systematic screening of blood donors for HTLV-I is necessary to help prevent the dissemination of the virus and the occurrence of post-transfusional cases.
回顾与I型人类T细胞白血病/淋巴瘤病毒(HTLV-I)相关的慢性脊髓病的临床、流行病学、免疫学及病毒学方面情况,该病目前称为热带痉挛性截瘫/HTLV-I相关脊髓病(TSP/HAM)。
1985年TSP/HAM首次被认识之后所做的研究,通过使用医学文献分析和检索系统II(MEDLARS II)及癌症文献检索(CANCERLIT)进行计算机检索来识别。从个人档案和现有文献的参考文献中获取了更多信息。
对总共400篇文章、90个书籍章节以及150篇涵盖HTLV-I和神经系统疾病各方面的会议摘要进行了严格分析,纳入了250篇出版物中的信息。
TSP/HAM存在于大多数HTLV-I流行地区,每10万居民中的患病率为5.1至128例。高达20%的患者在输注受HTLV-I污染的血液后发生TSP/HAM。病理特征显示为慢性脑脊膜炎。临床特征包括慢性进行性痉挛性截瘫或截瘫、括约肌功能障碍以及轻微感觉丧失。有时可观察到脊髓以上和周围神经受累。血清和脑脊液中存在高滴度的HTLV-I特异性抗体。高水平的体液和细胞免疫反应以及TSP/HAM与其他免疫性疾病的关联提示存在免疫介导过程。皮质类固醇和免疫抑制剂治疗通常仅导致短期改善。
TSP/HAM是世界许多地区常见的神经系统疾病。所有慢性进行性脊髓病患者均应检测血清和脑脊液中的HTLV-I特异性抗体。对献血者进行HTLV-I系统筛查对于帮助预防病毒传播和输血后病例的发生是必要的。