Rodgers-Johnson P, Morgan O S, Mora C, Sarin P, Ceroni M, Piccardo P, Garruto R M, Gibbs C J, Gajdusek D C
Laboratory of Central Nervous System Studies, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, MD 20892.
Ann Neurol. 1988;23 Suppl:S121-6. doi: 10.1002/ana.410230729.
We report clinical and laboratory investigations of 47 native-born Jamaican patients with endemic tropical spastic paraparesis and of 1 patient with tropical ataxic neuropathy. Mean age at onset was 40 years, with a female-male preponderance (2.7:1). Neurological features of endemic tropical spastic paraparesis are predominantly those of a spastic paraparesis with variable degrees of proprioceptive and/or superficial sensory impairment. Using enzyme-linked immunoabsorbent assay (ELISA), IgG antibodies to human T-lymphotropic virus type I (HTLV-I) were present in 82% of sera and 77% of cerebrospinal fluids. On Western blot analysis, IgG antibodies detected the p19 and p24 gag-encoded core proteins in both serum and cerebrospinal fluid. Titers were tenfold higher by ELISA in serum than in cerebrospinal fluid, and some oligoclonal bands present in fluid were not seen in serum. Serum-cerebrospinal fluid albumin ratios were normal, and IgG indexes indicated intrathecal IgG synthesis. Histopathological changes showed a chronic inflammatory reaction with mononuclear cell infiltration, perivascular cuffing, and demyelination that was predominant in the lateral columns. In 1 patient, a retrovirus morphologically similar to HTLV-I on electron microscopy was isolated from spinal fluid. Our investigations show that endemic tropical spastic paraparesis in Jamaica is a retrovirus-associated myelopathy and that HTLV-I or an antigenically similar retrovirus is the causal agent.
我们报告了47例牙买加本地出生的地方性热带痉挛性截瘫患者以及1例热带共济失调性神经病患者的临床和实验室研究情况。发病时的平均年龄为40岁,女性多于男性(比例为2.7:1)。地方性热带痉挛性截瘫的神经学特征主要为痉挛性截瘫,并伴有不同程度的本体感觉和/或浅感觉障碍。采用酶联免疫吸附测定法(ELISA),在82%的血清和77%的脑脊液中检测到了抗人类I型嗜T淋巴细胞病毒(HTLV-I)的IgG抗体。在蛋白质印迹分析中,IgG抗体在血清和脑脊液中均检测到了p19和p24 gag编码的核心蛋白。ELISA检测血清中的滴度比脑脊液中的高10倍,并且脑脊液中存在的一些寡克隆带在血清中未出现。血清-脑脊液白蛋白比值正常,IgG指数表明存在鞘内IgG合成。组织病理学变化显示为慢性炎症反应,伴有单核细胞浸润、血管周围套袖样改变以及脱髓鞘,脱髓鞘在侧柱中最为明显。在1例患者中,从脑脊液中分离出了一种在电子显微镜下形态与HTLV-I相似的逆转录病毒。我们的研究表明,牙买加的地方性热带痉挛性截瘫是一种与逆转录病毒相关的脊髓病,HTLV-I或一种抗原性相似的逆转录病毒是致病因子。