Uyama E, Miyajima M, Sugimoto M, Kawasaki S, Ikeda T, Araki S
First Department of Internal Medicine, Kumamoto University Medical School.
Rinsho Shinkeigaku. 1991 Mar;31(3):301-5.
A 66-year-old female suffering from HTLV-1 associated myelopathy (HAM) for more than 30 years was hospitalized because of memorial impairment, deafness, dysarthria, dysphagia, and complete paraplegia. She first noticed stiffness and weakness of the right leg at 35 years of age. Gait disturbance was slowly progressed and complete paraplegia developed 18 years later. Neurological examinations on admission revealed that she was bedridden with decubitus, mental deterioration (pre-dementia of subcortical type), bilateral optic nerve atrophy, severe sensory-neural deafness, dysarthria, complete paraplegia, and marked neurogenic bladder. Laboratory data showed mild normocytic anemia and moderate diabetes mellitus. Anti-HTLV-1 antibody titers in serum and CSF were 78,192X and 1,024X, respectively (PA method). Serum levels of soluble IL-2 receptor was markedly elevated (2,200 U/ml). Peripheral blood lymphocytes showed spontaneous proliferation when cultured for 5 days (3H-thymidine uptake; 45,285 cpm/5 X 10(4) cells). MRI examinations of the spinal cord disclosed a predominant atrophy of lower thoracic cord without any compressive lesions. Brain MRI showed diffuse high intensity lesions of the periventricular area on T2 weighted images. Such abnormalities were predominantly found in fronto-parietal region and were quite similar to those of leuko-ariosis. Single photon emission CT using 123I-iodoamphetamine showed hypoperfusion of cerebral white matter on delayed image. It has been reported that intellectual impairment and brain atrophy are not usually seen in HAM patients. The present case, however, shows that such abnormalities of the central nervous system could occur in HAM patients with a long duration of illness.
一名患有人类嗜T淋巴细胞病毒1型相关脊髓病(HAM)超过30年的66岁女性因记忆力减退、耳聋、构音障碍、吞咽困难和完全性截瘫而住院。她35岁时首次注意到右腿僵硬和无力。步态障碍逐渐进展,18年后发展为完全性截瘫。入院时的神经学检查显示,她因褥疮卧床不起,有精神衰退(皮质下型轻度痴呆)、双侧视神经萎缩、严重感音神经性耳聋、构音障碍、完全性截瘫和明显的神经源性膀胱。实验室检查数据显示有轻度正细胞性贫血和中度糖尿病。血清和脑脊液中的抗HTLV-1抗体滴度分别为78,192倍和1,024倍(PA法)。血清可溶性白细胞介素-2受体水平明显升高(2,200 U/ml)。外周血淋巴细胞培养5天后显示自发增殖(3H-胸腺嘧啶核苷摄取;45,285 cpm/5×10(4)个细胞)。脊髓的MRI检查显示下胸段脊髓主要为萎缩,无任何压迫性病变。脑部MRI在T2加权图像上显示脑室周围区域有弥漫性高信号病变。这些异常主要见于额顶叶区域,与脑白质疏松症非常相似。使用123I-碘安非他明的单光子发射CT显示延迟图像上脑白质灌注不足。据报道,HAM患者通常不会出现智力障碍和脑萎缩。然而,本病例表明,中枢神经系统的此类异常可能发生在病程较长的HAM患者中。