Waragai M, Takaya Y, Hayashi M
Department of Neurology, Kofu City Hospital.
Rinsho Shinkeigaku. 1995 May;35(5):549-52.
A 67-year-old woman began to have difficulty in walking due to spastic paraparesis, sensory impairment in the lower limbs and instability on standing at the age of 59 years. Eight years later, she developed complete spastic paraplegia and urinary incontinence. On examination, she was found to have down-beat nystagmus and ocular hypermetria. Brain CT scan and MRI revealed cerebellar atrophy, predominantly in the vermis. HTLV-I antibody titer was increased in both serum and cerebrospinal fluid (CSF). She was diagnosed as having HAM and placed on prednisolone, salazosulfapyridine and high-dose vitamin C. Her symptoms including gait disturbance, nystagmus and ocular hypermetria improved but did not completely disappear. In parallel with clinical improvement, the antibody titer to HTLV and the ratio of CD4/CD8 lymphocyte subsets in blood decreased. The above observations suggest that the cerebellar signs and cerebellar atrophy share the same etiology with HAM. The presence of nystagmus and prominent cerebellar signs plus the CT and MRI findings strongly suggest that the lesions in HAM are not limited to the spinal cord but extend to the brainstem and cerebellum.
一名67岁女性在59岁时开始出现行走困难,原因是痉挛性截瘫、下肢感觉障碍和站立不稳。八年后,她发展为完全性痉挛性截瘫和尿失禁。检查发现她有下跳性眼球震颤和眼球辨距过度。脑部CT扫描和MRI显示小脑萎缩,主要累及小脑蚓部。血清和脑脊液(CSF)中HTLV-I抗体滴度均升高。她被诊断为患有人类T淋巴细胞病毒I型相关脊髓病(HAM),并接受泼尼松龙、柳氮磺胺吡啶和大剂量维生素C治疗。她的症状,包括步态障碍、眼球震颤和眼球辨距过度有所改善,但并未完全消失。随着临床症状的改善,血液中HTLV抗体滴度和CD4/CD8淋巴细胞亚群比例下降。上述观察结果表明,小脑体征和小脑萎缩与HAM具有相同的病因。眼球震颤和明显的小脑体征以及CT和MRI检查结果强烈提示,HAM的病变不仅局限于脊髓,还延伸至脑干和小脑。