PDF(Pubmed)
Abstract：
BACKGROUND: Sporadic late onset nemaline myopathy (SLONM) is a muscle disorder characterized by the presence of nemaline rods in muscle fibers. SLONM has no known genetic cause but has been associated with monoclonal gammopathy of undetermined significance and with human immunodeficiency virus (HIV) infection. Human T-cell leukemia virus-1 (HTLV-1) is a known causative agent of adult T-cell leukemia/lymphoma and HTLV-1 associated myelopathy/tropical spastic paraplegia (HAM/TSP), a chronic inflammatory neurological disease. HTLV-1 has been reported to be implicated in inflammatory myopathies, as well as in HIV infection.; however, there have been no reports of an association between HTLV-1 infection and SLONM to date.
METHODS: A 70-year-old Japanese woman presented with gait disturbance, lumbar kyphosis, and respiratory dysfunction. The diagnosis of HAM/TSP with SLONM was made based on characteristic clinical symptoms of HAM/TSP, such as spasticity in the lower extremities, and cerebrospinal fluid test results; and of SLONM, such as generalized head drooping, respiratory failure, and muscle biopsy results. Steroid treatment was initiated and improvement in her stooped posture was observed after 3 days of treatment.
CONCLUSIONS: This is the first case report of SLONM combined with HTLV-1 infection. Further studies are needed to elucidate the relationship between retroviruses and muscle diseases.
METHODS: A 70-year-old Japanese woman presented with gait disturbance, lumbar kyphosis, and respiratory dysfunction. The diagnosis of HAM/TSP with SLONM was made based on characteristic clinical symptoms of HAM/TSP, such as spasticity in the lower extremities, and cerebrospinal fluid test results; and of SLONM, such as generalized head drooping, respiratory failure, and muscle biopsy results. Steroid treatment was initiated and improvement in her stooped posture was observed after 3 days of treatment.
CONCLUSIONS: This is the first case report of SLONM combined with HTLV-1 infection. Further studies are needed to elucidate the relationship between retroviruses and muscle diseases.
摘要：
背景：散发性晚发性线虫肌病（SLONM）是一种肌肉疾病，其特征在于肌肉纤维中存在线虫棒。SLONM没有已知的遗传原因，但与意义不明的单克隆丙种球蛋白病和人类免疫缺陷病毒（HIV）感染有关。人类T细胞白血病病毒1（HTLV-1）是成人T细胞白血病/淋巴瘤和HTLV-1相关的脊髓病/热带痉挛性截瘫（HAM/TSP）的已知病原体，慢性炎症性神经疾病.据报道，HTLV-1与炎症性肌病有关，以及艾滋病毒感染。;然而,迄今为止,尚无HTLV-1感染与SLONM相关的报道.
方法：一名70岁的日本女性出现步态障碍，腰椎后凸畸形,和呼吸功能障碍。SLONM对HAM/TSP的诊断依据HAM/TSP的特征性临床症状,比如下肢痉挛，和脑脊液测试结果；以及SLONM，比如广义的头部下垂，呼吸衰竭,肌肉活检结果.开始类固醇治疗，治疗3天后观察到她弯腰姿势的改善。
结论：这是SLONM合并HTLV-1感染的首例病例报告。需要进一步的研究来阐明逆转录病毒与肌肉疾病之间的关系。
方法：一名70岁的日本女性出现步态障碍，腰椎后凸畸形,和呼吸功能障碍。SLONM对HAM/TSP的诊断依据HAM/TSP的特征性临床症状,比如下肢痉挛，和脑脊液测试结果；以及SLONM，比如广义的头部下垂，呼吸衰竭,肌肉活检结果.开始类固醇治疗，治疗3天后观察到她弯腰姿势的改善。
结论：这是SLONM合并HTLV-1感染的首例病例报告。需要进一步的研究来阐明逆转录病毒与肌肉疾病之间的关系。
