Smadja D, Cabre P, Bellance R, Vernant J C
Service de neurologie, Hôpital Pierre Zobda-Quitman, Fort-de-France.
Bull Soc Pathol Exot. 1993;86(5 Pt 2):433-8.
To date, 271 cases of HTLV1-associated paraplegia have been observed in Martinique (French West Indies). The clinical picture consisted mostly in a spastic paraparesis or paraplegia with sphincter disturbances (80%) and lower limbs pains (60%). The severity of the disease appeared variable: after a mean disease duration of 6.5 years, 40% of the patients could walk without help, 35% used a single crutch, and 25% used a couple of crutches or were confined to a wheelchair. A variable neuromuscular component was observed in 70 cases (25.4%). In 38 cases, the peripheral signs (SIGNS) or the myositis were only mild. In contrast, 25 patients presented with severe amyotrophy evoking amyotrophic lateral sclerosis, and 7 other had features of dermatopolymyositis. Lastly, an extra-neural spreading of the disease was extremely frequent, including lymphocytic alveolitis (76%), sicca syndrome (69%) and more rarely uveitis, arthritis or vasculitis.
迄今为止,在马提尼克岛(法属西印度群岛)已观察到271例与人类嗜T淋巴细胞病毒1型(HTLV1)相关的截瘫病例。临床表现主要为痉挛性轻截瘫或截瘫,并伴有括约肌功能障碍(80%)和下肢疼痛(60%)。疾病的严重程度各不相同:平均病程6.5年后,40%的患者能够独立行走,35%的患者使用单拐,25%的患者使用双拐或只能依靠轮椅行动。70例(25.4%)患者存在不同程度的神经肌肉症状。38例患者的周围体征或肌炎症状较轻。相比之下,25例患者出现严重肌萎缩,类似肌萎缩侧索硬化,另有7例患者具有皮肌炎特征。最后,疾病的神经外扩散极为常见,包括淋巴细胞性肺泡炎(76%)、干燥综合征(69%),较少见的有葡萄膜炎、关节炎或血管炎。