Kim J Y, Lee K W, Roh J K, Chi J G, Lee S B
Department of Neurology, College of Medicine, Seoul National University, Korea.
J Korean Med Sci. 1994 Apr;9(2):145-54. doi: 10.3346/jkms.1994.9.2.145.
We reviewed the clinical, electrophysiological, radiological, and histopathological findings in 25 patients with benign focal amyotrophy. There were 14 patients with upper limb type and 11 with lower limb type. 18 patients had unilateral involvement and 7 had bilateral involvement asymmetrically. The characteristics clinical features were sporadic occurrence, predominance in young males, nonprogressive course or initial progression for 1 to 3 years followed by stationary state, segmental distribution of muscle weakness and atrophy localized to one limb or both homologous limbs markedly asymmetrically, and absence of any definite sensory loss or central nervous system involvement. The electrophysiological, radiological, and muscle histopathological findings suggested chronic focal anterior horn cell disease. Although the prevalence of this disease is still unknown, the importance of recognition is being emphasized because of its common occurrence in our country and the benign prognosis.
我们回顾了25例良性局灶性肌萎缩患者的临床、电生理、放射学和组织病理学检查结果。其中上肢型14例,下肢型11例。18例为单侧受累,7例为双侧不对称受累。其特征性临床特点为散发性发病,以青年男性为主,病程非进行性或最初进展1至3年后进入静止期,肌无力和萎缩呈节段性分布,局限于一个肢体或双侧同源肢体且明显不对称,无任何明确的感觉丧失或中枢神经系统受累。电生理、放射学和肌肉组织病理学检查结果提示为慢性局灶性前角细胞疾病。尽管该病的患病率尚不清楚,但鉴于其在我国的常见性及良好预后,强调了对其认识的重要性。