Liao Y S, Chen S T, Tang L M, Ro L S
Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei, Taiwan, ROC.
J Formos Med Assoc. 1996 Apr;95(4):329-32.
Dejerine-Sottas disease is an uncommon hereditary neuropathy which has not been reported in Taiwan. We describe a 57-year-old woman who had slowly progressive weakness in her four limbs since adolescence. None of her close relatives had the disease and no consanguinity was noted. Neurologic examination showed severe weakness and vibratory sensation loss in the four limbs. The tendon reflexes were generally absent. Electrophysiologic studies suggested a systemic myelinopathic process. Light and electron microscopy of the sural nerve biopsy specimens revealed many onion-bulb shapes formed by the Schwann cell processes or basement membranes without any evidence of myelin sheaths around the axons, which are characteristic features of Dejerine-Sottas disease.
德热里纳 - 索塔斯病是一种罕见的遗传性神经病变，台湾地区尚无相关报道。我们报告一名57岁女性，自青少年期起四肢逐渐出现进行性无力。她的近亲中无人患此病，且无近亲结婚情况。神经系统检查显示四肢严重无力及振动觉丧失。腱反射通常消失。电生理研究提示全身性髓鞘病变过程。腓肠神经活检标本的光镜和电镜检查显示，施万细胞突起或基底膜形成许多洋葱球样结构，轴突周围无髓鞘形成的证据，这是德热里纳 - 索塔斯病的特征性表现。
