Nakamura N, Fujiya S, Yahara O, Fujioka Y, Kawakami Y
Clin Neuropathol. 1986 Jan-Feb;5(1):40-6.
An autopsy case of stiff-man syndrome is presented. The patient was a 54-year-old man who had suffered from fluctuating rigidity and tonic spasm of the trunk and leg muscles for three years. These symptoms were induced by emotional stress, strain at voiding and other external stimuli. Physical examination revealed stiffness of the neck, trunk and legs without obvious muscle weakness or atrophy. Deep tendon reflexes were hyperactive, while sensation was normal. EMG showed the stiffened muscles to have continuous motor unit activity which was decreased by myoneural block, spinal anesthesia, diazepam or sleep. Administration of diazepam (60 mg daily) gave some relief of the stiffness for several days. However, respiratory arrest suddenly occurred. Although the patient was resuscitated, he died nine days after this episode. Examination of the spinal cord revealed loss and degeneration of nerve cells with marked gliosis in the medial motor nuclei of the anterior horns. A symmetric degeneration in the bilateral anterior columns which has never been described as a spinal cord lesion in the stiff-man syndrome was also seen.
本文报告1例僵人综合征的尸检病例。患者为一名54岁男性，躯干和腿部肌肉出现波动性僵硬及强直性痉挛达3年。这些症状由情绪应激、排尿用力及其他外部刺激诱发。体格检查发现颈部、躯干和腿部僵硬，无明显肌肉无力或萎缩。深腱反射亢进，感觉正常。肌电图显示僵硬肌肉有持续的运动单位活动，这种活动可因肌神经阻滞、脊髓麻醉、地西泮或睡眠而减少。给予地西泮（每日60毫克）可使僵硬症状缓解数天。然而，患者突然发生呼吸骤停。尽管患者经复苏成功，但在此事件9天后死亡。脊髓检查发现前角内侧运动核神经细胞缺失和变性，伴有明显的胶质细胞增生。还发现双侧前柱有对称性变性，这在僵人综合征中从未被描述为脊髓病变。
