Oe H, Miyashita K, Tanaka K, Naritomi H, Kinugawa H, Sawada T
Department of Medicine, Niigata University.
Rinsho Shinkeigaku. 1996 Oct;36(10):1166-71.
We described a 60-year-old man with 5-year history of insulin dependent diabetes mellitus who developed continuous rigidity of truncal muscle and painless, rhythmic muscular spasm of trunk and proximal lower and upper extremities. The rigidity continued even in sleep. The painless muscle spasm was often precipitated by volitional movement and emotional stimuli. Intravenous administration of diazepam strongly attenuated the muscle spasm as well as truncal rigidity. Surface electromyography showed the continuous contraction of abdominal and paraspinal muscles. The rhythmic, clonic spasm of shoulder, triceps brachii, intercostal, abdominal, paraspinal and quadriceps femoris muscle induced by voluntary neck flexion was not compatible with typical stiff-man syndrome. Antibody against glutamic acid decarboxylase (GAD) was detected in the serum and cerebrospinal fluid of this patient. His condition was getting well with oral intake of sodium valproate. While painless, rhythmic spasm and persistent rigidity during sleep ruled out the patient from typical stiff-man syndrome, he was supposed to have the same pathophysiological mechanism as the anti-GAD autoantibody positive stiff-man syndrome.
我们描述了一位60岁男性,他有5年胰岛素依赖型糖尿病病史,出现了躯干肌肉持续僵硬以及躯干和近端上下肢无痛性、节律性肌肉痉挛。即使在睡眠中,僵硬仍持续存在。无痛性肌肉痉挛常由随意运动和情绪刺激诱发。静脉注射地西泮可显著减轻肌肉痉挛和躯干僵硬。表面肌电图显示腹部和椎旁肌肉持续收缩。由主动颈部屈曲诱发的肩部、肱三头肌、肋间肌、腹肌、椎旁肌和股四头肌的节律性阵挛性痉挛与典型的僵人综合征不符。在该患者的血清和脑脊液中检测到了抗谷氨酸脱羧酶(GAD)抗体。他口服丙戊酸钠后病情好转。虽然无痛、节律性痉挛以及睡眠期间的持续僵硬排除了该患者患有典型僵人综合征,但推测他与抗GAD自身抗体阳性的僵人综合征具有相同的病理生理机制。