Yamamoto K, Fukusako T, Nogaki H, Morimatsu M
Department of Neurology, Yamaguchi University School of Medicine.
Rinsho Shinkeigaku. 1992 Nov;32(11):1261-5.
A 51-year-old woman was admitted to our hospital because of gait disturbance and dysuria. Neurological examination revealed limb and truncal ataxia, orthostatic hypotension, cogwheel rigidity in all limbs, generalized hyperreflexia without pathological reflex, and horizontal gaze nystagmus. She became progressively worse and bedridden at age 52. Then she developed abnormal eye movements. Electrooculogram revealed vertical, horizontal or oblique macro square wave jerks and pendular nystagmus. Macro square wave jerks appeared during fixation and disappeared with eye closure or in the dark room. Macro square wave jerks were characterized by a duration of about 200 msec and an amplitude of 10 to 15 degrees. Pendular nystagmus with a duration of several seconds and amplitude of 5 to 15 degrees appeared when she changed her fixation or the point of fixation disappeared. Macro square wave jerks and pendular nystagmus were mildly suppressed after the intramuscular injection of 100 mg of phenobarbital, the oral intake of sodium valproate of 600 mg/day or baclofen of 60 mg/day. They were almost completely depressed after the intravenous injection of 3 mg of diazepam or the oral intake of clonazepam of 1.5 mg/day. We suggested that both macro square wave jerks and pendular nystagmus in this patient might be caused by the dysfunction of GABAergic system in the saccadic eye movement system.
一名51岁女性因步态障碍和排尿困难入住我院。神经系统检查发现肢体和躯干共济失调、体位性低血压、四肢齿轮样强直、全身反射亢进但无病理反射以及水平凝视性眼球震颤。她在52岁时病情逐渐加重并卧床不起。随后出现异常眼动。眼电图显示垂直、水平或斜向的大方形波急跳和钟摆样眼球震颤。大方形波急跳在注视时出现，闭眼或在暗室中消失。大方形波急跳的特点是持续时间约200毫秒，幅度为10至15度。当她改变注视点或注视点消失时，会出现持续数秒、幅度为5至15度的钟摆样眼球震颤。肌肉注射100毫克苯巴比妥、口服600毫克/天丙戊酸钠或60毫克/天巴氯芬后，大方形波急跳和钟摆样眼球震颤轻度受到抑制。静脉注射3毫克地西泮或口服1.5毫克/天氯硝西泮后，它们几乎完全被抑制。我们认为该患者的大方形波急跳和钟摆样眼球震颤可能均由扫视眼动系统中γ-氨基丁酸能系统功能障碍引起。
