Hashimoto S, Kawamura J, Yamamoto T, Kinoshita A, Segawa Y, Harada Y, Suenaga T
Department of Neurology, Tenri Hospital, Nara, Japan.
J Neurol Sci. 1992 Jun;109(2):132-9. doi: 10.1016/0022-510x(92)90159-i.
We report 7 patients who developed acute co-occurrences of fragmentary generalized myoclonus and asterixis. All patients were elderly and had other chronic diseases. This condition appeared acutely, progressed over several hours and then disappeared in 2-3 days with diazepam administration. No sequelae were noted, although most cases developed recurrences. The myoclonus occurred spontaneously and was slightly enhanced by action. The myoclonus was widely distributed but predominated in the neck, shoulder girdle, and upper extremities. Opsoclonus was not noted. Clinically apparent myoclonus was not evoked by sensory stimuli. Asterixis was observed in the upper extremities in all cases. Asterixis-like movements of the protruded tongue were also observed. Neurological findings other than the myoclonus and asterixis were unremarkable. Neither metabolic nor organic abnormalities clearly responsible for this condition were identified. Cerebral potentials preceding the myoclonic jerks recorded in one case suggested that the myoclonus may have been a spontaneous cortical myoclonus. We named this condition a transient myoclonic state with asterixis (TMA). Awareness of this syndrome is clinically important because of its benign nature, although it can recur.
我们报告了7例同时出现片段性全身性肌阵挛和扑翼样震颤的患者。所有患者均为老年人，且患有其他慢性疾病。这种情况急性出现，在数小时内进展，然后在给予地西泮后2 - 3天消失。尽管大多数病例会复发，但未发现后遗症。肌阵挛为自发性，动作可使其稍有增强。肌阵挛分布广泛，但以颈部、肩胛带和上肢为主。未观察到眼阵挛。感觉刺激未诱发明显的临床肌阵挛。所有病例在上肢均观察到扑翼样震颤。还观察到伸出舌头的扑翼样震颤样动作。除肌阵挛和扑翼样震颤外，神经学检查结果无明显异常。未发现明确导致这种情况的代谢或器质性异常。在1例患者中记录到的肌阵挛性抽搐之前的脑电位提示，肌阵挛可能是自发性皮质肌阵挛。我们将这种情况命名为伴有扑翼样震颤的短暂性肌阵挛状态（TMA）。尽管这种综合征可能复发，但其良性性质使其在临床上具有重要意义。
