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梅杰综合征:临床、药理学及放射学观察

Meige's syndrome: clinical, pharmacological and radiological observations.

作者信息

Chopra J S, Radhakrishnan K, Sridharan R

出版信息

Clin Neurol Neurosurg. 1981;83(3):145-52. doi: 10.1016/0303-8467(81)90016-0.

Abstract

The clinical pharmacological, and neuroradiological observations in six patients with spontaneous blepharospasm-oromandibular dystonia (Meige's) syndrome are recorded. This group consisted of five males and one female, mean age at onset being 50.3 years. The duration of symptoms ranged from three months to 12 years, three patients having had symptoms for over four years. The dyskinesia was arrhythmic and asymmetrical in the orbicularis oculi and masseter muscles electrophysiologically. Pharmacological studies evinced no consistent response to parenteral physostigmine, no response to oral levodopa and no significant improvement in the dyskinesia following oral haloperidol. Lumbar air encephalogram was done in five patients, and showed frontal cortical atrophy without ventricular dilation in three. It is concluded that Meige's syndrome is a distinct nosological entity, and that physostigmine test is unlikely to be helpful in the differential diagnosis from neuroleptic-induced tardive dyskinesia. Neurotransmitter imbalance in the basal ganglia in this disorder remains to be established, and at present there is no satisfactory drug treatment for this progressively disabling movement disorder.

摘要

记录了6例自发性眼睑痉挛-口下颌肌张力障碍(梅杰综合征)患者的临床药理学及神经放射学观察结果。该组包括5名男性和1名女性,平均发病年龄为50.3岁。症状持续时间从3个月至12年不等,3例患者症状持续超过4年。电生理检查显示,眼轮匝肌和咬肌的运动障碍无节律且不对称。药理学研究表明,肠胃外注射毒扁豆碱无一致反应,口服左旋多巴无反应,口服氟哌啶醇后运动障碍无明显改善。5例患者进行了腰椎气脑造影,其中3例显示额叶皮质萎缩且无脑室扩张。结论是,梅杰综合征是一种独特的疾病实体,毒扁豆碱试验在与抗精神病药物所致迟发性运动障碍的鉴别诊断中可能无帮助。该疾病中基底节神经递质失衡仍有待确定,目前对于这种逐渐致残的运动障碍尚无令人满意的药物治疗方法。

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