中国实用儿科杂志

中国实用儿科杂志 ›› 2011, Vol. 26 ›› Issue (11): 846-.

• 论著 • 上一篇    下一篇

口周肌阵挛伴失神临床及脑电图特征2例分析?

  

  1. 首都医科大学附属北京儿童医院神经内科与康复中心，北京　100045
  • 出版日期:2011-11-06 发布日期:2011-11-23

Clinical and electroencephalographic characteristics of perioral myoclonia with absences

  1. Neurology and Rehabilitation Center，Beijing Children’s Hospital Affiliated to Capital University of Medical Sciences，Beijing 100045，China
  • Online:2011-11-06 Published:2011-11-23

摘要：

摘要：目的　探讨口周肌阵挛伴失神（PMA）的临床和脑电图特征，以提高对本病的认识。方法　对2010年5月首都医科大学附属北京儿童医院收治的2例PMA患儿临床资料进行分析。结果　2例患儿均为学龄前儿童，2～4岁起病，临床发作以口周肌肉节律性抽动为突出表现，伴双眼凝视，不同程度意识障碍，持续时间短暂为数秒至十余秒，发作频繁。发作期脑电图为全导同步对称3 Hz为主棘慢波放电，发作间期脑电图为全导散在或短阵片断发放的中高波幅棘波、棘慢波。两例患儿出生史、生长发育史均正常，均有癫痫家族史，1例2～3岁时有3次全身强直-阵挛发作，1例病程中有失神持续状态发作。2例患儿分别予丙戊酸钠或丙戊酸钠联合氯硝西泮治疗，服药后发作均有消失，其中1例随访10个月目前已无发作。结论　PMA是以频繁的失神和口周肌肉节律性肌阵挛为特征的特发性全面性癫痫综合征，视频脑电图对正确诊断本病有重要作用。

关键词: 口周肌阵挛, 失神, 视频脑电图

Abstract:

Abstract：Objective　To gain better understanding of the clinical characteristics and electroencephalographic features of perioral myoclonia with absences. Methods　The clinical data of 2 patients were analyzed. Results　Of the 2 patients， onset age was from two years to four years old. The prominent manifestation of seizure was perioral rhythmic myoclonia with staring of their two eyes，accompanied by impairment of consciousness varying from mild to severe. The duration of seizures was brief from seconds to more than ten seconds and with a high frequency. Ictal EEG consisted of generalized discharges of 3 Hz spikes and wave complexes predominantly，interictal EEG showed brief run of generalized spike/spikes and wave. Of the 2 patients，their personal history （ pregnancy， birth， neonatal period and psychomotor development ） was normal. Their family history was positive（aunt or uncle had epilepsy）. Of the 2 patients， one had generalized tonic-clonic seizure three times from two to three years old，and the other had absence status. The 2 patients were treated with sodium valproate or sodium valproate combined with clonazepam，and they all were seizure free after treatment with anti-epileptic drugs initially，and one patient was seizure free for ten months by now. Conclusion　Perioral myoclonia with absences is an diopathic generalized epilepsies syndrome with characteristic absence seizure and perioral rhythmic myoclonia. Video-EEG monitoring is the most important method in the diagnosis of this illness.

Key words: perioral myoclonia, absence, video-EEG