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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3Hz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。
Objective To investigate the clinical and electroencephalographic features of perioccipitus myoclonic syndrome (PMA) in order to improve the understanding of this disease. Methods The clinical data of 2 PMA children admitted to Beijing Children’s Hospital Affiliated to Capital Medical University in May 2010 were analyzed. Results 2 cases of children were preschool children, 2 to 4 years old onset, clinical seizures with perioral muscle rhythmic tics as a prominent manifestation, with binocular gaze, varying degrees of disturbance of consciousness, the duration of a brief period of seconds to ten seconds, Frequent episodes. Attack of the EEG for the all-round synchronous symmetry 3Hz-spine slow wave discharge, the interictal EEG is all scattered scattered or short shots issued in high-amplitude wave spikes, spikes and waves. Two cases of children with a history of birth, growth and development were normal, have a family history of epilepsy, 1 case of 2 to 3 years old with 3 tonic-clonic seizures, 1 case of the absence of sustained state seizures. Two cases of children were treated with sodium valproate or sodium valproate combined with clonazepam, the seizures disappeared after taking medicine, of which 1 case was followed up for 10 months has no seizures. Conclusions PMA is an idiopathic generalized epilepsy syndrome characterized by frequent absence of denervation and perineal muscle myoclonus. Video EEG plays an important role in the correct diagnosis of this disease.