Clemens B
County Hospital, Neurological Department, Debrecen, Hungary.
Brain Dev. 1997 Jul;19(5):353-8. doi: 10.1016/s0387-7604(97)00568-8.
The author presents clinical and electroencephalographic data of an epileptic girl, whose absences started at the age of 2. Based on seizure semiology, serial EEG and video-EEG recording, 7 years of follow-up and other clinical data, the patient was diagnosed as having the PMA ('perioral myoclonus with absences') syndrome. There were some disagreement between clinical and EEG data, however. Finally, she became completely seizure free on valproate+lamotrigine medication. Detailed EEG and voltage mapping analysis disclosed that spike movements of ictal and interictal generalized spike-wave discharges showed always the same, very consistent, peculiar topographic pattern. This pattern was fundamentally different from spike patterns associated with typical absence seizures (recorded in other patients). Analysis of clinical, EEG and voltage mapping results-suggest that actually this case might be rather classified as myoclonic epilepsy. The conjunction of this case to the PMA syndrome has been discussed.
作者介绍了一名癫痫女孩的临床和脑电图数据，其失神发作始于2岁。基于发作症状学、系列脑电图和视频脑电图记录、7年的随访以及其他临床数据，该患者被诊断为患有PMA（“伴有失神的口周肌阵挛”）综合征。然而，临床和脑电图数据之间存在一些分歧。最后，她在丙戊酸盐+拉莫三嗪药物治疗下完全无癫痫发作。详细的脑电图和电压图谱分析显示，发作期和发作间期全身性棘波-慢波放电的棘波运动始终呈现相同、非常一致的特殊地形模式。这种模式与典型失神发作（在其他患者中记录）相关的棘波模式根本不同。对临床、脑电图和电压图谱结果的分析表明，实际上该病例可能更应归类为肌阵挛性癫痫。本文讨论了该病例与PMA综合征的关联。
