Deonna T W, Roulet E, Fontan D, Marcoz J P
Centre Hospitalier Universitaire Vaudois, Pediatric Department, Lausanne, Switzerland.
Neuropediatrics. 1993 Apr;24(2):83-7. doi: 10.1055/s-2008-1071519.
The authors report three children who suffered temporary oromotor or speech disturbances as focal epileptic manifestations within the frame of benign partial epilepsy of childhood with rolandic spikes and review similar cases described in the literature. The deficit can occur as an initial symptom of the disorder without visible epileptic seizures and interferes in a variable way with simple voluntary oromotor functions or complex movements including speech production, depending on the exact location and spread of the discharging epileptic focus around the perisylvian region. The most severe deficit produces the anterior operculum syndrome. More subtle non-linguistic deficits such as intermittent drooling, oromotor apraxia or dysfluency, as well as linguistic ones involving phonologic production, can occur. The rapidity of onset, progression and recovery of the deficit is very variable as well as its duration and presumably reflects the degree of epileptic activity. In some cases, rapid improvement with antiepileptic medication occurs and coincidence between the paroxysmal EEG activity (which is usually bilateral) and the functional deficit is seen. The clinical and EEG profile of the seizures disorder and the dynamic of the deficit in these cases bear a strong resemblance to what is seen in the acquired epilepsy-aphasia syndrome (Landau and Kleffner). The variations in clinical symptoms appear more related to the main site, local extension and bilaterality of the epileptic foci rather than a basic difference in physiopathology.
作者报告了三名儿童,他们在伴有中央颞区棘波的儿童良性部分性癫痫框架内,出现了暂时的口面部运动或言语障碍作为局灶性癫痫表现,并回顾了文献中描述的类似病例。这种功能缺陷可作为该疾病的初始症状出现,而无明显癫痫发作,并根据放电性癫痫病灶在外侧裂周围区域的确切位置和扩散情况,以不同方式干扰简单的随意口面部运动功能或包括言语产生在内的复杂运动。最严重的功能缺陷会导致前岛盖综合征。可能会出现更细微的非语言性缺陷,如间歇性流口水、口面部失用症或言语不流畅,以及涉及语音产生的语言性缺陷。功能缺陷的起病、进展和恢复速度以及持续时间差异很大,大概反映了癫痫活动的程度。在某些情况下,抗癫痫药物治疗后会迅速改善,且可观察到阵发性脑电图活动(通常为双侧性)与功能缺陷之间的一致性。这些病例中癫痫发作疾病的临床和脑电图特征以及功能缺陷的动态变化与获得性癫痫性失语综合征(Landau和Kleffner综合征)所见情况极为相似。临床症状的差异似乎更多地与癫痫病灶的主要部位、局部扩展和双侧性有关,而非生理病理学上的根本差异。