Gross-Selbeck G
Kinderneurologisches Zentrum, Kliniken der Landeshauptstadt, Düsseldorf.
Monatsschr Kinderheilkd. 1992 Aug;140(8):451-6.
Idiopathic epilepsies with generalized seizures of early childhood are based on a genetic predisposition. The onset takes place between the first and fifth years of age, boys are affected more often than girls. Dependent on the clinical symptomatology you have to distinguish: myoclonic seizures; atonic-astatic seizures; myoclonic-astatic seizures; absences; tonic-clonic seizures. In more than half of the cases a combination of these seizures can be observed. The differentiation of epilepsies with generalized seizures of multifocal origin (infantile spasms, Lennox-Gastaut syndrome and Pseudo-Lennox syndrome [atypical benign epilepsy]) may be difficult but is essential. Therapy of choice is valproate, often in combination with ethosuximide (in children with minor seizures) or with kaliumbromide or phenobarbital (in children with tonic-clonic seizures). Generally the prognosis is more unfavourable if epilepsy starts in the first year of life with afebrile and febrile generalized tonic-clonic or clonic seizures, if children are suffering from longlasting states of seizures and if development is disturbed before beginning of epilepsy.
儿童期起病的特发性全身性癫痫基于遗传易感性。发病年龄在1至5岁之间，男孩比女孩更易受累。根据临床症状学，必须区分：肌阵挛发作；失张力-无动性发作；肌阵挛-无动性发作；失神发作；强直-阵挛发作。超过半数病例可观察到这些发作类型的组合。多灶性起源的全身性癫痫（婴儿痉挛症、Lennox-Gastaut综合征和假性Lennox综合征[非典型良性癫痫]）的鉴别可能困难，但至关重要。首选治疗药物是丙戊酸盐，通常与乙琥胺（用于发作轻微的儿童）或溴化钾或苯巴比妥（用于强直-阵挛发作的儿童）联合使用。一般而言，如果癫痫在出生后第一年起病，伴有无热和发热性全身性强直-阵挛或阵挛发作，儿童患有长期癫痫发作状态，以及在癫痫发作开始前发育受到干扰，则预后更差。
