Wirrell Elaine, Farrell Kevin, Whiting Sharon
Department of Pediatrics, University of Calgary, AB, Canada.
Can J Neurol Sci. 2005 Nov;32(4):409-18. doi: 10.1017/s0317167100004388.
The epileptic encephalopathies comprise a group of devastating seizure syndromes which begin in infancy and early childhood and usually result in intractable epilepsy. While some syndromes are relatively easily diagnosed early in their course, others take time to evolve, hampering an early, confident diagnosis. Epileptic encephalopathies are associated with slowing of cognitive function and evolution of severe behavioral disorders, which are often more distressing to families than the epilepsy. While an underlying etiology may explain some of this co-morbidity, many children have no identifiable etiology found for their seizures. In these "idiopathic" cases, recurrent subtle seizures, frequent epileptiform discharge and non-convulsive status epilepticus probably all play a role in deterioration of cognitive function and evolution of behavior disorders. This paper will review the most common epileptic encephalopathy syndromes, discuss the cognitive and behavioral co-morbidities and review current therapeutic options.
癫痫性脑病是一组严重的癫痫综合征，始于婴儿期和儿童早期，通常导致难治性癫痫。虽然有些综合征在病程早期相对容易诊断，但其他综合征需要时间演变，这妨碍了早期的确切诊断。癫痫性脑病与认知功能减退和严重行为障碍的发展有关，这些对家庭来说往往比癫痫更令人痛苦。虽然潜在病因可能解释部分这种共病情况，但许多儿童的癫痫发作没有可识别的病因。在这些“特发性”病例中，反复出现的轻微癫痫发作、频繁的癫痫样放电和非惊厥性癫痫持续状态可能都在认知功能恶化和行为障碍发展中起作用。本文将回顾最常见的癫痫性脑病综合征，讨论认知和行为共病情况，并回顾当前的治疗选择。
