Rodriguez-Rodriguez S, Salas-Puig J, Alvarez-Carriles J C, Temprano-Fernandez T, Anton-Gonzalez C, Garcia-Martinez A
Hospital Universitari Vall d'Hebron, 08035 Barcelona, Espana.
Rev Neurol. 2011 Mar 1;52(5):257-63.
Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy that starts in childhood and has an unknown pathophysiological mechanism. Studies on the progress of LGS reveal a poor prognosis.
A retrospective study of 1629 patients with epilepsy was conducted. Patients included in the study were those with an age of onset during childhood, tonic seizures and atypical absences (who might also suffer from other types of seizures); electroencephalogram (EEG) showing generalised slow spike-wave and generalised fast activity; mental retardation and a follow-up time of at least 10 years.
The sample consisted of 12 patients, 9 of whom were males. Follow-up time: 20 years. Onset of seizures: 3 years. Cryptogenic LGS: 67%; symptomatic: 33%. Frequency of the seizures at onset: daily (83.3%) or weekly (16.7%). Types of seizures: tonic and atypical absences (100%); drop-attacks (83%); generalised tonic-clonic (75%); myoclonias (41.7%); partial (8.3%) and pseudo-epileptic (8.3%). EEG with slow background activity, generalised slow spike-wave and generalised fast activity: 100%. Fifty percent of the patients had at least one epileptic status. They used an average of 7.5 different antiepileptic drugs. At the end of the follow-up 33% were suffering from seizures on a daily basis; 17% were weekly and 42% monthly. A total of 8.3% were free from seizures. All of them were following combination therapy: 17% in bitherapy and 83% with an average of 3.8 drugs (range: 3-5). A total of 92% suffered from severe or very severe mental retardation. Fifty percent required neuroleptic drugs due to behavioural disorders.
Despite the fact that the diagnosis of LGS is serious, we observed a decrease in the number of seizures after several years of development, although the antiepileptic combination therapy remains constant. The mental retardation and behavioural disorders lead to a poor functional prognosis.
Lennox-Gastaut综合征（LGS）是一种始于儿童期的癫痫性脑病，其病理生理机制不明。对LGS进展的研究显示预后较差。
对1629例癫痫患者进行了回顾性研究。纳入研究的患者为儿童期起病、有强直发作和不典型失神发作（也可能有其他类型发作）、脑电图（EEG）显示广泛性慢棘慢波和广泛性快活动、智力发育迟缓且随访时间至少10年的患者。
样本包括12例患者，其中9例为男性。随访时间：20年。癫痫发作起始年龄：3岁。隐源性LGS：67%；症状性：33%。发作起始时的发作频率：每日发作（83.3%）或每周发作（16.7%）。发作类型：强直发作和不典型失神发作（100%）；跌倒发作（83%）；全身强直阵挛发作（75%）；肌阵挛发作（41.7%）；部分性发作（8.3%）和假性癫痫发作（8.3%）。脑电图显示背景活动缓慢、广泛性慢棘慢波和广泛性快活动：100%。50%的患者至少有一次癫痫持续状态。他们平均使用7.5种不同的抗癫痫药物。随访结束时，33%的患者每天发作；17%的患者每周发作，42%的患者每月发作。共有8.3%的患者无癫痫发作。所有患者均采用联合治疗：17%为两种药物联合，83%平均使用3.8种药物（范围：3 - 5种）。共有92%的患者患有重度或极重度智力发育迟缓。50%的患者因行为障碍需要使用抗精神病药物。
尽管LGS的诊断很严重，但我们观察到经过数年发展后发作次数有所减少，尽管抗癫痫联合治疗保持不变。智力发育迟缓和行为障碍导致功能预后较差。
