Oguni H, Hayashi K, Osawa M
Department of Pediatrics, Tokyo Women's Medical College, Japan.
Epilepsia. 1996;37 Suppl 3:44-7. doi: 10.1111/j.1528-1157.1996.tb01820.x.
We investigated the long-term prognosis of Lennox-Gastaut syndrome (LGS) in 72 patients followed up for > 10 years. Long-term seizure and intellectual outcomes were poor, as previously reported. The diagnosis of LGS was first made in the age range from 2 to 15 years with peak occurrence at 5 years. Progressive IQ score deterioration with age was apparent. At the last examination, 33% of patients with cryptogenic and 55% with symptomatic LGS had lost the characteristics of LGS, and their seizure disorders were classifiable as symptomatic generalized epilepsies, severe epilepsy with multiple independent spike foci, or localization-related epilepsies. Disabling drop attacks appeared in 46% of patients and tended to occur at older than 10 years. Gait deterioration was recognized in 12 patients and seemed to be due largely to progression of the epileptic encephalopathy. The gait disturbances, as well as increased frequency of violent drop attacks, were disabling in daily life and resulted in some patients being wheelchair bound.
我们对72例随访时间超过10年的Lennox-Gastaut综合征(LGS)患者的长期预后进行了调查。正如之前报道的那样,长期癫痫发作和智力预后较差。LGS的诊断首次出现在2至15岁的年龄范围内,发病高峰在5岁。随着年龄增长,智商分数逐渐下降的情况很明显。在最后一次检查时,33%的隐源性LGS患者和55%的症状性LGS患者已不再具有LGS的特征,他们的癫痫发作类型可归类为症状性全身性癫痫、具有多个独立棘波灶的严重癫痫或局灶性相关性癫痫。46%的患者出现致残性跌倒发作,且往往发生在10岁以上。12例患者出现步态恶化,似乎主要是由于癫痫性脑病的进展所致。步态障碍以及剧烈跌倒发作频率的增加,在日常生活中造成了残疾,导致一些患者需要依靠轮椅行动。