Fukushima K, Inoue Y, Fujiwara T, Yagi K
National Epilepsy Center, Shizuoka Higashi Hospital, 886 Urushiyama, Shizuoka 420-8688, Japan.
Brain Dev. 2001 Nov;23(7):698-704. doi: 10.1016/s0387-7604(01)00275-3.
To analyze the evolution of seizures, electroencephalographic (EEG) features, seizure outcomes, and social outcomes, in order to formulate a basis for the long-term prognosis of West syndrome (WS) associated with tuberous sclerosis (TS).
The clinical profiles of 50 patients with TS, who presented with WS in infancy and had been monitored for more than 10 years after the initial examination, were analyzed.
In 86% of the patients the seizure type did not change throughout the course of this study. However, 14% of patients had developed other types of seizures by the end of the follow-up period. The seizure outcome was unfavorable for patients with generalized seizures. Partial seizures were controlled in nine (64%) of the 14 patients, and normal or slightly subnormal intelligence accounted for seven of the 14 patients.
Therefore, in patients with WS associated with TS, the seizure outcome and social activity depended on the type of seizure that developed during the course of this disorder. The outcome of patients with WS associated with TS seems to be better than that of WS in general.
分析癫痫发作的演变、脑电图（EEG）特征、癫痫发作结局及社会结局，以便为结节性硬化症（TS）相关的韦斯特综合征（WS）的长期预后制定依据。
分析50例婴儿期出现WS且在初次检查后已接受超过10年监测的TS患者的临床资料。
在本研究过程中，86%的患者癫痫发作类型未发生变化。然而，到随访期末，14%的患者出现了其他类型的癫痫发作。全身性癫痫发作的患者癫痫发作结局不佳。14例患者中有9例（64%）的部分性癫痫发作得到控制，14例患者中有7例智力正常或略低于正常水平。
因此，在与TS相关的WS患者中，癫痫发作结局和社会活动取决于该疾病过程中出现的癫痫发作类型。与TS相关的WS患者的结局似乎总体上优于一般WS患者。
