Fukushima K, Inoue Y, Fujiwara T, Yagi K
National Epilepsy Center, Shizuoka Higashi Hospital, Urushiyama, Japan.
Epilepsia. 1998;39 Suppl 5:50-4. doi: 10.1111/j.1528-1157.1998.tb05151.x.
We retrospectively analyzed the long-term clinical profiles of 47 patients with West syndrome (WS) associated with tuberous sclerosis (TS) the follow-up study for >10 years showed that WS developed into epilepsies of various types in all patients. Their final diagnoses were symptomatic generalized epilepsy (SGE: 29 patients, 62%), symptomatic partial epilepsy (PE: 14 patients, 30%), and undetermined epilepsy (UE: 4 patients, 8%). Eighty-five percent of all patients continued to show the same types of epilepsy that they had at their first examination, but in 15% the diagnosis had to be revised at the end of the follow-up period. The outcome of seizures was unfavorable for the SGE group. In the PE group, however, seizures were controlled in 9 (64%) of the 14 patients, and disturbances of daily activity were mild. In patients with WS associated with TS, the seizure outcome and social activity varied depending on the type of epilepsy or epileptic syndrome that developed from WS. The complicated clinical developments from WS with TS multiple cortical tubers related to epileptogenesis in addition to impairment of brain development.
我们回顾性分析了47例结节性硬化症（TS）相关的韦斯特综合征（WS）患者的长期临床资料。超过10年的随访研究表明，所有患者的WS均发展为各种类型的癫痫。他们的最终诊断为症状性全身性癫痫（SGE：29例，62%）、症状性部分性癫痫（PE：14例，30%）和未定型癫痫（UE：4例，8%）。所有患者中有85%在首次检查时所患癫痫类型在随访结束时仍未改变，但有15%的患者在随访期结束时诊断需修正。癫痫发作的转归对SGE组不利。然而，在PE组中，14例患者中有9例（64%）癫痫发作得到控制，日常活动障碍较轻。在TS相关的WS患者中，癫痫发作的转归和社会活动因WS发展而来的癫痫类型或癫痫综合征而异。除脑发育受损外，TS合并多个皮质结节的WS复杂的临床进展与癫痫发生有关。
