Ohtsuka Y, Ohmori I, Oka E
Department of Child Neurology, Okayama University, Medical School, Japan.
Epilepsia. 1998 Nov;39(11):1158-63. doi: 10.1111/j.1528-1157.1998.tb01306.x.
To study the clinical and electroencephalographic (EEG) characteristics of patients whose epilepsy is associated with tuberous sclerosis, with special reference to their clinical course.
We investigated the electroclinical and radiologic features of 38 patients with epilepsy associated with tuberous sclerosis.
Eleven patients showed only generalized epilepsy, and 10 showed only localization-related epilepsy throughout their clinical course. Among the other 17 cases, the nature of the epilepsy changed between generalized and localization-related epilepsies during the clinical course. A shift from generalized to localization-related epilepsies was more common than the reverse. Seventeen had West syndrome (WS), three had Lennox-Gastaut syndrome (LGS), and eight had epilepsies that evolved from WS to LGS. Tonic spasms, mostly in series, were seen in all 28 patients with generalized epilepsy. Eleven of the 28 patients had partial seizures and tonic spasms in the same period. Six of them showed "simultaneous seizures," consisting of tonic spasms in series and a partial seizure. Partial seizures were the main seizure type in 27 patients with localization-related epilepsy, but three of them also showed tonic spasms that included "simultaneous seizures." Ictal EEGs revealed multiple active foci in the same period that could shift during the clinical course. Neither the location nor number of tubers was related to the clinical course. As for seizure outcome, 12 (32%) of 38 patients were free from seizures at follow-up.
In epilepsies associated with tuberous sclerosis, there may be an interrelation between generalized and localization-related epilepsies, as well as one between generalized and partial seizures.
研究癫痫与结节性硬化症相关患者的临床及脑电图（EEG）特征，并特别关注其临床病程。
我们调查了38例癫痫与结节性硬化症相关患者的电临床和放射学特征。
11例患者在整个临床病程中仅表现为全身性癫痫，10例仅表现为局灶性相关性癫痫。在其他17例患者中，癫痫的性质在临床病程中在全身性癫痫和局灶性相关性癫痫之间发生了变化。从全身性癫痫转变为局灶性相关性癫痫比相反的情况更常见。17例患有West综合征（WS），3例患有Lennox-Gastaut综合征（LGS），8例癫痫从WS演变为LGS。28例全身性癫痫患者均出现强直性痉挛，多为连续发作。28例患者中有11例在同一时期出现部分性发作和强直性痉挛。其中6例表现为“同步发作”，包括连续强直性痉挛和部分性发作。部分性发作是27例局灶性相关性癫痫患者的主要发作类型，但其中3例也出现了包括“同步发作”在内的强直性痉挛。发作期脑电图显示同一时期有多个活跃病灶，且在临床病程中可能发生变化。结节的位置和数量均与临床病程无关。关于癫痫发作结果，38例患者中有12例（32%）在随访时无癫痫发作。
在与结节性硬化症相关的癫痫中，全身性癫痫和局灶性相关性癫痫之间可能存在相互关系，全身性癫痫和部分性发作之间也可能存在相互关系。
