Cusmai R, Chiron C, Curatolo P, Dulac O, Tran-Dinh S
Instituto di Neuropsichiatria Infantile, Universita La Sapienza, Roma, Italy.
Epilepsia. 1990 Nov-Dec;31(6):747-55. doi: 10.1111/j.1528-1157.1990.tb05516.x.
A series of 34 children with confirmed tuberous sclerosis (TS) were studied prospectively by both EEG and magnetic resonance imaging (MRI) at ages ranging from 5 months to 18 years. Size and topography of the cortical tubers were analyzed on axial and coronal views, in T2 sequences, and the large tubers greater than 10 mm were studied. In addition, EEG follow-up data were reviewed retrospectively. Twenty-six patients (76%) had both MRI large cortical tubers and EEG foci, 3 had normal EEG, 2 had normal MRI, and the remaining 3 had only small tubers. The number of large tubers was significantly related to EEG foci. A topographic MRI/EEG correspondence was observed for at least one tuber in 25 of the 26 patients, and correspondence was complete in 10 patients. Large cortical tubers without corresponding EEG foci were observed in 11 patients; these tubers mainly involved the frontal regions and were found before the patients were 2 years old. EEG foci without corresponding tubers were observed in 4 patients. In addition, secondary bilateral synchrony was preferentially observed in patients with frontal lesions and after the age of 2 years. These data confirm that the cortical tubers are epileptogenic and that their expression may be influenced by regional cortical maturation.
对34例确诊为结节性硬化症(TS)的儿童进行了前瞻性研究,研究年龄范围为5个月至18岁,采用脑电图(EEG)和磁共振成像(MRI)两种方法。在T2序列的轴位和冠状位视图上分析皮质结节的大小和位置,并对大于10mm的大结节进行研究。此外,对EEG随访数据进行了回顾性分析。26例患者(76%)既有MRI大皮质结节又有EEG病灶,3例EEG正常,2例MRI正常,其余3例只有小结节。大结节的数量与EEG病灶显著相关。26例患者中有25例至少有一个结节观察到MRI/EEG位置对应,10例患者对应完全。11例患者观察到无相应EEG病灶的大皮质结节;这些结节主要累及额叶区域,且在患者2岁前发现。4例患者观察到无相应结节的EEG病灶。此外,额叶病变患者和2岁以后的患者优先观察到继发性双侧同步。这些数据证实皮质结节具有致痫性,其表达可能受区域皮质成熟的影响。