Lennox-Gastaut syndrome

被引:6
|
作者
Archila, R
Papazian, O
机构
[1] Miami Childrens Hosp, Dept Neurol, Clin Neurophysiol Lab, Neurophysiol Clin, Miami, FL 33155 USA
[2] Cent Univ Venezuela, Caracas, Venezuela
关键词
electrical stimulation of the vagus nerve; epileptic encephalopathy of children; ketogenic diet; lamotrigine; Lennox-Gastaut syndrome; mental retardation; refractory epileptic seizures; topiramate;
D O I
10.33588/rn.2904.99149
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction. The Lennox-Gestaut syndrome is classified as an epileptic syndrome shown by the presence of various types of generalized seizures (tonic, atonic and atypical absences) which appear at a certain age (1-8 years), with an interictal EEG showing an abnormally slow basic rhythm interrupted by slow spike-and-wave complexes (<3 Hz) and progressive mental deterioration. Development. From the aetiological point of view there are cryptogenic (25%) and symptomatic (75%) forms. There is a previous history of West syndrome in 9.4-30% of the symptomatic cases. The commonest types of seizures are tonic (17-95%), atypical absences (17-60%) and atonic (25-56%). The mixed form of an epileptic stare with typical absences and tonic seizures is the most frequent (27%). Follow-up studies show that in 90% and 100% of cryptogenic and symptomatic patients, respectively, mental retardation develops and the initial seizures persist in 67% and 45% of the patients with cryptogenic and symptomatice forms respectively, when they become adults. Conclusions. There is still no successful treatment for these seizures and progressive mental deterioration occurs even when using the newer anti-epileptic drugs. Electrical stimulation of the vagus nerve seems a promising possibility but further experience is necessary.
引用
收藏
页码:346 / 349
页数:4
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