Blatter-Arifi V
Abteilung für Epileptologie und Elektroenzophalographie, Neurologische Universitätsklinik, Inselspital, Bern.
Schweiz Rundsch Med Prax. 1991 Sep 3;80(36):909-18.
In 1989/90 a follow-up was made possible on 72 of 78 patients who have been treated for the supposed or confirmed diagnosis of a Lennox-Gastaut-Syndrome at the university children hospital of Berne between 1964 and 1978. Nine patients were excluded of this study because the diagnosis was proved wrong retrospectively, leaving 63 cases. Of these, eleven patients (17.5%) have died. The remaining 52 (82.5%) were evaluated regarding their epilepsy, psychomotor development and social adaptation. The follow-up was good for 14.3%, intermediate for 23.8% and poor for 44.4%. Bad prognostic factors were found to be: first manifestation of epilepsy during the first year of life, occurrence of infantile spasms or hypsarrhythmia in the EEG and pathological neurological signs at the beginning of the disease. In the course of illness a change of seizure phenomenology was observed. The infantile spasms were seen only during the first three years of epilepsy. After the second year of disease psychomotor seizures became more and more frequent. Atypical absences, already seen at the beginning, were the most frequent form of seizure from the third year of epilepsy until the end of our observations. During the course of disease the frequency of generalized tonic and tonic-clonic seizures decreased little.
1989年至1990年期间，对1964年至1978年间在伯尔尼大学儿童医院因疑似或确诊为Lennox-Gastaut综合征而接受治疗的78名患者中的72名进行了随访。9名患者因诊断被回顾性证明错误而被排除在本研究之外，剩下63例。其中，11名患者（17.5%）已经死亡。对其余52名患者（82.5%）的癫痫、精神运动发育和社会适应情况进行了评估。随访结果良好的占14.3%，中等的占23.8%，差的占44.4%。发现不良预后因素为：癫痫在出生后第一年首次出现、脑电图出现婴儿痉挛或高度失律以及疾病开始时出现病理性神经体征。在疾病过程中观察到发作现象学的变化。婴儿痉挛仅在癫痫发作的前三年出现。疾病第二年过后，精神运动性发作越来越频繁。一开始就出现的非典型失神发作，从癫痫发作第三年到我们观察结束时是最常见的发作形式。在疾病过程中，全身性强直和强直阵挛发作的频率略有下降。
