HYPOTHALAMIC HAMARTOMAS AND ICTAL LAUGHTER - EVOLUTION OF A CHARACTERISTIC EPILEPTIC SYNDROME AND DIAGNOSTIC-VALUE OF MAGNETIC-RESONANCE IMAGING
HYPOTHALAMIC HAMARTOMAS AND ICTAL LAUGHTER - EVOLUTION OF A CHARACTERISTIC EPILEPTIC SYNDROME AND DIAGNOSTIC-VALUE OF MAGNETIC-RESONANCE IMAGING
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DOI：
10.1002/ana.410230502
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发表时间：
1988-05-01
影响因子：
11.2
通讯作者：
GLOOR, P
中科院分区：
文献类型：
--
作者：
BERKOVIC, SF;ANDERMANN, F;GLOOR, P
通过对4例患者的详细研究和文献回顾，我们进一步描述了与下丘脑错构瘤相关的癫痫综合征，其特征是在婴儿期开始大笑发作。由于儿童早期的心理发育通常是正常的，这种情况看起来是良性的，甚至可能不会被识别出来。笑的情节是短暂的，频繁的，机械的。这些特征使它区别于其他形式的癫痫性笑声，特别是颞叶癫痫。随后，癫痫发作时间延长，出现其他类型的癫痫发作，在4至10岁之间，继发性全身性癫痫的临床和脑电图特征出现。认知能力下降，经常出现严重的行为问题。癫痫控制和社会适应的预后较差。下丘脑错构瘤伴有皮质异常。病变最好通过磁共振成像检测，但可能难以通过计算机断层扫描识别。
Detailed study of 4 patients and review of the literature allowed us to delineate further the epileptic syndrome associated with hypothalamic hamartomas, which characteristically begins in infancy with laughing seizures. Because early childhood psychomotor development is usually normal, the condition appears benign and may not even be recognized. The episodes of laughter are brief, frequent, and mechanical in nature. These features distinguish it from other forms of epileptic laughter, particularly that which occurs in temporal lobe epilepsy. Subsequently, the seizures become longer, other seizure types appear, and between the ages of 4 and 10 years, the clinical and electroencephalographic features of secondary generalized epilepsy develpe. Cognitive deterioration occurs and severe behavior problems are frequent. Prognosis for seizure control and social adjustment is poor. Cortical abnormality occurs in association with the hypothalamic hamartoma. The lesions are best detected by magnetic resonance imaging but may be difficult to identify by computed tomographic scanning.