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首页> 外文期刊>Epilepsia: Journal of the International League against Epilepsy >Hypothalamic hamartoma and seizures: a treatable epileptic encephalopathy.
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Hypothalamic hamartoma and seizures: a treatable epileptic encephalopathy.

机译：下丘脑错构瘤和癫痫发作：可治疗的癫痫性脑病。

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摘要

Hypothalamic hamartomas may be associated with gelastic seizures, focal seizures, and a generalized epileptic encephalopathy, with severe seizures and cognitive and behavior decline. Despite earlier views to the contrary, good evidence now exists that all these clinical features are caused, directly or indirectly, by the hamartoma. Resection of these lesions was long regarded as too hazardous and unlikely to benefit seizure control. It is now clear that hypothalamic hamartomas can be effectively treated with a variety of surgical approaches with sustained seizure control and often seizure freedom. Qualitative observations suggest that behavior and cognition also improve with treatment, but quantitative validation is required. The specific approach should be tailored according to the surgical anatomy of the lesion and the experience of the surgeon. Choices include a transcallosal approach (good for intraventricular lesions), a pterional approach (useful for interpeduncular lesions), a transventricular endoscopic approach, or destruction of the lesion with radiofrequency probes or gamma knife radiosurgery. The previously dismal outlook for children with severe seizures associated with this lesion has now dramatically changed. These insights may have implications for other epileptic encephalopathies of childhood.
机译：下丘脑错构瘤可能与全能性癫痫发作，局灶性癫痫发作和全身性癫痫性脑病有关，伴有严重的癫痫发作以及认知和行为下降。尽管先前有相反的观点，但是现在有充分的证据表明所有这些临床特征都是由错构瘤直接或间接引起的。长期以来，这些病变的切除术被认为过于危险，不太可能有利于控制癫痫发作。现在很清楚，下丘脑错构瘤可以用各种手术方法有效地治疗，持续控制癫痫发作，并且通常具有癫痫发作的自由度。定性观察表明，行为和认知也随治疗而改善，但需要定量验证。具体方法应根据病变的外科解剖结构和外科医生的经验来定制。选择包括经call动脉入路（对脑室内病变有益），ter肌入路（对椎间盘间病变有用），经脑室内窥镜入路，或使用射频探头或伽马刀放射外科手术破坏病变。先前对于与该病灶相关的严重癫痫发作的儿童的前景令人沮丧，现在已经发生了巨大变化。这些见解可能对儿童期的其他癫痫性脑病有影响。

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