The treatment of patients with hypothalamic hamartomas, epilepsy and behavioural abnormalities: facts and hypotheses.
The treatment of patients with hypothalamic hamartomas, epilepsy and behavioural abnormalities: facts and hypotheses.
复制标题
下丘脑错构瘤、癫痫和行为异常患者的治疗：事实和假设。
作者：
A. Palmini;E. Paglioli‐Neto;J. Montes;Jean Farmer
近年来，人们对下丘脑错构瘤（HH）、癫痫和行为异常之间的关联越来越感兴趣，这使得该实体的临床表现、病理生理学和管理方面取得了重大进展。具有这些病变的患者可能在癫痫和行为障碍的严重程度范围内占据不同的点，并且可能随着时间的推移动态地向更恶性的癫痫进展。皮质下病变在产生痉挛性发作中的作用已经被证实，手术切除、破坏或分离错构瘤也获得了令人鼓舞的结果。目前的工作突出了几个方面，应考虑到为个别患者的医疗和手术治疗的选择。我们的结论与反思，我们仍然不明白的起源和手术治疗的神经精神残疾有关的这种疾病。
The growing interest in the association between hypothalamic hamartomas (HH), epilepsy and behavioural abnormalities witnessed in recent years, has led to significant progress regarding the clinical presentations, pathophysiology and management of this entity. Patients with these lesions may occupy different points within a spectrum of severity of the epileptic and behavioural disorder, and may dynamically progress toward more malignant epilepsies with time. The role of the subcortical lesion in the generation of the gelastic seizures has been established, and encouraging results have been obtained with surgical resection, destruction or disconnection of the hamartoma. The present work highlights several aspects that should be taken into account for the selection of medical and surgical treatment for individual patients. We conclude with a reflection on what we still do not understand as regards in the genesis and surgical management of the neuropsychiatric disabilities related to this disorder.