ABSTRACT
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.
