Hypothalamic hamartoma

Hypothalamic hamartoma (HH) is a rare developmental tumor which has a benign histology and does not progressively enlarge. Pedunculated HH attached to the tuber cinereum may cause precocious puberty whereas sessile HH which have intraventricular and intrahypothalamic components cause gelastic epilepsy and behavioral disorders. Large HH with involvement of both mammillary and tuberal regions tend to manifest with neurologic and endocrine problems [6,7,9]. Gelastic epilepsy is generally resistant to antiepileptic drugs (AED), ketogenic diet, and vagal nerve stimulation. The epilepsy syndrome that results may be progressive and devastating for the patient and their family. The localization of seizures can be deceptive, with some patients having had temporal lobectomy or frontal corticectomy without any effect on the seizures [3,6,17]. There is now good evidence that the epilepsy in these patients emanates from the HH [3].