This study presents seven patients with hypothalamic hamartomas diagnosed on the basis of MRI. Histological confirmation was performed in one patient who underwent surgery. Four patients presented with epilepsy, including gelastic seizures. Other symp...
This study presents seven patients with hypothalamic hamartomas diagnosed on the basis of MRI. Histological confirmation was performed in one patient who underwent surgery. Four patients presented with epilepsy, including gelastic seizures. Other symptoms were behavior abnormalities in 3 patients and precocious puberty in 4 patients. We classify hypothalamic hamartomas into four subgroups according to MRI finding : Types Ⅰa lesions were less than 10㎜ in diameter and pedunculaedly attached to tuber cinereum of hypothalamus without hypothalamic displacement. Type Ⅰb lesions less than 10㎜ and mamillary body. Type Ⅱa lesions were more than 10㎜ in diameter and sessilely attached to hypothalamus with slight hypothalmic displacement and Type Ⅱb lesions more than 10㎜ and with marked displacement of hypothalamus. We could achieve good result with surgical resection in one patient with Type Ⅱb hamartoma associated with gelastic seizure that was unresponsive to medical treatment. We performed gamma knife ragiosurgery in three patients with gelastic seizure(2 patients with Type Ⅱa and 1 patient with Type Ⅱb) and three patients with precocious puberty(2 patients with Type Ⅰa and 1 patient with Type Ⅰb). The authors propose direct surgery as a treatment for this progressive syndrome and gamma knife radiosurgery as alternative treatment for high risk patients.