HYPOTHALAMIC HAMARTOMAS - WITH SPECIAL REFERENCE TO GELASTIC EPILEPSYAND SURGERY

THIS STUDY PRESENTS six patients with hypothalamic hamartomas diagnose d on the basis of magnetic resonance imaging. Histological confirmatio n was performed in three patients who underwent surgery. Immunohistolo gical assays were used to determine the neurosecretory pattern. Four p atients presented with epilepsy, including gelastic seizures. Other sy mptoms included behavioral abnormalities in four patients and precocio us puberty and visual impairment in two patients. One patient presente d associated developmental defects. Coed results without morbidity wer e achieved with surgical resectioning in two patients with large hamar tomas associated with behavioral abnormalities and gelastic epilepsy t hat was unresponsive to conventional medical treatment and in one pati ent with visual impairment. We propose a classification of the hypotha lamic hamartomas based on topographical and clinical data obtained fro m 36 selected cases in the literature and six of our own cases. This c lassification should help to classify the various treatment methods an d the surgical risks into four subgroups (Types Ia, Ib, IIa, and IIb). We conclude that the surgical approach is a realistic alternative in certain cases, including large and broad-based Type IIb hamartomas ass ociated with gelastic epilepsy and behavioral disorders.