title: Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty. Report of two cases.
abstract: Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control. Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively. After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions. Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.
authors.affiliation: Department of Neurosurgery, Karl-Franzens University, Graz, Austria. Frank.Unger@lkh-graz.or.at
authors.forename: F
authors.initials: F
authors.lastname: Unger
authors.forename: O
authors.initials: O
authors.lastname: Schröttner
authors.forename: K
authors.initials: K
authors.lastname: Haselsberger
authors.forename: E
authors.initials: E
authors.lastname: Körner
authors.forename: R
authors.initials: R
authors.lastname: Ploier
authors.forename: G
authors.initials: G
authors.lastname: Pendl
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D004827:Epilepsy / Q000209:etiology*; D004829:Epilepsy, Generalized / Q000209:etiology; D004833:Epilepsy, Temporal Lobe / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006222:Hamartoma / Q000601:surgery*; D006801:Humans; D007027:Hypothalamic Diseases / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008297:Male; D011629:Puberty, Precocious / Q000209:etiology*; D016634:Radiosurgery / Q000379:methods; D012307:Risk Factors; D012449:Safety; D012919:Social Behavior; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants