Hypothalamic hamartoma in adults

Abstract

Objective To study the clinical features of hypothalamic hamartoma (HH) in adults. Methods A retrospective review of 27 cases of hypothalamic hamartoma in adults was performed, which account for 12.62% in all 214 hypothalamic hamartoma patients diagnosed in Beijing Tiantan Hospital from 1994 to 2008. Results The mean age of the patients diagnosed as hypothalamic hamartoma was 29.22 years old ranged from 18 to 66 years old, the rate of male to female was 2∶1. Eight patients (29.63%) were asymptomatic and 19 patients (70.37%) were symptomatic. The onset of symptom developed before adult life were found in 14 patients (73.68%), the other 5 patients (26.32%) developed a milder epileptic seizures in adult life which ranged from 19 to 51 years old. Most patients presented grand mal epilepsy. Hypothalamic hamartoma was diagnosed by the combination of medical history with CT and MRI which presented space occupying lesion at interpeduncular cistern or in the third ventricle. The first symptom presented in 19 cases of symptomatic patients were as follow: precocious puberty in 3 cases (15.79% ), gelastic seizure in 9 patients (47.37%), absence seizure in 2 patients (10.52%) and generalized tonic⁃clonic seizure in 5 patients (26.32%). Two patients combined with mental retardation. Among the 9 patients with gelastic seizure, 2 patients had generalized tonic⁃clonic seizure at the time of onset of gelastic seizure, 6 patients presented with generalized tonic⁃clonic seizure at the mean time of 10.70 years (ranged from 6 months to 21 years) after the onset of gelastic seizure, one patient presented with gelastic seizure only. All the patients performed CT and MRI, and the time of follow⁃up was 14 months to 9 years and 10 months. Six patients underwent resection of hypothalamic hamartoma through pterional approach or callosal⁃septum pellucidum⁃interforniceal approach. Three cases were cured (Engel class Ⅰ). Seizures were almost free in 2 cases (Engel classⅡ). Seizures were not changed in one case (Engel class Ⅴ). Two patients underwent γ⁃knife surgery: one case showed no change at seizure onset and another one showed reduction of seizure frequency (Engel class Ⅳ). Antiepileptic drugs were used in 9 patients, but no effect was seen on gelastic seizure. Conclusion Adults with hypothalamic hamartoma usually present with mild epilepsy and seldom occur intellectual disturbance. Treatment should be given with caution.

DOI：10.3969/j.issn.1672-6731.2010.03.019