Transcallosal Resection of Hypothalamic Hamartomas, with Control of Seizures, in Children with Gelastic Epilepsy
- 1 January 2001
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurosurgery
- Vol. 48 (1), 108-118
- https://doi.org/10.1097/00006123-200101000-00019
Abstract
OBJECTIVE: Hypothalamic hamartomas (HHs) are associated with precocious puberty and gelastic epilepsy; the seizures are often refractory to antiepileptic medications and associated with delayed development and disturbed behavior. The current opinion is that surgery to treat intrahypothalamic lesions is formidable and that complete excision is not technically achievable. We report our experience with a transcallosal approach to the resection of HHs. METHODS: Five children (age, 4–13 yr) with intractable epilepsy and HHs underwent preoperative clinical, electroencephalographic, and imaging evaluations. Two patients experienced only gelastic seizures, and three patients experienced mixed seizure disorders with drop attacks; all experienced multiple daily seizures. Patients were evaluated with respect to seizures, cognition, behavior, and endocrine status 9 to 37 months (mean, 24 mo) after surgery. The HHs were approached via a transcallosal-interforniceal route to the third ventricle and were resected using a microsurgical technique and frameless stereotaxy. RESULTS: Complete or nearly complete (>95%) excision of the HHs was achieved for all patients, with no adverse neurological, psychological, or visual sequelae. Two patients experienced mild transient diabetes insipidus after surgery. Two patients developed appetite stimulation, but no other significant endocrinological sequelae were observed. Three patients are seizure-free and two patients have experienced only occasional, brief, mild gelastic seizures after surgery, all with reduced antiepileptic medications. On the basis of parental reports and our own subjective observations, the children also exhibited marked improvements in behavior, school performance, and quality of life. CONCLUSION: Complete or nearly complete resection of HHs can be safely achieved via a transcallosal approach, with the possibility of seizure freedom and neurobehavioral improvements.Keywords
This publication has 27 references indexed in Scilit:
- Stereotactic radiofrequency ablation for the treatment of gelastic seizures associated with hypothalamic hamartomaJournal of Neurosurgery, 1999
- Stereotaxy for Hypothalamic Hamartoma with Intractable Gelastic Seizures: Technical Case ReportNeurosurgery, 1999
- Subsidence of seizure induced by stereotactic radiation in a patient with a hypothalamic hamartomaJournal of Neurosurgery, 1998
- Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsyAnnals of Neurology, 1997
- Human Epileptogenesis and Hypothalamic Hamartomas: New Lessons from an Experiment of NatureEpilepsia, 1997
- Hypothalamic HamartomasNeurosurgery, 1994
- Surgical Treatment of Intractable Seizures Due to Hypothalamic HamartomaEpilepsia, 1994
- Neurosurgical treatment of hypothalamic hamartomas causing precocious pubertyJournal of Neurosurgery, 1993
- Hypothalamic Hamartoma: Report of Two CasesNeurosurgery, 1985
- Pubertas praecox due to hypothalamic hamartoma: report of two cases surviving surgical removal of the tumour.Journal of Neurology, Neurosurgery & Psychiatry, 1967