Hypothalamic hamartoma presents with precocious puberty, epilepsy or both. There are two epileptic syndromes, one presenting initially in infancy with gelastic seizures evolving rapidly into a syndrome with multiple seizures, developmental delay and a moderate to severe behaviour disorder. The other presents later with a milder epileptic syndrome, again usually including gelastic seizures, but with normal intellect and behaviour. Magnetic resonance imaging identifies and gives a detailed anatomical picture of these lesions. Direct surgery, using microsurgical techniques and neuronavigation guidance has been used for these lesions. Three surgical approaches have been used, one lateral pterional, another midline frontal through the lamina terminalis and a third is a transcallosal interforniceal approach. In addition a disconnection procedure, usually pterional, aims to disconnect the lesion without the risks of major resection. The transcallosal interforniceal approach is the most successful with 69% of patients seizure-free. There are complications in about 24% of patients, the same as other approaches, but the complications are milder and include fewer neurological deficits than the other routes. Alternate strategies include stereotactic radiosurgery and radiofrequency ablation under stereotactic control.
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Epilepsia
January 2025
Department of Neuropediatrics and Muscular Disorders, Medical Center, Faculty of Medicine University of Freiburg, University of Freiburg, Freiburg im Breisgau, Germany.
Objective: Hypothalamic hamartomas (HHs) are associated with pharmacoresistant epilepsy. Stereotactic radiofrequency thermocoagulation (SRT) shows promise as a disconnecting intervention. Although magnetic resonance imaging (MRI) is typically used to determine the attachment and intervention side, it presents challenges in cases of bilaterally attached HH, where the epileptogenic side is unclear.
View Article and Find Full Text PDFIntroduction Pediatric hemifacial spasm (HFS) is rare, presenting early in infancy, and often fraught with subsequent psychomotor and intellectual deficits. Fourth ventricular hamartoma (FVH) is a rare cause of HFS with only 5 cases reported in literature. While Gamma-knife radiosurgery (GKRS) has been used to treat hypothalamic hamartomas, this is the first case of FVH treated with primary GKRS.
View Article and Find Full Text PDFNeurol Sci
December 2024
Neurology Unit, Department of Neurosciences, Azienda Ospedaliera Universitaria Integrata, Verona, Italy.
Background: Drug-resistant epilepsy (DRE) secondary to hypothalamic hamartoma (HH) often requires surgical resection or stereotactic radiosurgery, which frequently fail to provide satisfactory outcomes and are associated with severe side effects. Magnetic resonance-guided focused ultrasound (MRgFUS) may represent a minimally invasive surgical approach to HH by offering precise thermal ablation of sub-millimetric brain targets while sparing surrounding structures.
Methods: We present the case of a 19-year-old man with HH-associated DRE, who was successfully treated with MRgFUS.
J Pediatr Endocrinol Metab
December 2024
Pediatric Endocrinology Clinic, Bilkent City Hospital, Ankara, Türkiye.
Objectives: Hypothalamic hamartoma (HH) is a rare condition that causes epilepsy and central precocious puberty (CPP) at an early age. In this report, we describe a child with CPP secondary to HH and discuss the current literature.
Case Presentation: A 26-month-old girl was brought to our hospital for evaluation of breast enlargement.
J Neurosurg
December 2024
3Department of Neurosurgery, Niigata Seiro Hospital, Seiro, Niigata, Japan.
Objective: Since the recent development of stereotactic ablation surgery, which can provide good seizure outcomes without limitations in size or location, conventional classification systems have become unsuitable for surgical guidance. The present study aimed to evaluate the validity of a newly proposed classification system focusing on the attachment pattern.
Methods: This retrospective study investigated 218 patients with hypothalamic hamartomas who underwent MRI-guided stereotactic radiofrequency thermocoagulation and were followed for at least 1 year after their last surgery.
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