AI Article Synopsis
- An 18-year-old boy with refractory epilepsy and aggression due to a hypothalamic hamartoma underwent a stereotactic thermocoagulation procedure aimed at disconnecting the hamartoma from key brain structures.
- The surgical planning utilized advanced imaging techniques, integrating MRI with CT and a digital brain atlas to accurately target the lesion.
- Post-surgery, the patient showed significant improvements, with reduced aggression and a dramatic decrease in seizure frequency, with benefits maintained over 24 months.
Article Abstract
An 18-year-old boy with refractory epilepsy and aggressiveness associated to a hypothalamic hamartoma was submitted to a stereotactically guided lesion by thermocoagulation. The target was based on magnetic resonance (MR) images merged with computed tomography scan images taken on the day of surgery while patient was on a stereotactic frame. In order to reveal structures not discernible in MR images, the Schaltenbrand digital brain atlas was merged onto the patient's images. Target and trajectory of the depth electrode were chosen based on three-dimensional imaging reconstructions. A surgical plan was devised to disconnect the hypothalamic hamartoma from the hypothalamus, medial forebrain bundle, fasciculus princeps, and dorsal longitudinal fasciculus. Our target was placed at the inferior portion of the posterolateral component of the hamartoma, bordering the normal hypothalamus. The patient evolved with marked lessening of aggressiveness. Seizure frequency was reduced from several seizures per day to less than one tonic-clonic seizure during sleep per month and only two episodes suggestive of partial complex seizures during daytime. These results have remained consistent over a 24-month postoperative follow-up. Functional neuroanatomy of hypothalamic connections involved in seizure propagation and aggressive behavior was reviewed.
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Introduction 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 PDFMRgFUS disconnection surgery for hypothalamic hamartoma-related epilepsy: case report and literature review.
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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.
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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.
A proposed new classification system of hypothalamic hamartomas in the era of stereotactic ablation surgery.
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3Department of Neurosurgery, Niigata Seiro Hospital, Seiro, Niigata, Japan.
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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.
Outcome of Surgery for Hypothalamic Hamartoma-Related Epilepsy: A Systematic Review and Individual Participant Data Meta-Analysis.
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From the Department of Medicine (F.N.), Université de Montréal, Québec, Canada; David Geffen School of Medicine at the University of California, Los Angeles (K.G., A.F.); Warren Alpert Medical School of Brown University (J.-S.C.), Providence, RI; Division of Neurology (A.H.), Department of Pediatrics, Sainte-Justine University Hospital Centre, Montréal; Brain and Development Research Axis (A.H., A.G.W.), Centre de Recherche Azrieli du CHU Sainte-Justine, Montréal; Department of Neuroscience (M.R.K., D.K.N.), Université de Montréal; Research Centre of the University of Montreal Hospital Center (CRCHUM) (M.R.K., D.K.N.); Division of Neurology (M.R.K., D.K.N.), University of Montreal Hospital Center (CHUM); Laboratoire d'Imagerie Optique en Neurodéveloppement (LIONlab) (A.G.), CHU Sainte-Justine Research Center, Montréal; Cerebrum (A.G.), Department of Psychology, Université de Montréal, Québec, Canada; Department of Neurological Surgery (N.S.), Northwestern University Feinberg School of Medicine, Chicago, IL; Neuroscience Advanced Clinical Imaging Service (NACIS) (J.Y.-M.Y.), Department of Neurosurgery, The Royal Children's Hospital; Department of Paediatrics (J.Y.-M.Y.), The University of Melbourne; Neuroscience Research (J.Y.-M.Y.), Murdoch Children's Research Institute, Melbourne, Australia; Hope for Hypothalamic Hamartomas Organization (L.S., E.W.); APHM (B.D., D.S.), Timone Hospital, Epileptology and Cerebral Rhythmology, Marseille, France; Jane and John Justin Institute for Mind Health (M.S.P.), Cook Children's Health Care System, Fort Worth, TX; Division of Neurosurgery (K.M., G.M.I.), Hospital for Sick Children; Neurosciences & Mental Health (K.M., G.M.I.), SickKids Research Institute, Toronto, Ontario, Canada; Center for Neuroscience Research (W.D.G.), and Department of Neurology (W.D.G.), Children's National Hospital, George Washington University School of Medicine, Washington, DC; Department of Neurosurgery (D.M.), Université de Sherbrooke, Quebec, Canada; Pediatric Neurology Division (J.F.K.), Barrow Neurological Institute at Phoenix Children's Hospital, AZ; and Department of Neurosurgery (A.F.), David Geffen School of Medicine at the University of California, Los Angeles; Division of Neurosurgery (A.G.W.), Department of Surgery, Sainte-Justine University Hospital Centre, Montréal; Division of Neurosurgery (A.G.W.), Department of Surgery, University of Montreal Hospital Center (CHUM), Montréal, Québec, Canada.
Background And Objectives: There is a paucity of data directly comparing the outcome of surgical techniques available for the treatment of hypothalamic hamartomas (HHs). This study aims to evaluate the safety and efficacy of commonly used surgical approaches in the treatment of HH-related epilepsy.
Methods: A systematic review and individual participant data (IPD) meta-analysis was conducted.
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