Orphanet: Syndrome congenital d hamartome hypothalamique. Request PDF on ResearchGate | On Jul 1, , J. Mounach and others published Puberté précoce liée à un hamartome hypothalamique. Request PDF on ResearchGate | On May 1, , N. Ech-Cherif El Kettani and others published Hamartome hypothalamique révélant des crises gélastiques.

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J Korean Neurosurg Soc. Therefore, one stage operation or single modality of treatment will lose their effectiveness uamartome managing HHs An endoscopic procedure is performed under the general endotracheal anesthesia. There should be minimal working space within the third ventricle for forceps and coagulator, as well as definite interface either on the neuronavigational image or direct visualization.

Orphanet: Syndrome congenital d hamartome hypothalamique

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. Careful planning and a stepwise approach with the advanced surgical technique will make it possible 24hyplthalamique National Center for Biotechnology InformationU.

EEG and video-EEG seizure monitoring has limited utility in patients with hypothalamic hamartoma and epilepsy. Repeat surgery for hypothalamic hamartoma in refractory epilepsy. However, surgical resection for total removal of HHs have led to good seizure outcomes and behavioral improvement 10 Hamartoome per the Law relating to information storage and personal integrity, you have the right to oppose art 26 of that lawaccess art 34 of that hmaartome and rectify art 36 of that law your personal data.

Role of the hypothalamic hamartoma hamartoke the genesis of gelastic fits a video-stereo-EEG study Electroencephalogr Clin Neurophysiol. The disconnection will begin at the border between the hamartomas and the mammillary body and proceed along the midline posteroinferior floor of the third ventricle Fig.

Puberté précoce liée à un hamartome hypothalamique – EM|consulte

Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages. Access to the full text of this article requires a subscription. Thus, the authors performed an endoscopic disconnection only for the intraventricular type of hamartoma Type III. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.


Translation of “hypothalamus hamartome” in English

Other types of seizure, cognitive deterioration, and behavioral problems follow and frequently develop late in the first decade of life 4 Neuronavigation and the depth electrode will guide the depth of disconnection.

Surgical approaches to hypothalamic hamartomas. Because these patients are mostly clinically and socially impaired, pursuing resection or disconnection would be haamrtome beneficial for these patients Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification.

The direction will be posterior to anterior and lateral to inferior midline Fig. You can move this window by clicking on the headline. Access to the full text of this article requires a subscription. The presence of these neurons constitute relevant evidence for ictogenesis of HH 3918 Regis cB. Surgical indications are intractable GS, neurobehavioral deterioration, and the absence of a cortical lesion on MRI.

Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. Alternatively, hypothalamiqhe surgery, gamma knife surgery, and stereotactic radiofrequency thermo-coagulation have been introduced and presented good outcomes with fewer complications.

Therefore, concomitant scalp EEG and depth electrode recording hamratome HHs may be very useful in disconnecting this lesion from intractable epilepsy. Epilepsy, behavioral, and hormonal problems originate from the sessile type and precocious puberty are mostly derived from the pedunculated type 13437 Fig.

The HHs are divided into four categories based on MR imaging findings, demonstrating the relationship between the hamartoma and hypothalamus or the third ventricle.

The fiberoptic laser or monopolar coagulator hypothalamisue used through the working channel of hypothalamlque sheath. Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: When the thin pial membrane or arachnoid membrane is reached, dissection is complete. Because most of the patients have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure.

Under direct visualization, the hamartoma can be coagulated by a monopolar electric coagulator or removed by microforceps Fig.

Endoscopic Treatment of Hypothalamic Hamartomas

Treatment modality for namartome epilepsy in hypothalamic hamartomatous lesions. Top of the page – Article Outline. Hammartome epileptiform discharges arise from the broad attachment side 31 However, hypothalaique intraventricular HHs are excellent candidates for endoscopic resection or disconnection 823 Routine scalp EEG monitoring and long-term video-EEG monitoring, interictal and ictal 99 m-Tc hexamethylprophyleneamine oxime single photon emission computed tomography SPECTmagnetic resonance imaging MRIneuropsychological evaluation, ophthalmological assessments with perimetry, and endocrinological investigations are included.


A monopolar-coagulating electrode or an Nd-Yag laser system, a fiberoptic light guide, an Xe light source, and an endovision system are other supporting instruments. Instrumentation and procedure for endoscopic surgery The rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2.

The confines of the protruding hamartoma and the normal hypothalamus can be easily determined by direct visualization; however, for difficult cases, the depth electrode is inserted prior to endoscopic surgery to guide the depth and the margin of disconnection.

This rare non-neoplastic abnormal mixture of neuronal and glial tissue of the inferior hypothalamus has been postulated to be derived from the mammillo-thalamo-cingulate tract from which HH is networking other brain areas associated with GS or the pathway from the HH to the brainstem and cerebellum 16 This syndrome disappeared spontaneously within 10 days 8. Bourazza aH.

This system conveys discharges through the motor cortex and pyramidal tract to the ventral brainstem 11 Stereotactic Laser Ablation for Hypothalamic Hamartoma. Epileptic network of hypothalamic hamartoma: Top of the page – Article Outline. J Neuropathol Exp Neurol.

Support Center Support Center. The hanartome of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. Complications Transient short-term memory loss, weight gain, thalamic infarction with memory loss, and hemiparesis have been reported as early complications.