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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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Recent electroencephalography-functional magnetic resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated. The first step of disconnection with monopolar coagulator along the lateral border of HHs. An anterior wiping motion of the endoscope could result in critical damage to the fornix, hypothalamiqhe apparatus, or hypothalamus.

Abstract Hypothalamic hamartoma HH is a benign indolent lesion despite the presentation of refractory epilepsy. Daquin aJ.

Translation of “hypothalamus hamartome” in English

Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. Platinum microwire intraoperative recording of single neuron activity captured 10—20 spikes per second in over HH neurons from 14 patients.

Surgical outcomes Valdueza et al. Intralesional inside HHs recordings have revealed that the internal epileptiform discharges mix with low-voltage activities 152231 Acceptable morbidity has made multiple-staged operations possible 2431 Neuronavigational neuroendoscope with a high definition visualization system will never miss the perfect entry point, trajectory, and entry for the small ventricle EEG and video-EEG seizure monitoring has limited utility in patients with hypothalamic hamartoma and epilepsy.


Endoscopic Treatment of Hypothalamic Hamartomas

If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. Epilepsy, behavioral, and hormonal problems originate from the sessile type and precocious puberty are mostly derived from the pedunculated type 13437 Fig. Because breaking down the tract for spreading epileptiform discharge from HHs to the hypoothalamique cortical region will be the optimal solution for this lesion, bamartome aggressive maneuvers may not be necessary 19 As the authors describe 31even giant HHs larger than 25 mm can be efficiently disconnected under guidance of a depth electrode and frameless neuronavigation system.

Surgical indications are intractable GS, neurobehavioral deterioration, and the absence of a cortical lesion on MRI. Presurgical evaluation is very much the same as a phase 1 study for a medically intractable epilepsy patient.

J Korean Neurosurg Soc. This syndrome disappeared spontaneously within 10 days 8. Personal information regarding our website’s visitors, including their identity, is confidential. Their classification was based on either pedunculated or sessile sagittal orientation and the treatments included open surgical removal or medicinal intervention. However, surgical hypothalamlque for total removal of HHs have led to good seizure outcomes and behavioral improvement hamarto,e Journal page Archives Contents list.

In contrast, the cunei, bilateral thalami, caudate nuclei, hippocampi, paracentral gyri, and the default mode network precunei and inferior lateral parietal hypothalzmique are deactivated 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.


Since Choi et al. Based on their shape and relationship to the hypothalamus, hypothalaique are several classifications of HHs and the symptoms and severity depend on their dimensions e.

Personal information regarding our website’s visitors, including their identity, is confidential. However, disconnection from the brain for controlling intractable epilepsy would be always feasible regardless of HH size.

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. CT, MR, and pathologic findings. Repeat surgery for hypothalamic hamartoma in refractory epilepsy. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

Orphanet: Syndrome congenital d hamartome hypothalamique

Neuronavigation and the depth electrode will guide the depth of disconnection. An immediate seizure-free condition was the single most reliable factor for long-term seizure outcome.

The histopathology of hypothalamic hamartomas: More favorable outcomes were guaranteed mostly from near total or total resection Depth electrode insertion into the HH with EEG monitoring would provide the margin and depth of disconnection. Neurol Med Chir Tokyo ; Advances hypothaalmique Technical Standards in Neurosurgery. In earlier studies, the seizure outcomes were found to be variable with considerable complications 72026 ,