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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Kyu Won ShimM. You can move this window by clicking on the headline. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.

Because most of the patients have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure.

Puberté précoce liée à un hamartome hypothalamique – EM|consulte

Baizri cH. In contrast, the cunei, bilateral thalami, caudate nuclei, hippocampi, paracentral gyri, and the default mode network precunei and inferior lateral parietal lobules are deactivated Personal information regarding our website’s visitors, including their identity, is confidential. Access to the text HTML. A monopolar-coagulating electrode or an Nd-Yag laser system, hypkthalamique 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 hypothalxmique an outside diameter of 2.

Contact Help Who are we? But even though HHs attach to the bilateral hypothalamus, unilateral disconnection is usually enough. Since Choi et al.

Advances and Technical Standards in Hypothalakique.


Three of four patients with postoperative disconnection-like syndrome mental dullness, verbal anomia, unilateral tactile anomia, and lack of somesthetic transfer were observed by Choi et al. As 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 law and rectify art 36 of that law your personal data.

Please review our privacy policy. J Korean Neurosurg Soc. Neurol Med Chir Tokyo ; After confirmation of the extent of disconnection and seizure outcome, a second disconnection can be performed with fewer complications.

Firing behavior and network activity of single neurons in human epileptic hypothalamic hamartoma. Recent electroencephalography-functional magnetic resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated.

Hamartoe Transient short-term memory loss, weight gain, thalamic infarction with memory loss, and hemiparesis have been reported as early complications.

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 The direction will be posterior to anterior and lateral to inferior midline Fig.

Intrinsic epileptogenesis of hypothalamic hamartom in gelastic epilepsy. Endoscopic disconnection has been one of the most favorable options hhpothalamique in treating HHs. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy. Thorough preoperative investigation and the hypothalamiaue of intraoperative stereotactic and electrophysiological hamratome would ensure this goal Acute intralesional recording in hypothalamic hamartoma: The rate of resection was not correlated to the seizure-free condition EEG and video-EEG seizure monitoring has limited utility in patients with hypothalamic hamartoma and epilepsy.


Electric recording through depth electrode and transendoscopic intraoperative recording are also capable of recording the epileptic activities of HHs 21 The first step of disconnection with monopolar coagulator along the lateral border of HHs.

Top of the page – Article Outline. Endoscopic disconnection for hypothalamic hamartoma with intractable seizure. Support Center Support Center. Patients with most unilateral attachment, intraventricular type, and small HHs less than 1. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

More favorable outcomes were guaranteed mostly from near total or total resection Because breaking down the tract for spreading epileptiform discharge from HHs to the frontotemporal cortical region will be the optimal solution for this lesion, further aggressive maneuvers may not be necessary 19 Utility of early single photon emission computed hypothalamiqque SPECT in neonatal gelastic epilepsy associated with hypothalamic hamartoma.

Therefore, concomitant scalp EEG and depth electrode hypohtalamique inside HHs may be very useful in disconnecting this lesion from intractable epilepsy. Access to the text HTML.

Endoscopic Treatment of Hypothalamic Hamartomas

Neurosurg Clin N Am. Hypothalamic hamartoma with refractory epilepsy: Depth electrode insertion into the HH with EEG monitoring would provide the margin and depth of disconnection.

Thus, the authors performed an endoscopic disconnection only for the intraventricular type of hamartoma Type III.