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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Role of the hypothalamic hamartoma in the genesis of gelastic fits a video-stereo-EEG study Electroencephalogr Clin Neurophysiol.

Relatively small ventricle and foramen of Monro. You can move this window by clicking on the headline.

Translation of “hypothalamus hamartome” in English

Endoscopic resection of hypothalamic hamartomas for refractory symptomatic epilepsy. Clear visualization of a distinct cleft or indentation and the border connecting HH to the hypothalamus will be the hypothalaamique point for performing staged endoscopic disconnection for giant HHs.

Transendoscopic intraoperative recording of gelastic seizures from a hypothalamic hamartoma. J Neurol Neurosurg Psychiatry. For severely affected patients, relieving intractable epilepsy would outweigh these complications and clinically significant damage to a mammillary body or fornix would not be evident.

Endoscopic Treatment of Hypothalamic Hamartomas

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.

Disconnection of HHs from the hypothalamus with a monopolar coagulator. Depth electrode insertion into the HH with EEG monitoring would provide the margin and depth of disconnection. Top of the page – Article Outline. Slit-like lateral ventricle and giant HHs within a 6-mm space of the third ventricle are no longer limitations in endoscopic disconnection Abilkassem bA. Personal information regarding our website’s visitors, including their identity, is confidential.

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

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The endoscopic disconnection may not always be possible for every HH. Regis cB.

Puberté précoce liée à un hamartome hypothalamique – EM|consulte

If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. Outline Masquer le plan. 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.

Complete removal of larger or giant HHs is very difficult or nearly impossible with acceptable morbidity. Thorough preoperative investigation and the use of intraoperative stereotactic and electrophysiological guidance would ensure this goal Complete disconnection between the HH and hypothalamus can be confirmed by observing air density along the disconnection site on postoperative brain CT scans or signal changes on MR images 78.

Consequently, complete disconnection is considered to be more important than resection. It has been well known that epileptic discharge originates from the hamartoma itself and propagates to subcortical and cortical areas.

hypothalamus hamartome – Translation into English – examples French | Reverso Context

Epilepsy, behavioral, and hormonal problems originate from the sessile type and precocious puberty are mostly derived from the pedunculated type 13437 Fig. Modern imaging techniques and subsequent state of the art technology in hypothalamiquee image guidance systems are critical to performing this delicate job Journal page Archives Contents list. Furthermore, they did not suggest any treatment strategy for Type 2 sessile HHs. GABAA receptor-mediated hamsrtome of L-type calcium channels induces neuronal excitation in surgically resected human hypothalamic hamartomas.

Surgical outcomes Valdueza et al. Because most of the patients have small ventricles, usually a neuronavigation system is set before beginning the main surgical procedure. National Center for Biotechnology InformationU. In bilateral attachment and giant HH cases, preoperative electrophysiological evaluation mostly revealed unilateral propagation of epileptiform discharge hypotjalamique Support Center Support Center.

Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al.


Fast-firing neurons are mostly in synchronous pairs with synchronous firing 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. Firing behavior and network hypotualamique of single neurons in human epileptic hypothalamic hamartoma. You can move this window by clicking on the headline. Their classification was hammartome on either pedunculated or sessile sagittal orientation and the treatments included open surgical removal or medicinal intervention.

These classifications have been accomplished according to the symptomatology and the feasibility of treatment modalities Fig. Delalande O, Fohlen M.

Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery and gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. This activating network results in epileptic discharges through the premotor and frontal opercular areas voluntary systemwhich are located on the same side of the broader attachment When the telescope has been advanced through the foramen of Monro, the HH protruding from the floor and the lateral wall of the third ventricle is usually seen Fig.

Therefore, concomitant scalp EEG and depth electrode recording inside HHs may be very useful in disconnecting this lesion from intractable epilepsy.

The rate of resection was not correlated to the seizure-free condition Repeat endoscopic disconnection can effectively rescue the recurrence of seizure. Patients with most unilateral attachment, intraventricular type, and small HHs less than 1.

The presence of these hamatrome constitute relevant evidence for ictogenesis of HH 3918 Neuronavigation and the depth electrode will guide the depth of disconnection.