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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Outline Masquer le plan. Endocrine assessment would include sex hormone and growth hormone levels, thyroid function, prolactin level, and cortisol reserve with glucagon stimulation.

Hypothalamic hamartomas associated with epilepsy: You hyppothalamique move this window by clicking on the headline. Delalande O, Fohlen M. However, surgical resection for total removal of HHs have led to good seizure outcomes and behavioral improvement 10 Villeneuve abV.

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 approaches to hypothalamic hamartomas. Contact Help Who are we?

Access to the full text of this article requires a subscription. Autonomic and hormonal htpothalamique changes in gelastic seizures from 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.


Endoscopic Treatment of Hypothalamic Hamartomas

Hypothalamic hamartoma and seizures: Electric recording through depth electrode and transendoscopic intraoperative recording are also capable of recording the epileptic activities of HHs 21 Abstract Hypothalamic hamartoma HH is a benign indolent lesion despite the presentation of refractory epilepsy.

Access to the PDF text. Because these patients are mostly clinically and socially impaired, pursuing resection or disconnection would be more beneficial for these patients Since the hypothalamic area is critical itself, even though we must achieve a good seizure outcome, we should avoid critical complications. The presence of these neurons constitute relevant hypothalxmique for ictogenesis of HH 3918 Transendoscopic intraoperative recording of gelastic seizures from a hypothalamic hamartoma.

Endoscopic disconnection for hypothalamic hamartoma with intractable seizure.

The rate of resection was not correlated to the seizure-free condition Recently stereotactic laser ablation has been introduced and good seizure control has been achieved.

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

Acute intralesional recording in hypothalamic hamartoma: Le syndrome hamartome hypothalamique: Kyu Won ShimM. Repeat surgery for hypothalamic hamartoma in refractory epilepsy.

Endoscopic Treatment of Hypothalamic Hamartomas

An optimal surgical approach should be selected based on the individual status of the lesion and the patient Fig. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.


J Neuropathol Exp Neurol. These classifications have been accomplished according to the symptomatology and the feasibility of treatment modalities Fig.

Complete removal of larger or giant HHs is very difficult or nearly impossible with acceptable morbidity. High frequency stimulation of the mamillothalamic tract for the treatment of resistant seizures associated with hypothalamic hamartoma. 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.

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After confirmation of the extent of disconnection and seizure outcome, hamratome second disconnection can be performed with fewer complications. Top of the page – Article Outline. Please review our privacy policy. Therefore, one stage operation or single modality of treatment will lose their effectiveness in managing HHs Because small HHs are often attached unilaterally, unilateral disconnection will be enough.

Fast-firing neurons are mostly in synchronous pairs with synchronous firing

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