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.

Author: Mosida Gamuro
Country: Mali
Language: English (Spanish)
Genre: Life
Published (Last): 13 August 2006
Pages: 101
PDF File Size: 8.18 Mb
ePub File Size: 3.23 Mb
ISBN: 287-6-34123-239-6
Downloads: 22754
Price: Free* [*Free Regsitration Required]
Uploader: JoJom

Based on their shape and relationship to the yhpothalamique, there are several classifications of HHs and the symptoms and severity depend on their dimensions e. 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.

The mass hypothqlamique not be under the optic tract. Thorough preoperative investigation and the use of intraoperative stereotactic and electrophysiological guidance would ensure this goal The HHs are divided into four categories based on MR imaging findings, demonstrating the relationship between the hamartoma and hypothalamus or the third ventricle.

Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction.

Endoscopic Treatment of Hypothalamic Hamartomas

Treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions. Endoscopic disconnection has been one of the most favorable options performed in treating HHs.

CT, MR, and pathologic findings. The rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2. Recent electroencephalography-functional hypothalakique resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated.

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. Stereotactic Laser Ablation for Hypothalamic Hamartoma. Most epileptiform discharges arise from the broad attachment side 31 Ictogenesis and symptomatogenesis of gelastic seizures in hypothalamic hamartomas: Contact Help Who are we?


Neurol Med Chir Tokyo ; Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy. Memory, endocrine function, behavior, and vision are potentially at risk after surgery 782327 Patients with most unilateral attachment, intraventricular type, and small HHs less than 1. Hamarrtome confines of the protruding hamartoma and the normal hypothalamus can be easily determined by direct visualization; however, for difficult cases, hypothalmique depth electrode is inserted prior to endoscopic surgery to guide the depth and the margin of disconnection.

Endoscopic approach to hypothalamic hamartoma Hypohtalamique.

Clear visualization of a distinct cleft or indentation and the border connecting HH to the hypothalamus will be the critical point for performing staged endoscopic disconnection for giant HHs. The first step of disconnection with monopolar coagulator along the lateral border of HHs.

Translation of “hypothalamus hamartome” in English

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.

The depth electrode will guide the disconnection surgery as well as confirm the successful disconnection 28 Electrophysiological properties of human hypothalamic hamartomas. 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.

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

Abstract Hypothalamic hamartoma HH is a benign indolent lesion despite the presentation of refractory epilepsy. Access to the full text of this article requires a subscription. However, there are known factors to achieving good outcomes with minimal complications 10 Outline Masquer le plan.


Access to the PDF text.

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

Laguitton aM. Surgical outcomes Valdueza et al.

Endoscopic Treatment of Hypothalamic Hamartomas

The telescope with the sheath is then advanced into the lateral ventricle through the peel-away catheter with irrigation of isotonic solution. 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.

Utility of early single photon emission computed tomography SPECT in neonatal gelastic epilepsy associated with hypothalamic hamartoma.

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 Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification.

J Neuropathol Exp Neurol. 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.

Hypothalamic hamartoma with refractory epilepsy: Alternatively, endoscopic surgery, gamma knife surgery, and stereotactic hy;othalamique thermo-coagulation have been introduced and presented good outcomes with fewer complications.