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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J Neurol Neurosurg Psychiatry. The presence of these neurons constitute relevant evidence for ictogenesis of HH 3918 Presurgical evaluation is very much the same as a phase 1 study for a medically intractable epilepsy patient.

Neuronavigation and the depth electrode will guide the depth of disconnection. Top of the page – Article Outline. Neuronavigational neuroendoscope with a high definition visualization system will never miss the perfect entry point, trajectory, and entry for the small ventricle However, disconnection from the brain for controlling intractable epilepsy would be always feasible regardless of HH size.

The rigid neuroendoscope used for endoscopic disconnection consists of a degree Hopkins pediatric telescope Karl Storz, Tuttlingen, Germany with an outside diameter of 2.

Epileptic network of hypothalamic hamartoma: 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 Fast-firing neurons are mostly in synchronous pairs with synchronous firing Careful planning and a stepwise approach with the advanced surgical technique will make it possible 24 Contact Help Who are we?

High frequency stimulation of the mamillothalamic tract for the treatment of resistant seizures associated with hypothalamic hamartoma.

Access to the PDF text. The direction will be posterior to anterior and lateral to hypothalamiquue midline Fig. Author information Article notes Copyright and License information Disclaimer. Furthermore, they did not suggest any treatment strategy for Type 2 sessile HHs. However, surgical resection for total removal of HHs have led to good seizure outcomes and behavioral improvement 10 Consequently, complete disconnection is considered to be more important than resection.


Endoscopic Treatment of Hypothalamic Hamartomas

National Center for Biotechnology InformationU. The histopathology of hypothalamic hamartomas: Intralesional inside HHs recordings have revealed that the internal epileptiform discharges mix with low-voltage activities 15hypothala,ique31 Their report concluded that, as hemispherectomy gave way to hemispherotomy because of serious complications, the complete removal of HHs for controlling seizures was not required anymore.

Contact Help Who are we? Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to hypothaoamique this data to third parties. 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.

Zerhouni aA. 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.

Baizri cH. The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. Stereotactic Laser Ablation for Hypothalamic Hamartoma.

Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. As the nearby vicinity of HHs consists of very critical structures, the utmost effort should be paid to reduce collateral damage.

Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages.

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Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications. Hypothalamic hamartomas associated with epilepsy: Disconnection of HHs from the hypothalamus with a monopolar coagulator.


Relatively small ventricle and foramen of Monro. J Hamarotme Exp Neurol.

Firing behavior and network activity of single neurons in human epileptic hypothalamic hamartoma. These classifications have been accomplished according to the symptomatology and the feasibility of treatment modalities Fig.

In contrast, the cunei, bilateral thalami, caudate nuclei, hippocampi, paracentral gyri, and the default mode network precunei and inferior lateral parietal lobules are deactivated Cognitive functioning before and after surgical resection for hypothalamic hamartoma and epilepsy. Therefore, one stage operation or single modality of treatment will lose their effectiveness in managing HHs If you are a subscriber, please sign in ‘My Account’ at hamartoe top right of the screen.

Puberté précoce liée à un hamartome hypothalamique – EM|consulte

Their classification was based on either pedunculated or sessile sagittal orientation and the treatments included open surgical removal or medicinal intervention. If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. The confines of the protruding hamartoma and the normal hypothalamus can be easily determined by direct visualization; however, for difficult cases, the depth electrode is inserted prior to endoscopic surgery to guide the depth and the margin of disconnection.

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