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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Based on their shape and relationship to the hypothalamus, there are several classifications of HHs and the symptoms and severity depend on their dimensions e. 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. Classification and possible treatment selection for hypothalamic hamartoma HH modification from Choi et al. For severely affected patients, relieving intractable epilepsy would outweigh these complications and clinically significant damage to a mammillary body or hypothlamique would not be evident.
Complete removal of larger or giant HHs is very difficult or nearly impossible with acceptable morbidity. In earlier studies, the seizure outcomes were found to be variable with considerable complications 72026 Villeneuve abV.
Translation of “hypothalamus hamartome” in English
Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction. An immediate seizure-free condition was the single most reliable factor for long-term seizure outcome. Zerhouni aA. An anterior wiping motion of the endoscope could result in critical damage to the fornix, optic apparatus, or hypothalamus. Intralesional inside HHs recordings have hamartoje that the internal epileptiform discharges mix with low-voltage activities 152231 Drop attacks and other secondary generalized epilepsy symptoms usually develop as the patient ages.
Puberté précoce liée à un hamartome hypothalamique – EM|consulte
The direction will be posterior to anterior and lateral to inferior midline Fig. Discrete appearance of HHs to show the interface between HHs and the hypothalamus.
From hypothalamic hamartoma to cortex: Neuronavigation and the depth electrode will guide the depth of disconnection. This syndrome disappeared spontaneously within 10 days 8. Presurgical evaluation is very much the same as a phase 1 study for a medically intractable epilepsy patient. The yamartome electrode will guide the disconnection surgery as well as confirm the successful disconnection 28 Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma.
Role of the hypothalamic hamartoma in the genesis of gelastic fits ha,artome video-stereo-EEG study Electroencephalogr Clin Neurophysiol. Recent electroencephalography-functional magnetic resonance image EEG-fMRI studies revealed that the ipsilateral hypothalamus, brainstem tegmentum, and contralateral cerebellum are activated. The rate of resection was not correlated to the seizure-free condition The disconnection will begin at the border between the hamartomas and the mammillary body and proceed along the midline posteroinferior floor of the third ventricle Fig.
Endoscopic surgery for hypothalamic hamartomas causing medically refractory gelastic epilepsy. You can move this window hypothalamiqu clicking on the headline. But even though HHs attach to the bilateral hypothalamus, unilateral hypothalamiqje is usually enough.
Access to the PDF text. Compulsive bursts of laughter are a typical and classical feature of GS 13 Endoscopic disconnection for hypothalamic hamartoma with intractable seizure.
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. Their report concluded that, as hemispherectomy gave way to hemispherotomy because of serious complications, the complete removal of HHs for hamartomd seizures was not required anymore. The fiberoptic laser or monopolar coagulator is used through the working channel of the sheath.
Recently stereotactic laser ablation has been introduced and good seizure control has been achieved. Surgical indications are intractable GS, neurobehavioral deterioration, and the absence of a cortical lesion on MRI.
Acute intralesional recording in hypothalamic hamartoma: You can move this window by clicking on the headline.