Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy

Palmini, André; Chandler, Christopher; Andermann, Frédérick; Costa, Jaderson Costa da; Paglioli‐Neto, Eliseu; Polkey, Charles E.; Rosenblatt, Bernard; Montes, José L.; Martínez, J.; Farmer, Jean Pierre; Sinclair, Benjamin; Aronyk, Keith; Paglioli, Eliseu; Coutinho, Lígia Maria Barbosa; Raupp, Sérgio Fernando; Portuguez, Mirna Wetters

doi:10.1212/wnl.58.9.1338

Cited by 172 publications

(195 citation statements)

References 33 publications

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“…These results were later replicated at the Barrow Neurological Institute. 23 In 2002, Palmini et al 27 reported on 13 patients who underwent surgery for HHs from 3 centers, 12 using a frontotemporal approach and 1 using an endoscopic approach. Delalande and Fohlen 9 reported on 17 patients in 2003 who underwent surgery for HH (14 transsylvian and 9 endoscopic approaches).…”

Section: The Royal Children's Hospital Experiencementioning

confidence: 99%

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The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

FOC

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The prognosis for patients with hypothalamic hamartoma has improved dramatically over the last 20 years, for 3 main reasons. First, because of improved understanding of the anatomy and pathophysiology of these varied lesions. Second, due to advances in brain imaging and refinements in microsurgery, including the anterior transcallosal interforniceal approach, endoscopic, and skull-base approaches. And third, because of increasing experience with stereotactic radiosurgery, interstitial radiotherapy, and radiofrequency lesioning. Patients with hypothalamic hamartoma should be managed in comprehensive epilepsy centers where the treatments are individualized and concentrated in the hands of surgeons who can perform the full range of surgery, including approaches to the third ventricle. Total seizure-freedom rates of 52% to 66% have been achieved with surgery.

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Section: The Royal Children's Hospital Experiencementioning

confidence: 99%

“…9, 27 Rekate et al 29 reported the early outcome of 44 patients with normal-sized ventricles who underwent endoscopic surgery. Fourteen patients had complete endoscopic removal of the HH, and there was total control of seizures in 13 of these patients.…”

Section: The Endoscopic Approachmentioning

confidence: 99%

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

FOC

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“…The hypothesis of an intrinsic epileptogenesis of HH gained impetus after the stereo-EEG demonstration of ictal onsets originating from the hypothalamic lesion, 32 and was further corroborated by subsequent reports showing production of typical gelastic seizures by depth electrode stimulation, ictal SPECT studies demonstrating HH hyperperfusion, and interictal spike source analysis. 4,9,11,12,16,21,22,26,35,45 The epileptogenic activity of HHs has been recently demonstrated at the cellular level: detailed electrophysiological analysis of slices taken from surgical specimens showed the predominance of small GABAergic inhibitory neurons exhibiting an intrinsic "pacemaker-like" behavior. 54 The concept that surgical intervention should mainly target the HH was not easy to accept.…”

Section: Pathophysiology: Intrinsic Epileptogenesis Of Hhsmentioning

confidence: 99%

Radiosurgery for hypothalamic hamartomas

Romanelli

Muacevic²,

Striano

2008

FOC

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✓ Radiosurgery plays an important role in the treatment of refractory seizures induced by hypothalamic hamartomas (HHs). These lesions, deeply located and surrounded by a delicate vascular and neuronal network, are often associated with catastrophic epilepsy leading to progressive cognitive and behavioral deterioration. Surgical approaches include microsurgical resection, endoscopic resection or disconnection, radiofrequency lesioning, and interstitial brachytherapy. Radiosurgery is an emerging treatment modality for HHs, which provides excellent seizure outcomes and no lasting complications to date.

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“…Despite occasional suggestions of a cortical origin for this activity, surgical results based on these findings were uniformly poor (Cascino et al, 1993). The few cases with a good outcome for epilepsy were all submitted to surgical resection of the hamartoma (Nishio et al, 1989(Nishio et al, , 1994Valdueza et al, 1994;Rosenfeld et al, 2001;Palmini et al, 2002).…”

Section: Introductionmentioning

confidence: 99%

Interictal spike EEG source analysis in hypothalamic hamartoma epilepsy

Leal

Passão²,

Calado

et al. 2002

Clinical Neurophysiology

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Objective: The epilepsy associated with the hypothalamic hamartomas constitutes a syndrome with peculiar seizures, usually refractory to medical therapy, mild cognitive delay, behavioural problems and multifocal spike activity in the scalp electroencephalogram (EEG). The cortical origin of spikes has been widely assumed but not specifically demonstrated.Methods: We present results of a source analysis of interictal spikes from 4 patients (age 2-25 years) with epilepsy and hypothalamic hamartoma, using EEG scalp recordings (32 electrodes) and realistic boundary element models constructed from volumetric magnetic resonance imaging (MRIs). Multifocal spike activity was the most common finding, distributed mainly over the frontal and temporal lobes. A spike classification based on scalp topography was done and averaging within each class performed to improve the signal to noise ratio. Single moving dipole models were used, as well as the Rap-MUSIC algorithm.Results: All spikes with good signal to noise ratio were best explained by initial deep sources in the neighbourhood of the hamartoma, with late sources located in the cortex. Not a single patient could have his spike activity explained by a combination of cortical sources.Conclusions: Overall, the results demonstrate a consistent origin of spike activity in the subcortical region in the neighbourhood of the hamartoma, with late spread to cortical areas. q

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Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy

Abstract: Resection can alleviate both the seizures and the behavioral and cognitive abnormalities of hypothalamic hamartomas, but complications are frequent.

Cited by 172 publications

References 33 publications

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Radiosurgery for hypothalamic hamartomas

Interictal spike EEG source analysis in hypothalamic hamartoma epilepsy

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