Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study)

Munari, C.; Kahane, Philippe; Francione, Stefano; Hoffmann, D.; Tassi, Laura; Cusmai, Raffaella; Vigevano, Federico; Pasquier, B; Betti, Osvaldo

doi:10.1016/0013-4694(95)00063-5

Cited by 295 publications

(217 citation statements)

References 9 publications

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“…Ictal and interictal epileptic abnormalities localized in the temporal and frontal lobes are frequent and lead many epileptologists to believe that gelastic (and other) seizures originate from independent neocortical foci. 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.…”

Section: Pathophysiology: Intrinsic Epileptogenesis Of Hhsmentioning

confidence: 79%

“…Electrophysiological characterization of HH neurons has shown that an intrinsic epileptogenic activity is present. 4,5,21,22,32,54 Epileptogenic HH are invariably attached to one or both of the mammillary bodies. 15 Spread of the epileptogenic activity and induction of generalized seizures require access to the thalamocortical loops via the mammillothalamic tract.…”

mentioning

confidence: 99%

“…15 Spread of the epileptogenic activity and induction of generalized seizures require access to the thalamocortical loops via the mammillothalamic tract. 5,15,21,32 Several surgical approaches have been proposed for the treatment of epileptogenic HHs, including microsurgical resection via the transcallosal interforniceal route or the pterional or subfrontal translamina terminalis route, use of radiosurgery in children, offering a totally noninvasive option devoid of major complications.…”

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confidence: 99%

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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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Section: Pathophysiology: Intrinsic Epileptogenesis Of Hhsmentioning

confidence: 79%

mentioning

confidence: 99%

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confidence: 99%

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Radiosurgery for hypothalamic hamartomas

Romanelli

Muacevic²,

Striano

2008

FOC

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“…The good results from surgery strongly implied the hamartoma as the source of the ictal activity and its intrinsic epileptogenicity was confirmed with recordings from deep electrodes placed inside the lesion (Munari et al, 1995).…”

Section: Introductionmentioning

confidence: 84%

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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“…17,23 Moreover, the symptoms elicited by stimulation of the malformation demonstrated a specific distribution of the epileptogenic areas within the epileptic foci. 17,23 In the 2 cases reported by Khan et al, 19 the EEG findings were not reported, and the unilateral stimulation was effective in reducing the seizure frequency in both cases. This study demonstrates indirectly the findings of Munari and Kahane and their colleagues, and confirms the role of the invasive study for the correct mapping of the epileptic network of the hamartoma.…”

Section: Discussionmentioning

confidence: 99%

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

Marras

Rizzi²,

Villani

et al. 2011

FOC

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Hypothalamic hamartomas (HHs) are developmental malformations associated with a range of neurological problems, including intractable seizures. There is increasing evidence of the epileptogenicity of the hamartoma and of the inhomogeneous distribution of the epileptic abnormalities within the malformation. The management strategy for treatment and results differ according to the insertion plane and the extension of the malformation into the hypothalamus. Cases characterized by extensive involvement of the hypothalamus are particularly challenging. The authors describe the case of a patient with drug-resistant epilepsy and a large hypothalamic hamartoma with an extensive area of attachment. The patient underwent implantation of 2 deep brain electrodes. The intraoperative recording showed a synchronous interictal epileptic discharge in the left temporal lobe and on the left side of the lesion. The patient was treated with chronic high-frequency stimulation. No side effects due to the stimulation were reported. At 18 months' follow-up, a reduction in complex partial seizure frequency was reported, but no significant reduction in overall seizure frequency was noticed (p = 0.14, t-test). The authors report on neurophysiological studies of the relationship between HH and epilepsy, and also discuss the literature on chronic high-frequency stimulation, including its rationale and the results of chronic stimulation of various targets for the treatment of drug-resistant epilepsy due to HH.

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Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study)

Cited by 295 publications

References 9 publications

Radiosurgery for hypothalamic hamartomas

Radiosurgery for hypothalamic hamartomas

Interictal spike EEG source analysis in hypothalamic hamartoma epilepsy

Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma

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