Hypothalamic hamartomas and gelastic epilepsy

Tasch, Edwin; Cendes, Fernando; Li, L. M.; Dubeau, François; Montes, José L.; Rosenblatt, Bernard; Andermann, F.; Arnold, D. L.

doi:10.1212/wnl.51.4.1046

Cited by 44 publications

(32 citation statements)

References 8 publications

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“…The relative intensities of the NAA/Cr and the NAA/Cho ratios were not significantly different from those found in the temporal lobes of healthy volunteers. Within the HH, the NAA/Cho ratio was decreased; however, contrary to the findings of Tasch et al, 105 the NAA/Cr ratio was greatly increased in the aforementioned patient. 87 Other small studies have reported similar findings.…”

Section: Magnetic Resonance Spectroscopycontrasting

confidence: 85%

“…Nine published reports with a total of 44 patients have described the MRS findings evaluating the NAA, Cr, Cho, and mI content within the hamartoma. The group in Montreal was the first to study the extent of neuronal injury in the temporal lobes and hamartomas of 5 patients with HHs and gelastic seizures by using proton MRS. 105 The NAA/Cr ratio was significantly decreased in the patients with hamartomas compared with the hypothalami of healthy individuals. Likewise, Pascual-Castroviejo et al 87 used MRS to study the temporal lobes and the HH in 1 patient.…”

Section: Magnetic Resonance Spectroscopymentioning

confidence: 90%

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Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Mittal

Montes

et al. 2013

FOC

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Hypothalamic hamartomas are uncommon but well-recognized developmental malformations that are classically associated with gelastic seizures and other refractory seizure types. The clinical course is often progressive and, in addition to the catastrophic epileptic syndrome, patients commonly exhibit debilitating cognitive, behavioral, and psychiatric disturbances. Over the past decade, investigators have gained considerable knowledge into the pathobiological and neurophysiological properties of these rare lesions. In this review, the authors examine the causes and molecular biology of hypothalamic hamartomas as well as the principal clinical features, neuroimaging findings, and electrophysiological characteristics. The diverse surgical modalities and strategies used to manage these difficult lesions are outlined in the second article of this 2-part review.

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Section: Magnetic Resonance Spectroscopycontrasting

confidence: 85%

Section: Magnetic Resonance Spectroscopymentioning

confidence: 90%

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Mittal

Montes

et al. 2013

FOC

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“…It was not until Cascino et al [13]published their unsuccessful results with subdural recording-guided corticectomies in 7 patients with HH and GS that neurologists and epilepsy surgeons understood that subdural telemetry data are misleading in HH. A similar experience was later described by Kuzniecky et al [12], who, with other investigators, presented new data provided by more sophisticated methodology, including ictal SPECT, monitoring with depth electrodes [12, 15]and MRS study [24], all of which pointing to the HH as the site of the epileptic focus.…”

Section: Discussionmentioning

confidence: 76%

Surgical Treatment of Hypothalamic Hamartoma and Refractory Seizures

Kramer

Spector²,

Nasser³

et al. 2001

Pediatr Neurosurg

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Refractory gelastic seizures are often associated with hypothalamic hamartoma (HH). Presurgical evaluation in such children often points to a distinct cortical region as the source of the seizures. A case of a child with HH and refractory seizures is presented. Video-EEG monitoring revealed a well-defined epileptic focus in the left frontal region. In accordance with the current understanding of the nature of hamartoma-related seizures, the hamartoma was resected. Follow-up evaluations revealed a marked improvement in seizure frequency and global functioning.

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“…8,14,15,20,23) In all cases the goal of the treatment was the removal of the entire lesion since it is commonly agreed that the hamartoma is the trigger of the epilepsy 3,5,7,8,11,13,21) and so the resection of hypothalamic hamartoma must be as complete as possible to achieve good seizure remission. 16) The present study suggests that complete removal of the hamartoma can be replaced by disconnection, using either open surgery or endoscopy, since the hamartoma is a stable lesion and entire anatomical removal is not necessary to treat the epilepsy.…”

Section: Discussion I Surgical Approaches and Techniquesmentioning

confidence: 99%

Disconnecting Surgical Treatment of Hypothalamic Hamartoma in Children and Adults With Refractory Epilepsy and Proposal of a New Classification.

Delalande

Fohlen

2003

Neurol. Med. Chir.(Tokyo)

255

175

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Hypothalamic hamartoma associated with intractable epilepsy is a major problem when surgical management is discussed. Professor Olivier Delalande is Chief Neurosurgeon of the Department of Pediatric Neurosurgery, Fondation Ophtalmologique Adolf de Rothschild, in Paris, France. In recent international symposia, he has reported a series of hypothalamic hamartoma in children and adults with refractory epilepsy, managed with various surgical techniques according to his new classification of hypothalamic hamartomas. With the permission of Professor Akira Yamaura, Chief Editor of the Neurologia medico-chirurgica, this stimulating paper has appeared in this issue. The strategies of his surgery using surgical excision, surgical disconnection or endoscopic disconnection are challenging but safe. His surgical outcomes are that 8 patients are seizure free, one patient has only brief gelastic seizures and 8 patients are dramatically improved. I hope that the authors will have the opportunity to again examine the outcomes in their series several years from now.Tatsuya TANAKA, M.D., D.M.Sc., President of the 30th Annual Meeting of the Japan Society for Pediatric Neurosurgery. AbstractA series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had partial seizures and three had generalized tonic-clonic seizures. The mean seizure frequency was 21 per day. Four patients had borderline intelligence quotient and the others were mentally retarded. Five patients presented with precocious puberty, one with acromegaly, and four suffered from obesity. Brain magnetic resonance imaging, performed at least twice in each patient, showed the hamartoma as a stable homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle with variable extension to the bottom. Ictal single photon emission computed tomography, performed in four patients, showed hyperperfusion within the hamartoma in two patients. Twenty-five operations were performed in the 17 patients. The first patient underwent total removal of the hamartoma, whereas the following 16 patients underwent disconnection through open surgery (14 procedures) and/or endoscopy (9 procedures). Eight patients became seizure-free, one patient had only brief gelastic seizures, and eight patients were dramatically improved with a mean follow up of 18.6 months (8 days to 43 months). Surgery was safe in all but two patients: the first patient had transient hemiplegia and the third cranial nerve paresis, and the other developed hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, and behavior and school performance were greatly improved in most patients. Our series illustrates the feasibility and relative safety of disconnection surgery for hypothalamic hamartomas with seizure relief in 5...

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Hypothalamic hamartomas and gelastic epilepsy

Abstract: We found no detectable neuronal damage in the temporal lobes of patients with hypothalamic hamartomas and gelastic epilepsy. This is further evidence that gelastic seizures do not originate in the temporal lobes of these patients.

Cited by 44 publications

References 8 publications

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Hypothalamic hamartomas. Part 1. Clinical, neuroimaging, and neurophysiological characteristics

Surgical Treatment of Hypothalamic Hamartoma and Refractory Seizures

Disconnecting Surgical Treatment of Hypothalamic Hamartoma in Children and Adults With Refractory Epilepsy and Proposal of a New Classification.

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