Epilepsy surgery for tuberous sclerosis

Romanelli, Pantaleo; Verdecchia, Magda; Rodas, R A; Seri, Stefano; Curatolo, Paolo

doi:10.1016/j.pediatrneurol.2004.05.012

Cited by 61 publications

(35 citation statements)

References 43 publications

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“…Drug-resistance or intractability is common in some cases of childhood EE such as LGS, WS, Sturge-Weber syndrome, and Ohtahara syndrome [1][2][3], and is anticipated to exist at a certain level before the extensive drug trials. Therefore, the description of "intractability" that failure to at least two AEDs over a 2-year period [14] becomes meaningless for childhood-onset EE when surgery is applied. In this study, early surgery was performed for a portion of patients with WS.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Lee

Kang

et al. 2014

Brain and Development

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Section: Discussionmentioning

confidence: 99%

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Lee

Kang

et al. 2014

Brain and Development

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“…Seizures occur in the first years of life in approximately 80% of patients with TSC (Curatolo & Verdecchia, 2003;Curatolo et al, 2005). Involvement of cortical tubers and/or perituberal zones in epileptogenesis is demonstrated by the excellent outcome following surgical removal of cortical tubers, with a high percentage of patients rendered seizure-free (Romanelli et al, 2004;Weiner et al, 2006).…”

Section: Introductionmentioning

confidence: 99%

Tuberous sclerosis: A primary pathology of astrocytes?

Sosunov

Weiner

et al. 2008

Epilepsia

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SUMMARYPurpose: Cortical tubers are epileptogenic lesions in patients with tuberous sclerosis complex (TSC). Giant cells and dysplastic neurons are pathological hallmarks of cortical tubers. Severe astrogliosis, which is invariably present in tubers, has attracted much less attention. We hypothesize that the development of astrogliosis in cortical tubers constitutes a primary pathology of astrocytes and is directly related to TSC 1/2 mutations. Methods: To begin to test this hypothesis, we performed immunohistochemical and electron microscopic analysis of brain tuber tissue resected from epileptic TSC patients. We compared alterations in tuber astrocytes to those found in other acute and chronic human epilepsy pathologies. Results: We found that astrogliosis in tubers is comprised of a mixture of "gliotic" and "reactive" astrocytes. The majority of tuber astrocytes are "gliotic" astrocytes that are morphologically and immunophenotypically similar to astrocytes in areas of gliosis in hippocampal sclerosis (HS). However, specific immunostaining features differentiate TSC gliosis from HS gliosis. "Reactive" tuber astrocytes are large-sized, vimentin positive cells in the vicinity of giant cells that show activation of the mammalian target of rapamycin (mTOR) pathway, consistent with mutated TSC gene function. These cells resemble acutely reactive human astrocytes seen in tissue resected from depth electrode implantation patients. Oligodendrocytes and NG2 expressing glial cells do not have any detectable alterations within tubers. Conclusion: We conclude that astrocytes are the type of glial cell selectively impacted in cortical tuber pathology. We propose that tubers may be dynamic lesions, with progression of astrocytes over time from "reactive" to "gliotic." Tuber astrogliosis in TSC may represent a genetic "model" of gliosis that is phenotypically similar to gliosis seen in acquired human pathologies.

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“…Improvement in quality of life of these patients, related to either a reduction in seizure frequency and or reduction in medications, is experienced by 95% of patients [28 ]. This is a remarkable result, given the severity of the epileptic syndromes associated with TSC and the high epileptogenicity of the abnormal cortex.…”

Section: Epilepsy Surgerymentioning

confidence: 96%

“…For patients with intractable seizures arising from well mapped epileptogenic zones that may be located in or adjacent to the eloquent cortex, stereotactic radiosurgery could have the potential to represent a suitable alternative to resective surgery [28 ].…”

Section: Epilepsy Surgerymentioning

confidence: 99%

Current management for epilepsy in tuberous sclerosis complex

Curatolo

Bombardieri

Cerminara

2006

Current Opinion in Neurology

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Epilepsy in tuberous sclerosis seems to arise from the interaction between multiple areas, all of which have increased excitability and reduced inhibition. Understanding the mechanisms of epileptogenesis might increase the availability of development of a more specific and efficacious treatment. New evidence suggests that it is possible to noninvasively identify children with tuberous sclerosis who are highly likely to become seizure free following surgical treatment.

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Epilepsy surgery for tuberous sclerosis

Cited by 61 publications

References 43 publications

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy

Tuberous sclerosis: A primary pathology of astrocytes?

Current management for epilepsy in tuberous sclerosis complex

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