Epilepsy secondary to tuberous sclerosis: lessons learned and current challenges.
Childs Nerv Syst
; 26(11): 1495-504, 2010 Nov.
Article
en En
| MEDLINE
| ID: mdl-20358377
ABSTRACT
BACKGROUND:
In tuberous sclerosis complex (TSC), a substantially increased risk of developing epilepsy is present as a result of a disruption of a TSC gene expression in the brain and secondary abnormal cellular differentiation, migration, and proliferation. Dysregulated excitation probably has its roots in the disruption of GABAergic interneuron development. There is an age-dependent electroclinical expression of seizures, and epilepsy is often quite severe and unremitting.DISCUSSION:
The majority of patients (>60%) who are candidates for surgery remain seizure-free after tuberectomy. During the recent years technical advances in the localization of the epileptogenic zone during the recent years have lead to a 63% of Engel class I status after surgery compared with a previous 52%. In medically refractory patients not suitable for surgery, vagus nerve stimulation has proved efficacy in significantly reducing seizure frequency in more than 50% of cases. New evidence suggests that mTOR inhibitors may be helpful in the management of intractable epilepsy for individuals with TSC.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Esclerosis Tuberosa
/
Epilepsia
Tipo de estudio:
Diagnostic_studies
/
Prognostic_studies
Límite:
Animals
/
Child
/
Child, preschool
/
Humans
/
Infant
Idioma:
En
Revista:
Childs Nerv Syst
Asunto de la revista:
NEUROLOGIA
/
PEDIATRIA
Año:
2010
Tipo del documento:
Article
País de afiliación:
Italia