Current management for epilepsy in tuberous sclerosis complex.
OBJECTIVE
This article reviews the most significant advances in the field of epilepsy associated with tuberous sclerosis complex, with emphasis on new advances in the knowledge of the pathophysiological mechanisms of epileptogenicity, progress in identifying the epileptogenic zone, and the rationale for surgical management in individuals with intractable seizures.


RESULTS
Advances in our understanding of the mechanisms and genetics underlying infantile spasms and catastrophic epilepsy associated with tuberous sclerosis complex may facilitate more effective interventions. Early effective seizure control could significantly reduce the adverse developmental effects of chronic epilepsy in tuberous sclerosis. Vigabatrin is the first choice in the short-term treatment of infantile spasms. Some individuals, however, develop seizures that remain highly intractable. The factors that influence the intractability of epilepsy associated with tuberous sclerosis complex remain poorly understood. Multimodality neuroimaging has improved detection of epileptogenic foci, allowing an increased number of individuals to be evaluated for resective surgery. Epilepsy surgery is often associated with significant improvement of the neurologic outcome.


CONCLUSIONS
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.
Pediatric Neurology Unit, Department of Neurosciences, Tor Vergata University, Via Montpellier no. 1, 00133 Rome, Italy. curatolo@uniroma2.it
Paolo
P
Curatolo
Roberta
R
Bombardieri
Caterina
C
Cerminara
D000818:Animals; D000927:Anticonvulsants / Q000627:therapeutic use; D001930:Brain Injuries / Q000150:complications; D003072:Cognition Disorders / Q000209:etiology; D004827:Epilepsy / Q000209:etiology / Q000628:therapy*; D006801:Humans; D009493:Neurosurgery / Q000379:methods / Q000639:trends; D014402:Tuberous Sclerosis / Q000150:complications*; D020888:Vigabatrin / Q000627:therapeutic use
D000927:Anticonvulsants; D020888:Vigabatrin