Early control of seizures improves long-term outcome in children with tuberous sclerosis complex.
Epilepsy associated with tuberous sclerosis complex (TSC) is characterized by early onset and intractable seizures in the majority of children. There is a solid evidence of clinical efficacy of vigabatrin in interrupting infantile spasms associated with TSC. Due to an early diagnosis we were able to start vigabatrin at the very early onset of seizures in 10 children, who subsequently underwent a long-term neurodevelopmental follow-up. At the final evaluation, a seizure free status was achieved in 50% of patients; 30% of individuals had a normal or borderline mental development, with no patients developing severe mental retardation and/or autism. Early control of seizures has a crucial role in preventing subsequent epileptic encephalopathy, and in reducing the cognitive/behavioural consequences of seizures, but does not guarantee for a normal mental outcome in children with TSC.
Department of Neurosciences, Pediatric Neurology Unit, Tor Vergata University, via Montpellier 1, Rome 00133, Italy.
Roberta
R
Bombardieri
Mariangela
M
Pinci
Romina
R
Moavero
Caterina
C
Cerminara
Paolo
P
Curatolo
D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis / Q000453:epidemiology; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D015995:Prevalence; D012640:Seizures / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology*; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000453:epidemiology*; D013997:Time Factors; D014402:Tuberous Sclerosis / Q000453:epidemiology*; D020888:Vigabatrin / Q000627:therapeutic use*
D000927:Anticonvulsants; D020888:Vigabatrin