Managing epilepsy in tuberous sclerosis complex

EA Thiele - Journal of child neurology, 2004 - journals.sagepub.com
EA Thiele
Journal of child neurology, 2004journals.sagepub.com
Epilepsy is very common in tuberous sclerosis complex and occurs in 80 to 90% of affected
individuals during their lifetime. Onset usually occurs during childhood, and up to one third
of children with tuberous sclerosis complex will develop infantile spasms. Although not
completely understood, the incidence of epilepsy is thought to relate to the neuropathologic
features of the disorder, including cortical tubers and other dysgenetic features. Individuals
with tuberous sclerosis complex frequently have epileptiform features to their …
Epilepsy is very common in tuberous sclerosis complex and occurs in 80 to 90% of affected individuals during their lifetime. Onset usually occurs during childhood, and up to one third of children with tuberous sclerosis complex will develop infantile spasms. Although not completely understood, the incidence of epilepsy is thought to relate to the neuropathologic features of the disorder, including cortical tubers and other dysgenetic features. Individuals with tuberous sclerosis complex frequently have epileptiform features to their electroencephalograms. Treatment of epilepsy in tuberous sclerosis complex is similar to epilepsy resulting from other causes and includes anticonvulsant medications, the vagus nerve stimulator, and the ketogenic diet. Vigabatrin has been shown to be particularly effective in treating infantile spasms in the setting of tuberous sclerosis complex. Epilepsy surgery has a very important role in the management of children and adults with pharmacoresistant epilepsy in tuberous sclerosis complex. (J Child Neurol 2004;19:680—686).
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