Managing epilepsy in tuberous sclerosis complex.
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.
Thiele
Elizabeth Anne
EA
Department of Neurology, Harvard Medical School, Boston, MA, USA. ethiele@partners.org
D000927:Anticonvulsants / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D004035:Diet Therapy; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D007231:Infant, Newborn; D007659:Ketones; D013036:Spasms, Infantile / Q000188:drug therapy / Q000209:etiology; D004561:Transcutaneous Electric Nerve Stimulation; D014402:Tuberous Sclerosis / Q000150:complications*; D020888:Vigabatrin / Q000627:therapeutic use
D000927:Anticonvulsants; D007659:Ketones; D020888:Vigabatrin