Topiramate therapy of epilepsy associated with Angelman's syndrome.
Angelman's syndrome, a genetic disorder involving a defect in the DNA coding for subunits of the gamma-aminobutyric acid (GABA) type A receptor, often is associated with intractable epilepsy. Topiramate is a novel anticonvulsant that enhances GABAergic neurotransmission. Five children with Angelman's syndrome and epilepsy were treated with topiramate for clinical indications. The drug was effective and well tolerated, possibly because of its GABAergic properties. Further studies are necessary to confirm and elucidate this observation.
Franz
D N
DN
Department of Pediatrics, University of Cincinnati College of Medicine, Children's Hospital Medical Center, 45229-3039, USA. frand0@chmcc.org
Glauser
T A
TA
Tudor
C
C
Williams
S
S
D017204:Angelman Syndrome / Q000150:complications*; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004827:Epilepsy / Q000188:drug therapy* / Q000209:etiology*; D005260:Female; D005632:Fructose / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006801:Humans; D008297:Male; D000077236:Topiramate
D000927:Anticonvulsants; D000077236:Topiramate; D005632:Fructose