Affiliations: Texas College of Osteopathic Medicine, University of
North Texas Health Science Center, Fort Worth, TX, USA | Comprehensive Epilepsy Program, Cook Children's
Medical Center, Fort Worth, TX, USA | Department of Pathology, Cook Children's Center, Fort
Worth, TX, USA
Note: [] Correspondence: M. Scott Perry, M.D., Neuroscience 901 7th
Avenue, Suite 120, Fort Worth, TX 76104, USA. Tel.: +1 682 885 2500; Fax: +1
682 885 2510; E-mail: Scott.perry@cookchildrens.org
Abstract: Severe myoclonic epilepsy of infancy or Dravet syndrome is a
catastrophic epilepsy of genetic origin that begins with febrile seizures in
the first year of life and evolves to intractable epilepsy with cognitive
decline and frequent episodes of status epilepticus. While multiple treatment
strategies are employed, rarely are patients with Dravet syndrome considered
for resective epilepsy surgery. We report a case of Dravet syndrome in which
palliative resection of epileptogenic foci revealed cortical dysplasia of
Taylor-type. We discuss the association of cortical dysplasia with Dravet
syndrome and consider the utility of palliative surgical therapy in a
genetically based epilepsy syndrome.
Keywords: Dravet syndrome, severe myoclonic epilepsy of infancy, epilepsy surgery, cortical dysplasia
