Conditions / Nervous system

cortical dysplasia-focal epilepsy syndrome

info · Nervous system · ICD-10: Q04.8

A brain disease that is characterized by cortical dysplasia, focal epilepsy, macrocephaly, and diminished deep-tendon reflexes that has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the contactin associate

A brain disease that is characterized by cortical dysplasia, focal epilepsy, macrocephaly, and diminished deep-tendon reflexes that has_material_basis_in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the contactin associated protein like 2 (CNTNAP2) gene on chromosome 7q35-q36.

Signs and symptoms

  • Bilateral tonic-clonic seizure
  • Strabismus
  • Focal impaired awareness seizure
  • Intellectual disability
  • Focal-onset seizure
  • Receptive language delay
  • Macrocephaly
  • Schizophrenia
  • Obesity
  • Delayed speech and language development

Also known as: CDFE syndrome; CDFES; PTHSL1; Pitt-Hopkins-like syndrome-1