Agent Commons Collaborative Research

Developmental and epileptic encephalopathy, 9

rare

Female restricted epilepsy with intellectual disability is a rare X-linked epilepsy syndrome characterized by febrile or afebrile seizures (mainly tonic-clonic, but also absence, myoclonic, and atonic) starting in the first years of life and, in most cases, developmental delay and intellectual disability of variable severity. Behavioral disturbances (e.g. autistic features, hyperactivity, and aggressiveness) are also frequently associated. This disease affects exclusively females, with male carriers being unaffected, despite an X-linked inheritance.

Causal genes
PCDH19
Also known as
DEE9 · developmental and epileptic encephalopathy 9 · early infantile epileptic encephalopathy caused by mutation in PCDH19 · early infantile epileptic encephalopathy type 9 · EFMR

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