IRF2BPL

Clinical Characteristics

Individuals with NEDAMSS and confirmed IRF2BPL variants have neurodevelopmental regression that includes losing fine and gross motor skills as well as speech after an initial period of normal development. The onset of the regression has been reported to be between 2 and 10 years of age.  Choreoathetosis, ataxia, dystonia, and spasticity have also been observed.