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Early infantile developmental and epileptic encephalopathy

ORPHA:1934· ICD-10 G40.3

Definition

A severe form of age-related epileptic encephalopathies characterized by the onset of tonic spasms within the first 3 months of life that can be generalized or lateralized, independent of the sleep cycle, and that can occur hundreds of times per day, leading to psychomotor impairment and death.

Prevalence
1-9 / 100 000
Inheritance
Autosomal dominant, Autosomal recessive, Not applicable, X-linked recessive
Age of onset
Neonatal