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Seizures-intellectual disability due to hydroxylysinuria syndrome

ORPHA:79156· ICD-10 E72.3

Definition

A rare inborn error of metabolism characterized by infantile onset of global developmental delay, severe intellectual disability, seizures, and movement disorder (including tremor, hyperkinesia, and myoclonus), associated with excessive excretion of hydroxylysine in urine. There have been no further descriptions in the literature since 1970.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy