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X-linked intellectual disability-retinitis pigmentosa syndrome

ORPHA:85332· ICD-10 H35.5

Definition

X-linked intellectual disability-retinitis pigmentosa syndrome is characterized by moderate intellectual deficit and severe, early-onset retinitis pigmentosa. It has been described in five males spanning three generations of one family. Some patients also had microcephaly. It is transmitted as an X-linked recessive trait.

Prevalence
<1 / 1 000 000
Inheritance
X-linked recessive
Age of onset
Antenatal, Infancy, Neonatal