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Paraplegia-intellectual disability-hyperkeratosis syndrome

ORPHA:2824· ICD-10 G82.1

Definition

A rare X-linked syndromic intellectual disability characterized by intellectual impairment of variable severity, progressive lower limb spasticity, and diffuse palmoplantar hyperkeratosis. Additional manifestations include pes cavus, extensor plantar responses, hand tremor, and mild dysmorphic facial features.

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