vitalwiki

Familial dysautonomia

ORPHA:1764· ICD-10 G90.1

Definition

A rare hereditary sensory and autonomic neuropathy characterized by decreased pain and temperature perception, absent deep tendon reflexes, proprioceptive ataxia, afferent baroreflex failure and progressive optic neuropathy.

Prevalence
1-5 / 10 000
Inheritance
Autosomal recessive
Age of onset
Childhood, Infancy, Neonatal