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Congenital Insensitivity/Indifference to Pain
Hsan type i
Sensory and Autonomic Neuropathies - HSAN Type I
HSAN Type I has late onset in late childhood or adolescence. The nerve fibres subserving pain sensation and their connections to the spinal cord degenerate. Impaired pain sensation starts distally (the furthest point) in the legs and ultimately involves the arms. Due to the unawareness of pain, damage to skin, nails, joints etc occurs without the affected person being aware until they see the bruise, or the spreading skin infection, abscess, or see the deformity at a site of fracture. Thus pain provides a valuable protection.
Inheritance patterns
Autosomal Dominant.
Prenatal diagnosis
This is possible. The change in a gene, SPTLC1 on chromosome 9 is responsible for HSAN Type I.
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