Congenital insensitivity to pain with anhidrosis-cipa : a case report

Abstract EN

Congenital insensitivity to pain with anhidrosis is also known as hereditary sensory autonomic neuropathy type IV.

It is an extremely rare autosomal recessive disorder due to lack of maturation of small myelinated and unmyelinated fibers of peripheral nerves, which convey sensation of pain and temperature.

It is characterized by recurrent episodes of hyperpyrexia, anhidrosis (inability to sweat) due to lack of innervation of normal sweat glands, absent reaction to noxious stimuli, self-mutilating behavior and mental retardation.

The clinical presentation of a child with this rare disease is worth to describe, as the first case to be reported in Jordan.

Special training programs to prevent self-mutilation and accidental injuries are necessary but are hampered by the low IQ.