Inherited amyloid neuropathy is a condition in which there is extracellular deposition of the fibrillary protein, amyloid, in peripheral nerve and sensory and autonomic ganglia, as well as around blood vessels in nerves and other tissues.
The disease usually appears in early adulthood.
Manifestations
Affects (1) small neural fibers responsible for pain & temperature leading to loss of the ability to perceive mechanical and thermal injury and tissue damage. As a result, painless injuries present a major hazard of this disorder; in advanced stages they can lead to chronic infections or osteomyelitis of the feet or hands, and may require amputation. (2) autonomic functions manifested as a gastrointestinal motility disorder or orthostatic hypotension.
The condition is progressive and death usually occurs before 50yrs.
Treatment
Mainly supportive therapy.
