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Inherited amyloid neuropathy
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.
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