Familial Amyloidotic Polyneuropathy

Amyloidosis has received considerable attention recently because of its association with Alzheimer's disease. Actually, the amyloid in the cortical plaques, which is characteristic of Alzheimer's disease, is a localized form of amyloid deposition. Although intracranial vascular amyloid deposits which contain the A4 or beta-protein are usually associated with Alzheimer's disease, deposition of this amyloid fibril substance in other organs of the body has not been described. Much less attention has been paid to amyloid involvement of the PNS which is a fascinating subject in itself and is the subject of this review.