Primary Systemic Amyloidosis with Involvement of the Nervous System: Report of a Case

Abstract

A case of primary systemic amyloidosis with involvement of the nervous system is reported in a 62-yr. -old white male. The patient presented bizarre neurologic mani-festations. Spinal fluid examination revealed no abnormalities. The patient died of congestive heart failure. Necropsy studies revealed diffuse amyloid involvement of the heart, lungs, the entire gastrointestinal tract in the muscularis mucosae and both muscular layers, the muscularis of the urinary bladder, the tunica albuginea of the testes, and the voluntary muscles. Amyloid deposits were found in the anterior and posterior roots of the spinal cord, the filum terminale, sympathetic ganglia, peripheral nerves and the spinal dura. The diffuse involvement of the nervous system is unusual, this being the 6th case recorded in the literature.