Amyloidosis

Abstract

Schleiden coined the word “amyloid” in 1838 to describe a normal amylaceous constituent of plants. Virchow used the term in 1854 to describe the “lardaceous” changes in the liver at autopsy in a patient with amyloidosis, while primary amyloidosis was probably first described in 1856 by Wilks. Neurologic involvement in systemic amyloidosis, however, was not mentioned in the literature until the early part of this century. In Germany, Konigstein in 1925 observed atrophy and weakness of hand muscles, likely carpal tunnel syndrome, associated with amyloid deposits in the epineurium, vasa nervorum, and alimentary canal at autopsy in a 60-year-old man. Lubarsch in 1929 and Larsen in 1930 described cases of polyneuropathy with amyloid infiltration of nerves and ganglia. DeNavasquez and Treble in 1938 presented a particularly well-studied patient with progressive weakness, arreflexia, and prominent autonomic insufficiency but normal sensation, who at postmortem examination had extensive infiltration of the dorsal root and sympathetic ganglia and peripheral nerves with epineurial and endoneurial amyloid deposits. In these early case reports, the prominence of polyneuropathy in patients with amyloidosis was established.