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Axonal Neuropathy Associated With Cold Agglutinins: A Vasculitic‐Ischaemic Neuropathy
Author(s) -
Bezzi G,
Previtali S,
Nemni R,
Epis R,
Compagi V,
Canal N,
Quattrini A.
Publication year - 2001
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2001.01007-4.x
Subject(s) - medicine , peripheral neuropathy , hemoglobinuria , pathology , peripheral nervous system , autoimmune hemolytic anemia , polyneuropathy , myelin , immunology , antibody , central nervous system , hemolysis , endocrinology , diabetes mellitus
Cold agglutinin (CA) disease is an autoimmune hemolytic process characterized by chronic anemia, hemoglobinuria, cold induced rash, and acrocyanosis of exposed body parts. Although few cases of peripheral neuropathies have been observed in patients with CA, the mechanism of peripheral nervous system involvement is still uncertain. However, similar to other neuropathies due to IgM paraproteinaemia, such as anti‐myelin associated glycoprotein, or anti‐acidic glycolipid sulphate‐3‐glucuronyl paragloboside, the direct effect of the antibody on nerve constituents is considered the pathogenetic mechanism causing the demyelinating neuropathy. We report a patient with CA and sensorimotor peripheral neuropathy with electrophysiological and histological findings of a severe acute axonal neuropathy. Pathological findings show vascular damage in the nerve, suggesting a major role for ischaemic/vasculitic pathogenetic mechanism of the peripheral neuropathy in CA.