title: Canomad: report of a case with a 40-year history and autopsy. Is this a sensory ganglionopathy with neuromuscular junction blockade?
abstract: BACKGROUND
  An 80-year-old man had a 40-year history of chronic sensory ataxic neuropathy and 11 years of relapsing/remitting episodes of rapid deterioration with perioral paresthesiae and weakness of bulbar, respiratory, and limb muscles.
  
  
  METHODS
  An immunoglobulin M (IgM) paraprotein was detected 12 years before death, and Waldenstrom macroglobulinemia was diagnosed on bone marrow biopsy 3 years before death. Chronic ataxic neuropathy with ophthalmoplegia, IgM paraprotein, cold agglutinins, and anti-disialyl antibodies (CANOMAD) was diagnosed.
  
  
  RESULTS
  Comprehensive autopsy showed severe dorsal column atrophy and dorsal root ganglionopathy. A different pathology was identified in cranial and peripheral nerves, dorsal roots, and cauda equina, comprising infiltration of clonal B-lymphocytes within the endoneurium, perineurium, and leptomeninges.
  
  
  CONCLUSIONS
  The autopsy provides evidence of the pathogenesis of the relapsing remitting component of CANOMAD, and we postulate that this may relate to the presence of clonal IgM anti-disiayl gangliosides secreting B-lymphocytes within nerves.
authors: Department of Anatomical Pathology, St. Vincent's Hospital Melbourne, PO Box 2900, Fitzroy, Victoria 3065, Australia.
authors: Penelope A
authors: PA
authors: McKelvie
authors: Peter C
authors: PC
authors: Gates
authors: Timothy
authors: T
authors: Day
mesh_terms: D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology / Q000473:pathology*; D001259:Ataxia / Q000276:immunology / Q000473:pathology*; D001340:Autonomic Nerve Block; D002908:Chronic Disease; D017809:Fatal Outcome; D017950:Ganglia, Sensory / Q000473:pathology*; D006801:Humans; D008297:Male; D009469:Neuromuscular Junction / Q000473:pathology*; D009886:Ophthalmoplegia / Q000276:immunology / Q000473:pathology*; D013997:Time Factors
keywords: CANOMAD; IgM paraprotein; Waldenstrom macroglobulinemia; clonal B-lymphocytes; dorsal root ganglionopathy; relapsing remitting cranial polyneuropathy