Amyloid-like IgM deposition neuropathy: a distinct clinico-pathologic and proteomic profiled disorder. J Peripher Nerv Syst 2012 Jun;17(2):182-90
Date
06/28/2012Pubmed ID
22734903Pubmed Central ID
PMC3895329DOI
10.1111/j.1529-8027.2012.00406.xScopus ID
2-s2.0-84863496144 (requires institutional sign-in at Scopus site) 19 CitationsAbstract
Some patients with immunoglobulin paraproteinemic neuropathy have intra-nerve deposits that morphologically mimick amyloid, but do no stain with Congo red. Patients with amyloid-like deposits were identified. The nerve amyloid-like aggregates were studied by laser microdissection and dual mass spectrometry. Three male patients, all with IgM gammopathy, and neuropathy were identified. Follow-up, disease duration was 5, 19, and 7 years, respectively. All had progressive asymmetric sensory-onset distal axonal polyneuropathy with late motor involvement. Autonomic symptoms occurred in only one after 13 years of symptoms. None had clinical cardio-renal involvement. One had skin papules with dermal amyloid-like deposits. Endoneurial amyloid-like deposits had granulo-fibrillar ultrastructure. Mass spectrometry of laser-dissected deposits identified IgM pentameric macroglobulin (heavy, light, and joining chains) without amyloid-associated proteins including absent apolipoprotein E and serum amyloid P-component. Amyloid-like neuropathy has distinct clinical, pathologic, and proteomic features which expand the spectrum of IgM neuropathies. Patients have favorable survival, relative absence of autonomic features, and distinct proteomic profiles of the infiltrative protein in nerve.
Author List
Figueroa JJ, Bosch EP, Dyck PJ, Singer W, Vrana JA, Theis JD, Dogan A, Klein CJAuthor
Juan Jose Figueroa MD Assistant Professor in the Neurology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AgedAmyloid
Amyloid Neuropathies
Humans
Immunoglobulin M
Male
Mass Spectrometry
Middle Aged
Paraproteinemias
Polyneuropathies
Proteomics
