TY - JOUR
T1 - Amyloid-like IgM deposition neuropathy
T2 - A distinct clinico-pathologic and proteomic profiled disorder
AU - Figueroa, Juan J.
AU - Bosch, E. Peter
AU - Dyck, P. James B.
AU - Singer, Wolfgang
AU - Vrana, Julie A.
AU - Theis, Jason D.
AU - Dogan, Ahmet
AU - Klein, Christopher J.
PY - 2012/6
Y1 - 2012/6
N2 - Some patients with immunoglobulin paraproteinemic neuropathy have intranerve 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. Amyloidlike 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.
AB - Some patients with immunoglobulin paraproteinemic neuropathy have intranerve 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. Amyloidlike 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.
KW - Amyloid
KW - IgM
KW - Mass spectrometry
KW - Neuropathy
UR - http://www.scopus.com/inward/record.url?scp=84863496144&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84863496144&partnerID=8YFLogxK
U2 - 10.1111/j.1529-8027.2012.00406.x
DO - 10.1111/j.1529-8027.2012.00406.x
M3 - Article
C2 - 22734903
AN - SCOPUS:84863496144
SN - 1085-9489
VL - 17
SP - 182
EP - 190
JO - Journal of the Peripheral Nervous System
JF - Journal of the Peripheral Nervous System
IS - 2
ER -