Amyloid-like IgM deposition neuropathy with multiple mononeuropathies and generalized neuropathy.
Neuromuscul Disord
; 33(5): 391-395, 2023 05.
Article
em En
| MEDLINE
| ID: mdl-37028153
ABSTRACT
Amyloid-like IgM deposition neuropathy is a distinct entity in the setting of IgM monoclonal gammopathy in which endoneurial perivascular entire IgM-particle accumulation leads to a painful sensory followed by motor peripheral neuropathy. We report a 77-year-old man presenting with progressive multiple mononeuropathies starting with painless right foot drop. Electrodiagnostic studies showed severe axonal sensory-motor neuropathy superimposed by multiple mononeuropathies. Laboratory investigations were remarkable for biclonal gammopathy of IgM kappa, IgA lambda and severe sudomotor and mild cardiovagal autonomic dysfunction. A right sural nerve biopsy showed multifocal axonal neuropathy, prominent microvasculitis, and prominent large endoneurial deposits of Congo-red negative amorphous material. Laser dissected mass spectrometry-based proteomics identified IgM kappa deposit without serum amyloid-P protein. This case has several distinctive features, including motor preceding sensory involvement, prominent IgM-kappa proteinaceous deposits replacing most of the endoneurium, a prominent inflammatory component, and improvement of motor strength after immunotherapy.
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Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Paraproteinemias
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Doenças do Sistema Nervoso Periférico
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Mononeuropatias
Tipo de estudo:
Diagnostic_studies
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Prognostic_studies
Limite:
Aged
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Humans
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Male
Idioma:
En
Ano de publicação:
2023
Tipo de documento:
Article