8 Citations (Scopus)

Abstract

The identification of a distinct subgroup of patients within the spectrum of lower motor neuron syndromes (LMNS) is crucial as some are potentially treatable. We describe the clinical and neuropathological characteristics of a patient presenting with a rapidly progressive LMNS associated with high titers of anti-GM1 antibodies, leading to respiratory failure within 10 months. Histopathological study of a biopsy of a obturator nerve motor branch demonstrated a predominantly axonal motor neuropathy, while electron microscopy analysis localized macrophages located within the periaxonal space. Immunohistochemistry demonstrated deposits of complement activation products (C3i) and immunoglobulins (IgM) on nerve fibers. The patient's clinical, immunological and pathological findings are consistent with a diagnosis of a chronic motor axonal neuropathy (CMAN), likely of immune-mediated origin.

Original languageEnglish
Pages (from-to)341-346
Number of pages6
JournalJournal of the Peripheral Nervous System
Volume16
Issue number4
DOIs
Publication statusPublished - Dec 2011

Fingerprint

Motor Neurons
Obturator Nerve
Complement Activation
Nerve Fibers
Respiratory Insufficiency
Immunoglobulin M
Immunoglobulins
Anti-Idiotypic Antibodies
Electron Microscopy
Immunohistochemistry
Macrophages
Biopsy

Keywords

  • acute motor axonal neuropathy
  • amyotrophic lateral sclerosis
  • anti-GM1 antibodies
  • lower motor neuron disease
  • nerve biopsy
  • neuropathology
  • peripheral neuropathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

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title = "Chronic motor axonal neuropathy",
abstract = "The identification of a distinct subgroup of patients within the spectrum of lower motor neuron syndromes (LMNS) is crucial as some are potentially treatable. We describe the clinical and neuropathological characteristics of a patient presenting with a rapidly progressive LMNS associated with high titers of anti-GM1 antibodies, leading to respiratory failure within 10 months. Histopathological study of a biopsy of a obturator nerve motor branch demonstrated a predominantly axonal motor neuropathy, while electron microscopy analysis localized macrophages located within the periaxonal space. Immunohistochemistry demonstrated deposits of complement activation products (C3i) and immunoglobulins (IgM) on nerve fibers. The patient's clinical, immunological and pathological findings are consistent with a diagnosis of a chronic motor axonal neuropathy (CMAN), likely of immune-mediated origin.",
keywords = "acute motor axonal neuropathy, amyotrophic lateral sclerosis, anti-GM1 antibodies, lower motor neuron disease, nerve biopsy, neuropathology, peripheral neuropathy",
author = "Nilo Riva and Francesca Gallia and Sandro Iannaccone and Massimo Corbo and Fabrizia Terenghi and Alberto Lazzerini and Federica Cerri and Giancarlo Comi and Angelo Quattrini and Eduardo Nobile-Orazio",
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T1 - Chronic motor axonal neuropathy

AU - Riva, Nilo

AU - Gallia, Francesca

AU - Iannaccone, Sandro

AU - Corbo, Massimo

AU - Terenghi, Fabrizia

AU - Lazzerini, Alberto

AU - Cerri, Federica

AU - Comi, Giancarlo

AU - Quattrini, Angelo

AU - Nobile-Orazio, Eduardo

PY - 2011/12

Y1 - 2011/12

N2 - The identification of a distinct subgroup of patients within the spectrum of lower motor neuron syndromes (LMNS) is crucial as some are potentially treatable. We describe the clinical and neuropathological characteristics of a patient presenting with a rapidly progressive LMNS associated with high titers of anti-GM1 antibodies, leading to respiratory failure within 10 months. Histopathological study of a biopsy of a obturator nerve motor branch demonstrated a predominantly axonal motor neuropathy, while electron microscopy analysis localized macrophages located within the periaxonal space. Immunohistochemistry demonstrated deposits of complement activation products (C3i) and immunoglobulins (IgM) on nerve fibers. The patient's clinical, immunological and pathological findings are consistent with a diagnosis of a chronic motor axonal neuropathy (CMAN), likely of immune-mediated origin.

AB - The identification of a distinct subgroup of patients within the spectrum of lower motor neuron syndromes (LMNS) is crucial as some are potentially treatable. We describe the clinical and neuropathological characteristics of a patient presenting with a rapidly progressive LMNS associated with high titers of anti-GM1 antibodies, leading to respiratory failure within 10 months. Histopathological study of a biopsy of a obturator nerve motor branch demonstrated a predominantly axonal motor neuropathy, while electron microscopy analysis localized macrophages located within the periaxonal space. Immunohistochemistry demonstrated deposits of complement activation products (C3i) and immunoglobulins (IgM) on nerve fibers. The patient's clinical, immunological and pathological findings are consistent with a diagnosis of a chronic motor axonal neuropathy (CMAN), likely of immune-mediated origin.

KW - acute motor axonal neuropathy

KW - amyotrophic lateral sclerosis

KW - anti-GM1 antibodies

KW - lower motor neuron disease

KW - nerve biopsy

KW - neuropathology

KW - peripheral neuropathy

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