TY - JOUR
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
UR - http://www.scopus.com/inward/record.url?scp=83755184165&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=83755184165&partnerID=8YFLogxK
U2 - 10.1111/j.1529-8027.2011.00366.x
DO - 10.1111/j.1529-8027.2011.00366.x
M3 - Article
C2 - 22176149
AN - SCOPUS:83755184165
VL - 16
SP - 341
EP - 346
JO - Journal of the Peripheral Nervous System
JF - Journal of the Peripheral Nervous System
SN - 1085-9489
IS - 4
ER -