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Chronic motor axonal neuropathy
Author(s) -
Riva Nilo,
Gallia Francesca,
Iannaccone Sandro,
Corbo Massimo,
Terenghi Fabrizia,
Lazzerini Alberto,
Cerri Federica,
Comi Giancarlo,
Quattrini Angelo,
NobileOrazio Eduardo
Publication year - 2011
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1111/j.1529-8027.2011.00366.x
Subject(s) - pathological , medicine , pathology , nerve biopsy , motor neuron , acute motor axonal neuropathy , antibody , immunohistochemistry , peripheral neuropathy , electromyography , disease , immunology , physical medicine and rehabilitation , endocrinology , diabetes mellitus
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.