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Abstract
Discussion Forum (0)
Autoimmune neuropathies are named by eponyms, by descriptive terminology or because of the presence of specific antibodies and are traditionally classified, on the basis of pathology and electrophysiology, as primary demyelinating or axonal. However, acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction/disruption, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. Moreover patients with antibodies (mainly IgG4) against paranodal junction components, often with distinct clinical features and no or poor response to IVIg, have been classified as CIDP although not showing macrophage-mediated demyelination or inflammation. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the nodo-paranodopathy category has been proposed to better characterise these disorders and overcome some inadequacies of the classical dichotomous classification.
Autoimmune neuropathies are named by eponyms, by descriptive terminology or because of the presence of specific antibodies and are traditionally classified, on the basis of pathology and electrophysiology, as primary demyelinating or axonal. However, acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction/disruption, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. Moreover patients with antibodies (mainly IgG4) against paranodal junction components, often with distinct clinical features and no or poor response to IVIg, have been classified as CIDP although not showing macrophage-mediated demyelination or inflammation. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the nodo-paranodopathy category has been proposed to better characterise these disorders and overcome some inadequacies of the classical dichotomous classification.
Nodo-Paranodopathies: Clinical Phenotypes and Treatment
Antonino Uncini
Affiliations:
Antonio Uncini, MD, University “G. d’Annunzio” Chieti-Pescara, Italy, Monsampolo del Tronto, Italy
Antonio Uncini, MD, University “G. d’Annunzio” Chieti-Pescara, Italy, Monsampolo del Tronto, Italy
Abstract
Discussion Forum (0)
Autoimmune neuropathies are named by eponyms, by descriptive terminology or because of the presence of specific antibodies and are traditionally classified, on the basis of pathology and electrophysiology, as primary demyelinating or axonal. However, acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction/disruption, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. Moreover patients with antibodies (mainly IgG4) against paranodal junction components, often with distinct clinical features and no or poor response to IVIg, have been classified as CIDP although not showing macrophage-mediated demyelination or inflammation. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the nodo-paranodopathy category has been proposed to better characterise these disorders and overcome some inadequacies of the classical dichotomous classification.
Autoimmune neuropathies are named by eponyms, by descriptive terminology or because of the presence of specific antibodies and are traditionally classified, on the basis of pathology and electrophysiology, as primary demyelinating or axonal. However, acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction/disruption, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. Moreover patients with antibodies (mainly IgG4) against paranodal junction components, often with distinct clinical features and no or poor response to IVIg, have been classified as CIDP although not showing macrophage-mediated demyelination or inflammation. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the nodo-paranodopathy category has been proposed to better characterise these disorders and overcome some inadequacies of the classical dichotomous classification.
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