Cargando…

Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome

INTRODUCTION: Guillain-Barré syndrome (GBS) has been classified into demyelinating and axonal subtypes or forms, such as acute motor axonal neuropathy (AMAN) and regional pharyngeal-cervical-brachial variant (PCBv). OBJECTIVE: To study the relationship between motor nerve conduction blocks (CBs) and...

Descripción completa

Detalles Bibliográficos
Autores principales: Urdiales-Sánchez, Sara, González-Montaña, José-Ramiro, Diaz-Pérez, Ricardo, Calvo-Calleja, Pablo, Gutiérrez-Trueba, María-Antonia, Urdiales-Urdiales, Javier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9174782/
https://www.ncbi.nlm.nih.gov/pubmed/35693020
http://dx.doi.org/10.3389/fneur.2022.902172
_version_ 1784722314663821312
author Urdiales-Sánchez, Sara
González-Montaña, José-Ramiro
Diaz-Pérez, Ricardo
Calvo-Calleja, Pablo
Gutiérrez-Trueba, María-Antonia
Urdiales-Urdiales, Javier
author_facet Urdiales-Sánchez, Sara
González-Montaña, José-Ramiro
Diaz-Pérez, Ricardo
Calvo-Calleja, Pablo
Gutiérrez-Trueba, María-Antonia
Urdiales-Urdiales, Javier
author_sort Urdiales-Sánchez, Sara
collection PubMed
description INTRODUCTION: Guillain-Barré syndrome (GBS) has been classified into demyelinating and axonal subtypes or forms, such as acute motor axonal neuropathy (AMAN) and regional pharyngeal-cervical-brachial variant (PCBv). OBJECTIVE: To study the relationship between motor nerve conduction blocks (CBs) and prognosis in AMAN and PCBv. PATIENTS AND METHODS: We retrospectively analyzed six cases of AMAN and PCBv with serial nerve conduction studies (NCS) and electromyography (EMG). RESULTS: The serial NCS (1st−2nd and 3rd week) showed, as the most constant data, a decreased amplitude of the compound muscle action potential (CMAP) in 100% of cases. CBs were present in 66.6% of cases. EMG (3rd week) showed signs of severe denervation in 33.3%. All patients were treated from the 1st−2nd week of evolution with intravenous immunoglobulins (IVIGs). Patients with CBs (1st−2nd and 3rd week), showed reversible CBs or reversible conduction failure (RCF) and complete recovery at 1 month. Patients without CBs, with persistent reduced distal CMAP amplitude (dCMAP), showed severe acute denervation due to axonal degeneration (3rd week and 1st−3rd month) and a slow recovery of several months. CONCLUSIONS: Not all axonal forms of GBS have a poor prognosis. This study of AMAN and PCBv shows that patients with CBs can have reversible CBs or RCF, and good prognosis. Patients without CBs, with persistent reduction of dCMAP amplitude decrement, have severe acute denervation, and a worse prognosis. AMAN and PCBv have a continuous spectrum ranging from CBs due to dysfunction/disruption of Nodes of Ranvier, called nodopathies, with reversible CBs or RCF and good prognosis, to axonal degeneration with worse prognosis.
format Online
Article
Text
id pubmed-9174782
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-91747822022-06-09 Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome Urdiales-Sánchez, Sara González-Montaña, José-Ramiro Diaz-Pérez, Ricardo Calvo-Calleja, Pablo Gutiérrez-Trueba, María-Antonia Urdiales-Urdiales, Javier Front Neurol Neurology INTRODUCTION: Guillain-Barré syndrome (GBS) has been classified into demyelinating and axonal subtypes or forms, such as acute motor axonal neuropathy (AMAN) and regional pharyngeal-cervical-brachial variant (PCBv). OBJECTIVE: To study the relationship between motor nerve conduction blocks (CBs) and prognosis in AMAN and PCBv. PATIENTS AND METHODS: We retrospectively analyzed six cases of AMAN and PCBv with serial nerve conduction studies (NCS) and electromyography (EMG). RESULTS: The serial NCS (1st−2nd and 3rd week) showed, as the most constant data, a decreased amplitude of the compound muscle action potential (CMAP) in 100% of cases. CBs were present in 66.6% of cases. EMG (3rd week) showed signs of severe denervation in 33.3%. All patients were treated from the 1st−2nd week of evolution with intravenous immunoglobulins (IVIGs). Patients with CBs (1st−2nd and 3rd week), showed reversible CBs or reversible conduction failure (RCF) and complete recovery at 1 month. Patients without CBs, with persistent reduced distal CMAP amplitude (dCMAP), showed severe acute denervation due to axonal degeneration (3rd week and 1st−3rd month) and a slow recovery of several months. CONCLUSIONS: Not all axonal forms of GBS have a poor prognosis. This study of AMAN and PCBv shows that patients with CBs can have reversible CBs or RCF, and good prognosis. Patients without CBs, with persistent reduction of dCMAP amplitude decrement, have severe acute denervation, and a worse prognosis. AMAN and PCBv have a continuous spectrum ranging from CBs due to dysfunction/disruption of Nodes of Ranvier, called nodopathies, with reversible CBs or RCF and good prognosis, to axonal degeneration with worse prognosis. Frontiers Media S.A. 2022-05-25 /pmc/articles/PMC9174782/ /pubmed/35693020 http://dx.doi.org/10.3389/fneur.2022.902172 Text en Copyright © 2022 Urdiales-Sánchez, González-Montaña, Diaz-Pérez, Calvo-Calleja, Gutiérrez-Trueba and Urdiales-Urdiales. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Urdiales-Sánchez, Sara
González-Montaña, José-Ramiro
Diaz-Pérez, Ricardo
Calvo-Calleja, Pablo
Gutiérrez-Trueba, María-Antonia
Urdiales-Urdiales, Javier
Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title_full Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title_fullStr Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title_full_unstemmed Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title_short Nodopathies in the Early Diagnosis of Axonal Forms of Guillain-Barré Syndrome
title_sort nodopathies in the early diagnosis of axonal forms of guillain-barré syndrome
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9174782/
https://www.ncbi.nlm.nih.gov/pubmed/35693020
http://dx.doi.org/10.3389/fneur.2022.902172
work_keys_str_mv AT urdialessanchezsara nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome
AT gonzalezmontanajoseramiro nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome
AT diazperezricardo nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome
AT calvocallejapablo nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome
AT gutierreztruebamariaantonia nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome
AT urdialesurdialesjavier nodopathiesintheearlydiagnosisofaxonalformsofguillainbarresyndrome