Nodal conduction block: A unifying concept

Oh, Shin J.

doi:10.1002/mus.27115

“…If an autoimmune attack is aborted, axonal degeneration does not progress and nodal function rapidly recovers, which represents the pathomechanism of RCF 12,13 . RCF in AMAN has been considered a sign of nodopathy and has a favorable prognosis 14 . Many previous reports have suggested that AMAN without RCF has the worst prognosis in GBS 15,16 , similar to the results of our study.…”

Section: Discussionmentioning

confidence: 99%

Reversible conduction failure in acute inflammatory demyelinating polyneuropathy

Kim

¹

,

Lee

²

,

Sohn

³

2022

Sci Rep

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Reversible conduction failure (RCF) has been documented in acute motor axonal neuropathy (AMAN) and is considered a sign of nodopathy. Several reports of RCF in acute inflammatory demyelinating polyneuropathy (AIDP) have suggested that it could be a manifestation of nodopathy. We conducted this study to determine the frequency of RCF in AMAN and AIDP and to compare the clinical features between the two groups with or without RCF. RCF was observed in 38.9% and 18.5% patients in the AMAN and AIDP groups in our study, respectively. AIDP patients with anti-ganglioside antibodies represented 29.4% of the cohort. The clinical features of AIDP with RCF were more similar to those of AMAN with RCF than to those of typical AIDP. However, there were no significant differences in the frequency of anti-ganglioside antibody status between the groups. AIDP with RCF may be a manifestation of nodopathy. The current dichotomous electrodiagnostic criteria, classifying demyelinating and axonal neuropathy, are insufficient to define nodopathy. Further studies are required to revise the electrodiagnostic criteria for Guillain–Barré syndrome.

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“…If an autoimmune attack is aborted, axonal degeneration does not progress and nodal function rapidly recovers, which represents the pathomechanism of RCF [22,23] . RCF in AMAN has been considered a sign of nodopathy and has a favorable prognosis [24] . Many previous reports have suggested that AMAN without RCF has the worst prognosis in GBS [10,13] , similar to the results of our study.…”

Section: Discussionmentioning

confidence: 99%

Electrodiagnosis of Guillain–Barré Syndrome Reflecting Acute Inflammatory Demyelinating Polyneuropathy with Reversible Conduction Failure: A Concept of Nodopathy

Kim

¹

,

Lee

²

,

Sohn

³

2022

Preprint

0

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Reversible conduction failure (RCF) has been documented in acute motor axonal neuropathy (AMAN) and is considered a sign of nodopathy. Several reports of RCF in acute inflammatory demyelinating polyneuropathy (AIDP) have suggested that it could be a manifestation of nodopathy. We conducted this study to determine the frequency of RCF in AMAN and AIDP and to compare the clinical features between the two groups with or without RCF. RCF was observed in 38.9% and 18.5% patients in the AMAN and AIDP groups in our study, respectively. AIDP patients with anti-ganglioside antibodies represented 29.4% of the cohort. The clinical features of AIDP with RCF were more similar to those of AMAN with RCF than to those of typical AIDP. However, there were no significant differences in the frequency of anti-ganglioside antibody status between the groups. AIDP with RCF may be a manifestation of nodopathy. The current dichotomous electrodiagnostic criteria, classifying demyelinating and axonal neuropathy, are insufficient to define nodopathy. Further studies are required to revise the electrodiagnostic criteria for Guillain–Barré syndrome.

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“…Recent understanding of the pathogenesis of immune-mediated neuropathies has highlighted a need for more systematic classification ( 4 , 10 , 11 ). Nerve biopsy is not recommended as a routine diagnostic procedure due to its infeasibility and morbidity ( 5 , 35 ).…”

Section: Discussionmentioning

confidence: 99%

“…The role of electrodiagnosis (EDX) is even more limited, as nodo-paranodopathy exhibits significant overlap with classical axonopathies or demyelinating neuropathies in terms of electrophysiological changes. Dysfunction of voltage-gated sodium channels at the node of Ranvier may delay the generation of action potentials leading to significant conduction slowing into the “demyelinating range” ( 10 , 11 , 19 ). Where axonal degeneration may follow nodal immune attack, EDX of nodo-paranodopathy can mimic that of classical axonopathy.…”

Section: Introductionmentioning

confidence: 99%

Skin Biopsy as a Novel Diagnostic Aid in Immune-Mediated Neuropathies

Min

¹

,

Ju

²

,

Ha

³

et al. 2022

Journal of Neuropathology &Amp; Experimental Neurology

0

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Immune-mediated neuropathies are a heterogenous group of inflammatory peripheral nerve disorders. They can be classified according to the domain where the autoimmune process begins: the internode, paranode, or node. However, conventional diagnostic tools, electrodiagnosis (EDX), and autoantibody testing do not fully address this issue. In this institutional cohort study, we investigated the value of dermal myelinated fiber analysis for target domain-based classification. Twenty-seven consecutive patients with immune-mediated neuropathies underwent skin biopsies. The sections were stained with antibodies representative of myelinated fiber domains and were scanned using a confocal microscope. Clinical and pathological features of each patient were reviewed comprehensively. Quantitative morphometric parameters were subjected to clustering analysis, which stratified patients into 3 groups. Cluster 1 (“internodopathy”) was characterized by prominent internodal disruption, intact nodes and paranodes, demyelinating EDX pattern, and absence of nodal-paranodal antibodies. Cluster 2 (“paranodopathy”) was characterized by paranodal disruption and corresponding antibodies. Morphological changes were restricted to the nodes in cluster 3; we designated this cluster as “nodopathy.” This report highlights the utility of skin biopsy as a diagnostic aid to gain pathogenic insight and classify patients with immune-mediated neuropathies.

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Nodal conduction block: A unifying concept

Cited by 12 publications

References 23 publications

Reversible conduction failure in acute inflammatory demyelinating polyneuropathy

Reversible conduction failure in acute inflammatory demyelinating polyneuropathy

Electrodiagnosis of Guillain–Barré Syndrome Reflecting Acute Inflammatory Demyelinating Polyneuropathy with Reversible Conduction Failure: A Concept of Nodopathy

Skin Biopsy as a Novel Diagnostic Aid in Immune-Mediated Neuropathies

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