Electrophysiologic lessons from the European multicenter study of Guillain‐Barré syndrome subtype diagnosis

Lewis, Richard A.

doi:10.1002/mus.26122

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…GBS variants and subtypes with anti‐gangliosides antibodies are mainly classified as “axonal.” However, the term may be deceptive as, in the common knowledge, it is correlated to axonal degeneration and unfavorable prognosis and not to the possibility of a transient dysfunction of the nodal region with prompt recovery. In the effort to resolve this contradiction the term “nodal GBS” has been suggested, 67 but in my opinion these disorders could be better grouped as acute nodo‐paranodopathies with anti‐ganglioside antibodies.…”

Section: Nodo‐paranodopathiesmentioning

confidence: 99%

Autoimmune nodo‐paranodopathies 10 years later: Clinical features, pathophysiology and treatment

Uncini

2023

J Peripheral Nervous Sys

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Background and Aims: Autoimmune neuropathies are classified, on the basis of pathophysiology, as demyelinating or axonal. The term nodo-paranodopathy, introduced in 2013 to better categorize the neuropathies with antiganglioside antibodies and later expanded to include neuropathies with antibodies to nodal and paranodal axoglial complexes, characterizes disorders in which the nodal region is critical in the pathogenesis. These neuropathies, although presenting electrophysiologic demyelinating features do not show pathologic evidence of segmental demyelination, or, although being classified as axonal, can show reversible nerve conduction failure and rapid recovery contrary with the communal concept of an axonal neuropathy. Methods:In this personal view is reported, with a splitting approach, an update on autoimmune nodo-paranodopathies, classified according to the domains of peripheral nerve fiber, the target antigens and the antibody class and subclass involved. The clinical features, the electrophysiologic findings, the results of the immunopathological and ultrastructural studies, the pathophysiology and treatment are also described. Results and Interpretation:The nodo-paranodopathy category integrates the clinical classification of autoimmune neuropathies and expands the traditional dichotomous demyelinating and axonal classification. It helps to a better systematization pointing to the domain and target antigens of the autoimmune process, it resolves conflicting pathologic and electrophysiologic findings, reconciles the contradiction that axonal neuropathies may be rapidly reversible, avoids taxonomical confusion and possible misdiagnoses. Finally this categorization, through the identification of the specific antibody and its prevalent class and subclass, clarifies the pathophysiological mechanisms and addresses to a more targeted therapeutic approach.

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Section: Nodo‐paranodopathiesmentioning

confidence: 99%

Autoimmune nodo‐paranodopathies 10 years later: Clinical features, pathophysiology and treatment

Uncini

2023

J Peripheral Nervous Sys

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“…The axonal label applied to GBS with anti-gangliosides antibodies may be misleading as the term is commonly associated to axonal degeneration and not to a transitory dysfunction of the nodal region. In the attempt to overcame this contradiction, the term “nodal GBS” has been proposed,37 but we think that these GBS subtypes are better categorised as acute nodo-paranodopathies with anti-ganglioside antibodies.…”

Section: Nodo-paranodopathies With Antibodies To Gangliosidesmentioning

confidence: 99%

New classification of autoimmune neuropathies based on target antigens and involved domains of myelinated fibres

Uncini

Mathis

Vallat

2021

J Neurol Neurosurg Psychiatry

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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, autoimmune disorders targeting specific molecules of the nodal region, although not showing pathological evidence of demyelination, can exhibit all the electrophysiological changes considered characteristic of a demyelinating neuropathy and acute neuropathies with antiganglioside antibodies, classified as axonal and due to nodal dysfunction, can present with reversible conduction failure and prompt recovery that appear contradictory with the common view of an axonal neuropathy. These observations bring into question the concepts of demyelinating and axonal nerve conduction changes and the groundwork of the classical dichotomous classification.We propose a classification of autoimmune neuropathies based on the involved domains of the myelinated fibre and, when known, on the antigen. This classification, in our opinion, helps to better systematise autoimmune neuropathies because points to the site and molecular target of the autoimmune attack, reconciles some contrasting pathological and electrophysiological findings, circumvents the apparent paradox that neuropathies labelled as axonal may be promptly reversible and finally avoids taxonomic confusion and possible misdiagnosis.

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“…In 2018, Uncini and Kuwabara discussed the four sets of the diagnostic criteria of axonal GBS and concluded that GBS subtype diagnosis is important for understanding the underlying pathophysiology and prognostication but not for impact on treatment 12 . In an editorial paper in Muscle & Nerve in 2018, Lewis also introduced a term “nodal GBS” for "axonal GBS" to accommodate the concept of nodopathy in the context of traditional concept of demyelination 17 …”

mentioning

confidence: 99%

“…This may explain the relatively complete recovery in "axonal GBS". This will also accommodate “nodal GBS” as suggested by Lewis as a sub‐type of AIDP 17 …”

mentioning

confidence: 99%

Nodal conduction block: A unifying concept

2020

Muscle and Nerve

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A newly introduced term, “axonal conduction block,” brought a confusion in the electrodiagnostic diagnosis of Guillain‐Barrè syndrome (GBS). I am proposing the term “nodal conduction block” for “axonal conduction block.” This unifying concept of nodal conduction block will accommodate both the traditional concept of demyelination as well as the new concept of nodopathy in the “axonal form of GBS,”, making the practice of electrodiagnosis much easier.

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Electrophysiologic lessons from the European multicenter study of Guillain‐Barré syndrome subtype diagnosis

Cited by 5 publications

References 14 publications

Autoimmune nodo‐paranodopathies 10 years later: Clinical features, pathophysiology and treatment

Autoimmune nodo‐paranodopathies 10 years later: Clinical features, pathophysiology and treatment

New classification of autoimmune neuropathies based on target antigens and involved domains of myelinated fibres

Nodal conduction block: A unifying concept

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