Atypical chronic inflammatory demyelinating polyneuropathies

Kuwabara, Satoshi; Misawa, Sonoko; Mori, Masahiro

doi:10.1136/jnnp-2018-319411

Cited by 4 publications

(4 citation statements)

References 10 publications

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“…In this regard, each atypical CIDP subtype, and even typical CIDP, should be more strictly defined clinically. 3 The European Academy of Neurology/PNS (formerly EFNS/PNS) Task Force has just started the second revision of the CIDP guidelines which is expected to define clinical criteria for CIDP subtypes.…”

Section: Introductionmentioning

confidence: 99%

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

Lehmann

Burke

Kuwabara

2019

J Neurol Neurosurg Psychiatry

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153

109

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

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Section: Introductionmentioning

confidence: 99%

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

Lehmann

Burke

Kuwabara

2019

J Neurol Neurosurg Psychiatry

Self Cite

153

109

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“…Progression to typical CIDP, however, was not absolute and a considerable proportion of patients maintained their atypical CIDP phenotype even after several years from symptoms onset [ 7 ▪▪ ]. Transition from atypical to typical CIDP has been questioned by some authors [ 47 ] but other studies confirmed that phenotypes can change over times [ 9 , 34 , 35 ]. Whether this progression reflects only a greater spread of neuropathy or is instead the result of specific pathogenetic mechanisms is still to be clarified.…”

Section: Definitions and Clinical Characteristics Of The Atypical Chr...mentioning

confidence: 99%

Atypical chronic inflammatory demyelinating polyradiculoneuropathy: recent advances on classification, diagnosis, and pathogenesis

Doneddu

Dentoni

Orazio

2021

Current Opinion in Neurology

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Purpose of reviewIn recent years, there has been an intense debate in literature regarding the definition of the individual variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), their possible pathogenetic mechanisms, and impact in the diagnosis of CIDP.Recent findingsThe 2021 European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines revised the definition of the individual CIDP variants and implemented their diagnostic criteria. Diagnosis of atypical CIDP is challenging and misdiagnosis is frequent, leading to diagnostic delay and consequent greater accumulation of disability and treatment dependency. Recent studies suggest that patients with typical CIDP have an antibody-mediated mechanism of neuropathy whereas in those with Lewis--Sumner syndrome (LSS) neuropathy is preferentially mediated by macrophages and T cells.SummaryAlthough the validity of the 2021 EFNS/PNS diagnostic criteria for atypical CIDP is unknown, they will hopefully lead to greater uniformity in the selection of patients to be enrolled in future studies and to a greater diagnostic accuracy. New data are emerging on the possible pathological mechanisms of individual variants and this could result in the discovery of specific diagnostic biomarkers and new therapies.

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“…Chemical analysis of the cerebrospinal fluid of patients reveals the cell-protein separation phenomenon and electromyography indicates demyelinating or axon damage. Atypical manifestations of CIDP include simple motor dysfunction, simple sensory dysfunction, and Lewis–Sumner syndrome (multifocal demyelinating sensory and motor neuropathy with persistent conduction block), for which conventional immune therapy is not effective ( Kuwabara et al, 2015 , 2019 ). The prevalence of CIDP varies by country because of different diagnostic criteria used; it is estimated to be 1.6–8.9 per 100,000 adults ( Broers et al, 2019 ; Lehmann et al, 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Impact of Neurofascin on Chronic Inflammatory Demyelinating Polyneuropathy via Changing the Node of Ranvier Function: A Review

Gao

Kong

Liu

et al. 2021

Front. Mol. Neurosci.

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The effective conduction of action potential in the peripheral nervous system depends on the structural and functional integrity of the node of Ranvier and paranode. Neurofascin (NF) plays an important role in the conduction of action potential in a saltatory manner. Two subtypes of NF, NF186, and NF155, are involved in the structure of the node of Ranvier. In patients with chronic inflammatory demyelinating polyneuropathy (CIDP), anti-NF antibodies are produced when immunomodulatory dysfunction occurs, which interferes with the conduction of action potential and is considered the main pathogenic factor of CIDP. In this study, we describe the assembling mechanism and anatomical structure of the node of Ranvier and the necessary cell adhesion molecules for its physiological function. The main points of this study are that we summarized the recent studies on the role of anti-NF antibodies in the changes in the node of Ranvier function and its impact on clinical manifestations and analyzed the possible mechanisms underlying the pathogenesis of CIDP.

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Atypical chronic inflammatory demyelinating polyneuropathies

Cited by 4 publications

References 10 publications

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

Atypical chronic inflammatory demyelinating polyradiculoneuropathy: recent advances on classification, diagnosis, and pathogenesis

Impact of Neurofascin on Chronic Inflammatory Demyelinating Polyneuropathy via Changing the Node of Ranvier Function: A Review

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