Therapeutic strategy and electron microscopic abnormality in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 igG4 antibody

Kuwahara, Motoi; Suzuki, Hidekazu; Oka, Naoki; Yanagimoto, Satoshi; Sadakane, Shuji; Fukumoto, Yuta; Yamana, Masaki; Kawai, Shigeto; Okazaki, Masahiro; Kusunoki, Susumu

doi:10.1016/j.jns.2017.08.1829

Cited by 2 publications

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“…An assessment of longitudinal sections revealed the detachment of the myelin terminal loops from the axolemma at the paranode, a finding consistent with a previous report on neurofascin 155 antibody-positive CIDP cases ( Fig. 1 ) ( 14 , 18 , 19 ).…”

Section: Case Reportsupporting

confidence: 91%

“…In the present case, positivity for anti-neurofascin 155 antibodies was detected at 23 years after the onset of facial symptoms and 19 years after the initiation of steroid treatment. An antibody examination was not performed in the initial phase of neuropathy, but a sural nerve biopsy specimen obtained before the initiation of treatment showed a typical axo-glial detachment at the paranode, which has also been previously reported in anti-neurofascin 155 antibody-positive CIDP cases ( 14 , 18 , 19 ).…”

Section: Discussionsupporting

confidence: 52%

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Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years

et al. 2018

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We herein report a woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom positivity for anti-neurofascin 155 antibodies was revealed 23 years after the onset of neuropathy. The patient initially reported numbness in the face at 50 years of age and subsequently manifested features compatible to typical CIDP. Steroid administration initiated at 54 years of age ameliorated her neuropathic symptoms. Although the nerve conduction indices at 59 years of age deteriorated, those at 68, 72, and 73 years of age showed a gradual recovery. The deterioration and subsequent restoration of compound muscle action potential amplitudes was the most dramatic, suggesting that a conduction block can be reversed earlier than other electrophysiological indices.

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Section: Case Reportsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 52%

Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years

et al. 2018

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“…As IgG4 is a main immunoglobulin subclass of anti-neurofascin 155 and anti-contactin 1 antibodies, a low capacity to bind Fc receptors and an inability to activate complements of this subclass of immunoglobulin may explain the poor response to IVIg observed in patients with these antibodies [10,11]. In addition, immunoadsorption plasmapheresis should be avoided in these patients because it does not eliminate IgG4 [67]. It has been suggested that rituximab, a chimeric monoclonal antibody that binds to CD20, could have efficacy in patients with antineurofascin 155 and anti-contactin 1 antibodies [68].…”

Section: Insights Into Classification and Therapeutic Strategiesmentioning

confidence: 99%

Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

Koike

Katsuno

2020

Neurol Ther

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is classically defined as polyneuropathy with symmetric involvement of the proximal and distal portions of the limbs. In addition to this ''typical CIDP'', the currently prevailing diagnostic criteria proposed by the European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) define ''atypical CIDP'' as encompassing the multifocal acquired demyelinating sensory and motor (MADSAM), distal acquired demyelinating symmetric (DADS), pure sensory, pure motor, and focal subtypes. Although macrophage-induced demyelination is considered pivotal to the pathogenesis of CIDP, recent studies have indicated the presence of distinctive mechanisms initiated by autoantibodies against paranodal junction proteins, such as neurofascin 155 and contactin 1. These findings led to the emergence of the concept of nodopathy or paranodopathy. Patients with these antibodies tend to show clinical features compatible with typical CIDP or DADS, particularly the latter. In contrast, classical macrophage-induced demyelination is commonly found in some patients in each major subtype, including the typical CIDP, DADS, MADSAM, and pure sensory subtypes. Differences in the distribution of lesions and the repair processes underlying demyelination by Schwann cells may determine the differences among subtypes. In particular, the preferential involvement of proximal and distal nerve segments has been suggested to occur in typical CIDP, whereas the involvement of the middle nerve segments is conspicuous in MADSAM. These findings suggest that humoral rather than cellular immunity predominates in the former because nerve roots and neuromuscular junctions lack blood-nerve barriers. Treatment for CIDP consists of intravenous immunoglobulin (IVIg) therapy, steroids, and plasma exchange, either alone or in combination. However, patients with anti-neurofascin 155 and contactin 1 antibodies are refractory to IVIg. It has been suggested that rituximab, a monoclonal antibody to CD20, could have efficacy in these patients. Further studies are needed to validate the CIDP subtypes defined by the EFNS/PNS from the viewpoint of pathogenesis and establish therapeutic strategies based on the pathophysiologies specific to each subtype.

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Therapeutic strategy and electron microscopic abnormality in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 igG4 antibody

Cited by 2 publications

References 0 publications

Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years

Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years

Pathophysiology of Chronic Inflammatory Demyelinating Polyneuropathy: Insights into Classification and Therapeutic Strategy

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