Different distribution of demyelination in chronic inflammatory demyelinating polyneuropathy subtypes

Shibuya, Kazumoto; Tsuneyama, Atsuko; Misawa, Sonoko; Sekiguchi, Yukari; Beppu, Minako; Suichi, Tomoki; Suzuki, Yôichi; Nakamura, Keigo; Kondo, Hiroki; Kuwabara, Satoshi

doi:10.1016/j.jneuroim.2020.577170

Cited by 11 publications

(13 citation statements)

References 17 publications

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“…Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated neuropathy, characterized by a relapsing-remitting course and evidence of peripheral nerve demyelination [1]. Recent advances in immunological, neurophysiological and neuroimaging research have disclosed several different pathomechanisms according to the subtypes of CIDP [1][2][3][4][5][6]. The European Federation of Neurological Societies/ Peripheral Nerve Society (EFNS/PNS) criteria proposed clinical subtypes, such as typical CIDP, multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), distal acquired demyelinating symmetric (DADS) and others, based on clinical manifestations [7].…”

Section: Introductionmentioning

confidence: 99%

Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies

Shibuya

Tsuneyama

Misawa

et al. 2020

Euro J of Neurology

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Background and purpose Cranial nerve palsy is occasionally present in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), but its prevalence, characteristics and relations with the CIDP subtypes have rarely been investigated. The aim of this study was to systematically assess cranial nerve involvement in typical and atypical CIDP. Methods Clinical data were reviewed in 132 consecutive patients with CIDP, including typical CIDP (n = 89), multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) (n = 31), distal acquired demyelinating symmetric (DADS) (n = 9) and others (n = 3). Results The frequency of cranial nerve palsy was 11% in typical CIDP, 48% in MADSAM and 11% in DADS. Facial and bulbar palsy was most frequently present (9%), followed by ocular motor nerve palsy (5%). Bilateral involvement was seen in all typical CIDP and DADS patients, whereas 80% of MADSAM patients had unilateral palsy. The presence of cranial nerve involvement was associated with more severe limb muscle weakness in typical CIDP, but not in MADSAM. Cranial nerve palsy fully recovered in 90% of typical CIDP and in 67% of MADSAM patients. Conclusion Amongst the CIDP subtypes, cranial palsy is frequent and unilateral in MADSAM, and less frequent and bilateral in typical CIDP and DADS. In typical CIDP, facial and bulbar palsy reflects more severe and extensive inflammation.

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Section: Introductionmentioning

confidence: 99%

Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies

Shibuya

Tsuneyama

Misawa

et al. 2020

Euro J of Neurology

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“…Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated inflammatory neuropathy, consisting of several clinical subtypes, including typical, multifocal, distal, and others, according to the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) criteria. 1 These clinical subtypes exhibit different clinical characteristics and therapeutic responses, [1][2][3][4][5] suggesting different underlying pathophysiologies.…”

Section: Introductionmentioning

confidence: 99%

Different patterns of sensory nerve involvement in chronic inflammatory demyelinating polyneuropathy subtypes

et al. 2022

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Introduction/Aims: Among subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP), different immune pathophysiologies have been proposed. In this study, sensory nerve conduction studies were compared among clinical subtypes to attempt to better understand the underlying pathophysiology. Methods: A total of 138 patients with CIDP was classified into clinical subtypes: typical CIDP (N = 68), multifocal CIDP (N = 27), or other (N = 2). Patients with immunoglobulin M (IgM) neuropathy anti-myelin-associated glycoprotein neuropathy (MAG; N = 19) were also included as disease controls. Sensory nerve action potentials (SNAPs) were recorded in the median, ulnar, and superficial radial and sural nerves.Results: SNAP amplitudes (P < .05) and conduction velocities (P < .01) in the median nerve and conduction velocities (P < .05) in the ulnar nerve were lower in typical CIDP than in multifocal CIDP, whereas those in the radial and sural nerves were comparable in each group. Low median and normal sural SNAP amplitudes were more common in typical CIDP (P < .005) than in multifocal CIDP, suggesting predominant involvement at terminal portions of the nerves.Discussion: Terminal portions of sensory nerves are preferentially affected in typical CIDP compared with multifocal CIDP. These findings might be partially explained by the hypothesis of antibody-mediated demyelination in typical CIDP at the regions where the blood-nerve barrier is anatomically deficient, whereas multifocal CIDP predominantly affects the nerve trunks, largely due to cell-mediated demyelination, with disruption of the blood-nerve barrier.

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“…3 Several diseases affecting PNS myelin have been described, including (1) peripheral nerve trauma, 4 (2) autoimmune polyneuropathies such as Guillain-Barr e Syndrome, 5 chronic inflammatory demyelinating polyradiculo-neuropathy, 6 and (3) inherited demyelinating sensory and motor neuropathies, including Charcot-Marie-Tooth disease (CMT). 7 In this review, we will focus on CMT, the most frequent inherited diseases of the PNS with a prevalence of 1/1500 to 1/10000 worldwide. [8][9][10][11] CMT are progressive neuromuscular disorders characterized by degeneration of the peripheral nerves leading to muscle atrophy, weakness in the distal limbs, absence of deep tendon reflexes, and osteoarticular deformations such as pescavus.…”

Section: Introductionmentioning

confidence: 99%

Treating PMP22 gene duplication-related Charcot-Marie-Tooth disease: the past, the present and the future

Boutary

Echaniz‐Laguna

Adams

et al. 2021

Translational Research

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Tooth (CMT) disease is the most frequent inherited neuropathy, affecting 1/1500 to 1/10000. CMT1A represents 60%-70% of all CMT and is caused by a duplication on chromosome 17p11.2 leading to an overexpression of the Peripheral Myelin Protein 22 (PMP22). PMP22 gene is under tight regulation and small changes in its expression influences myelination and affect motor and sensory functions. To date, CMT1A treatment is symptomatic and classic pharmacological options have been disappointing. Here, we review the past, present, and future treatment options for CMT1A, with a special emphasis on the highly promising potential of PMP22-targeted small interfering RNA and antisense oligonucleotides.

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Different distribution of demyelination in chronic inflammatory demyelinating polyneuropathy subtypes

Cited by 11 publications

References 17 publications

Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies

Cranial nerve involvement in typical and atypical chronic inflammatory demyelinating polyneuropathies

Different patterns of sensory nerve involvement in chronic inflammatory demyelinating polyneuropathy subtypes

Treating PMP22 gene duplication-related Charcot-Marie-Tooth disease: the past, the present and the future

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