Different electrophysiological profiles and treatment response in ‘typical’ and ‘atypical’ chronic inflammatory demyelinating polyneuropathy

Kuwabara, Satoshi; Isose, Sagiri; Mori, Masahiro; Mitsuma, Satsuki; Sawai, Setsu; Beppu, Minako; Sekiguchi, Yukari; Misawa, Sonoko

doi:10.1136/jnnp-2014-308452

Cited by 102 publications

(147 citation statements)

References 32 publications

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“…Distribution patterns of conduction abnormalities was demonstrated to determine clinical phenotypes 19. It was also reported that electrophysiological profiles and treatment response were different between typical CIDP and MADSAM 10. Our results corroborate and expand findings of previous reports on the clinical–electrophysiological correlations in typical and atypical CIDP.…”

Section: Discussionsupporting

confidence: 90%

“…Because no formal clinical diagnostic criteria were available for these atypical subtypes, we adopted the following operational definitions as described in previous studies. MADSAM is defined as a typical mononeuropathy multiplex, and the asymmetry of weakness is determined as differences in muscle strength by one or more Medical Research Council (MRC) scales in the homonymous muscles 10. DADS is defined as a predominantly distal and symmetric sensorimotor polyneuropathy sparing proximal limb muscles 9.…”

Section: Methodsmentioning

confidence: 99%

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Electrodiagnostic data-driven clustering identifies a prognostically different subgroup of patients with chronic inflammatory demyelinating polyneuropathy

Baek

Hong

Choi

et al. 2019

J Neurol Neurosurg Psychiatry

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ObjectiveThis study aimed to explore the correlations between electrodiagnostic (EDX) features in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and to investigate whether EDX data-driven clustering can identify a distinct subgroup regarding clinical phenotype and treatment response.MethodsWe reviewed clinical and EDX data of 56 patients with definite CIDP fulfilling the 2010 European Federation of Neurological Societies and Peripheral Nerve Society criteria at two teaching hospitals. A hierarchical agglomerative clustering algorithm with complete linkage was used to partition the patients into subgroups with similar EDX features. A stepwise logistic regression analysis was performed to evaluate predictors of the long-term outcome.ResultsEDX data-driven clustering partitioned the patients into two clusters, identifying a distinct subgroup characterised by coexistence of prominent conduction slowing and markedly reduced distally evoked compound muscle action potential (CMAP) amplitudes. This cluster of patients was significantly over-represented by an atypical subtype (distal acquired demyelinating symmetric polyneuropathy) compared with the other cluster (70% vs 26.1%, p=0.042). Furthermore, patients in this cluster invariably showed favourable long-term treatment outcome (100% vs 63%, p=0.023). In logistic regression analyses, the initial disability (OR 6.1, 95% CI 2.4 to 25.4), F-wave latency (OR 0.93, 95% CI 0.86 to 0.98) and distal CMAP duration (OR 0.96, 95% CI 0.91 to 0.99) were significant predictors of the poor long-term outcome.ConclusionOur results show that EDX data-driven clustering could differentiate a pattern of EDX features with prognostic implication in patients with CIDP. Reduced distally evoked CMAPs may not necessarily predict poor responses to treatment, and active treatment is warranted when prominent slowing of conduction is accompanied in the distal segments.

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Section: Discussionsupporting

confidence: 90%

Section: Methodsmentioning

confidence: 99%

Electrodiagnostic data-driven clustering identifies a prognostically different subgroup of patients with chronic inflammatory demyelinating polyneuropathy

Baek

Hong

Choi

et al. 2019

J Neurol Neurosurg Psychiatry

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“…A better response to IVIg than to steroids was reported in some series of patients with LSS,4 5 7 16 17 while others6 reported a reduced response to IVIg but not to steroids compared with typical CIDP. Patients with DADS were reported to have a less satisfactory response to treatment than patients with typical CIDP 18 19.…”

Section: Discussionmentioning

confidence: 91%

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

Doneddu

Cocito

Manganelli

et al. 2018

J Neurol Neurosurg Psychiatry

120

154

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ObjectivesA few variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been described, but their frequency and evolution to typical CIDP remain unclear. To determine the frequency and characteristics of the CIDP variants, their possible evolution to typical CIDP, and treatment response.MethodsWe applied a set of diagnostic criteria to 460 patients included in a database of Italian patients with CIDP. Clinical characteristics and treatment response were reviewed for each patient. The Kaplan-Meier curve was used to estimate the progression rate from atypical to typical CIDP.ResultsAt the time of inclusion, 376 (82%) patients had a diagnosis of typical CIDP while 84 (18%) had atypical CIDP, including 34 (7%) with distal acquired demyelinating symmetric neuropathy (DADS), 17 (4%) with purely motor, 17 (4%) with Lewis-Sumner syndrome (LSS) and 16 (3.5%) with purely sensory CIDP. Based on retrospective review of the symptoms and signs present at onset and for at least 1 year, 180 (39%) patients had an initial diagnosis compatible with atypical CIDP that in 96 (53%) patients evolved to typical CIDP. Mean disease duration was longer in patients evolving to typical CIDP than in those not evolving (p=0.0016). Patients with DADS and LSS had a less frequent response to immunoglobulin than those with typical CIDP, while patients with purely motor and sensory CIDP had a similar treatment response.ConclusionsThe proportion of patients with atypical CIDP varies during the disease course. DADS and LSS have a less frequent response to intravenous immunoglobulin compared with typical CIDP, raising the possibility of a different underlying pathogenetic mechanism.

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“…CIDP is an autoimmune-mediated peripheral nerve disease, with segmental demyelination and remyelination resulting in onion bulb formation and varying degrees of interstitial edema and endoneurial inflammation (7). The clinical presentation of CIDP is variable, including typical (proximal and distal weakness with large-fiber sensory loss), atypical (distal large-fiber sensorimotor neuropathy), and multifocal sensorimotor neuropathy (Lewis–Sumner syndrome) (8, 9). The heterogeneous morphology by ultrasound may reflect both the clinical and pathological heterogeneities of the disease.…”

Section: Discussionmentioning

confidence: 99%

Multiple Sites Ultrasonography of Peripheral Nerves in Differentiating Charcot–Marie–Tooth Type 1A from Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Niu

Liu

2017

Front. Neurol.

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IntroductionMultiple sites measurement of cross-sectional areas (CSA) by ultrasound was performed to differentiate Charcot–Marie–Tooth type 1A (CMT1A) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).MethodsNine patients with CMT1A, 28 patients with CIDP, and 14 healthy controls (HC) were recruited prospectively. Consecutive ultrasonography scanning was performed from wrist to axilla on median and ulnar nerves. CSAs were measured at 10 predetermined sites of each nerve.ResultsCMT1A had significantly larger CSAs at all sites of median and ulnar nerves (p < 0.01). In CMT1A, CSAs increased gradually and homogeneously from distal to proximal along the nerve, except potential entrapment sites. CIDP displayed three different morphological patterns, including mild enlargement in 15 patients, prominent segmental enlargement in 12, and slight enlargement in 1, among which different treatment responses were observed. All patients with mild nerve enlargement treated with intravenous immunoglobulin were responsive (7/7), while less than half of those with prominent segmental enlargement (3/7) were responsive (p < 0.01).DiscussionConsecutive scan along the nerve and multiple sites measurement by ultrasound could supply more detailed morphological feature of the nerve and help to differentiate CMT1A from CIDP.

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Different electrophysiological profiles and treatment response in ‘typical’ and ‘atypical’ chronic inflammatory demyelinating polyneuropathy

Cited by 102 publications

References 32 publications

Electrodiagnostic data-driven clustering identifies a prognostically different subgroup of patients with chronic inflammatory demyelinating polyneuropathy

Electrodiagnostic data-driven clustering identifies a prognostically different subgroup of patients with chronic inflammatory demyelinating polyneuropathy

Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

Multiple Sites Ultrasonography of Peripheral Nerves in Differentiating Charcot–Marie–Tooth Type 1A from Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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