Abstract

OBJECTIVES: A 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.

METHODS: We 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.

RESULTS: At 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.

CONCLUSIONS: The 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.

Original languageEnglish
Pages (from-to)125-132
Number of pages8
JournalJournal of neurology, neurosurgery, and psychiatry
Volume90
Issue number2
DOIs
Publication statusPublished - Feb 2019

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Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Databases
Therapeutics
Intravenous Immunoglobulins

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

In: Journal of neurology, neurosurgery, and psychiatry, Vol. 90, No. 2, 02.2019, p. 125-132.

Research output: Contribution to journalArticle

@article{1d2934ea531645a8969eaeca49da14d5,
title = "Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database",
abstract = "OBJECTIVES: A 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.METHODS: We 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.RESULTS: At 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.CONCLUSIONS: The 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.",
author = "{Italian CIDP Database study group} and Doneddu, {Pietro Emiliano} and Dario Cocito and Fiore Manganelli and Raffaella Fazio and Chiara Briani and Massimiliano Filosto and Luana Benedetti and Anna Mazzeo and Marfia, {Girolama Alessandra} and Andrea Cortese and Brigida Fierro and Stefano Jann and Ettore Beghi and Clerici, {Angelo Maurizio} and Marinella Carpo and Angelo Schenone and Marco Luigetti and Giuseppe Lauria and Giovanni Antonini and Tiziana Rosso and Gabriele Siciliano and Guido Cavaletti and Giuseppe Liberatore and Lucio Santoro and Erdita Peci and Stefano Tronci and Marta Ruiz and {Cotti Piccinelli}, Stefano and Antonio Toscano and Giorgia Mataluni and Laura Piccolo and Giuseppe Cosentino and Mario Sabatelli and Eduardo Nobile-Orazio",
note = "{\circledC} Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.",
year = "2019",
month = "2",
doi = "10.1136/jnnp-2018-318714",
language = "English",
volume = "90",
pages = "125--132",
journal = "Journal of Neurology, Neurosurgery and Psychiatry",
issn = "0022-3050",
publisher = "BMJ Publishing Group",
number = "2",

}

TY - JOUR

T1 - Atypical CIDP

T2 - diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database

AU - Italian CIDP Database study group

AU - Doneddu, Pietro Emiliano

AU - Cocito, Dario

AU - Manganelli, Fiore

AU - Fazio, Raffaella

AU - Briani, Chiara

AU - Filosto, Massimiliano

AU - Benedetti, Luana

AU - Mazzeo, Anna

AU - Marfia, Girolama Alessandra

AU - Cortese, Andrea

AU - Fierro, Brigida

AU - Jann, Stefano

AU - Beghi, Ettore

AU - Clerici, Angelo Maurizio

AU - Carpo, Marinella

AU - Schenone, Angelo

AU - Luigetti, Marco

AU - Lauria, Giuseppe

AU - Antonini, Giovanni

AU - Rosso, Tiziana

AU - Siciliano, Gabriele

AU - Cavaletti, Guido

AU - Liberatore, Giuseppe

AU - Santoro, Lucio

AU - Peci, Erdita

AU - Tronci, Stefano

AU - Ruiz, Marta

AU - Cotti Piccinelli, Stefano

AU - Toscano, Antonio

AU - Mataluni, Giorgia

AU - Piccolo, Laura

AU - Cosentino, Giuseppe

AU - Sabatelli, Mario

AU - Nobile-Orazio, Eduardo

N1 - © Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.

PY - 2019/2

Y1 - 2019/2

N2 - OBJECTIVES: A 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.METHODS: We 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.RESULTS: At 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.CONCLUSIONS: The 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.

AB - OBJECTIVES: A 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.METHODS: We 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.RESULTS: At 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.CONCLUSIONS: The 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.

U2 - 10.1136/jnnp-2018-318714

DO - 10.1136/jnnp-2018-318714

M3 - Article

VL - 90

SP - 125

EP - 132

JO - Journal of Neurology, Neurosurgery and Psychiatry

JF - Journal of Neurology, Neurosurgery and Psychiatry

SN - 0022-3050

IS - 2

ER -