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Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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Efficacy and Safety of Rituximab in Refractory CIDP With or Without IgG4 Autoantibodies (RECIPE): Protocol for a Double-Blind, Randomized, Placebo-Controlled Clinical Trial.

Shinobu Shimizu, Masahiro Iijima, Yuki Fukami, Natsuko Tamura, Masahiro Nakatochi, Masahiko Ando, Ryoji Nishi, Haruki Koike, Kenichi Kaida, Michiaki Koga, Takashi Kanda, Hidenori Ogata, Jun-Ichi Kira, Masahiro Mori, Satoshi Kuwabara, Masahisa Katsuno,

BACKGROUND:Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral neuropathy that is currently classified into several clinical subtypes, which are presumed to have different pathogenic mechanisms. Recently, studies identified a subgroup of patients with CIDP who were positive for IgG4 autoantibodies against paranodal proteins, such as neurofascin-155 and contactin-1, who ... Read more >>

JMIR Res Protoc (JMIR research protocols)
[2020, 9(4):e17117]

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Use of Magnetic Resonance Neurography for Evaluating the Distribution and Patterns of Chronic Inflammatory Demyelinating Polyneuropathy.

Xiaoyun Su, Xiangquan Kong, Zuneng Lu, Min Zhou, Jing Wang, Xiaoming Liu, Xiangchuang Kong, Huiting Zhang, Chuansheng Zheng,

OBJECTIVE:To evaluate the distribution and characteristics of peripheral nerve abnormalities in chronic inflammatory demyelinating polyneuropathy (CIDP) using magnetic resonance neurography (MRN) and to examine the diagnostic efficiency. MATERIALS AND METHODS:Thirty-one CIDP patients and 21 controls underwent MR scans. Three-dimensional sampling perfections with application-optimized contrasts using different flip-angle evolutions and T1-/T2- ... Read more >>

Korean J Radiol (Korean journal of radiology)
[2020, 21(4):483-493]

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The Misdiagnosis of CIDP: A Review.

Jeffrey A Allen,

There is a growing realization that many patients are incorrectly diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), with at least half of patients that carry a diagnosis of CIDP in the USA possibly having a different explanation for their neuropathy or having no neuropathy at all. Many misdiagnosed patients go ... Read more >>

Neurol Ther (Neurology and therapy)
[2020, :]

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Abnormality of multimodal evoked potentials in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Edyta Dziadkowiak, Maria Ejma, Malgorzata Wieczorek, Pawel Pec, Krzysztof Slotwiński, Slawomir Budrewicz, Anna Zimny, Magdalena Koszewicz,

INTRODUCTION:Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, sometimes including the central nervous system. The aim of the study was the assessment of the prevalence of central sensory impairment and its reliance on peripheral nerve damage in patients with CIDP. MATERIAL AND METHODS:Multimodal (visual-VEP, ... Read more >>

Neurol. Sci. (Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology)
[2020, :]

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Immune-mediated inflammatory polyneuropathy overlapping Charcot-Marie-Tooth 1B.

Marcio Luiz Escorcio-Bezerra, Wladimir Bocca Vieira Rezende Pinto, Denis Bernardi Bichuetti, Paulo Victor Sgobbi Souza, Richard Mady Nunes, Luiz Henrique Libardi Silva, Karlla Danielle Ferreira Lima, Gilberto Mastrocola Manzano, Acary Souza Bulle Oliveira, Alex Machado Baeta,

Charcot Marie Tooth (CMT) due to myelin protein zero (MPZ) mutations, may cause a wide variation of phenotypes, depending on the localization of the mutation within the gene. Among the most common phenotypes are: an infantile onset disease with extremely slow nerve conduction velocities (CMT1B) and an adult onset phenotype ... Read more >>

J Clin Neurosci (Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia)
[2020, :]

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Absent well-characterized onconeural antibodies in 283 patients with various polyneuropathies: A retrospective monocenter study.

Benjamin Berger, Alexander Jürgen Klingler, Sebastian Rauer, Oliver Stich,

BACKGROUND:Paraneoplastic neurological syndromes (PNS) might present as polyneuropathies (PNP). Because PNS are rare and PNP are frequent, it may be difficult to decide in patients with PNP of unclear or presumably idiopathic etiology whether to test for onconeural antibodies, which are highly predictive for PNS. In this regard, this is ... Read more >>

J. Neurol. Sci. (Journal of the neurological sciences)
[2020, 413:116804]

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Bendamustine-rituximab (BR) combined therapy for treatment of immuno-mediated neuropathies associated with hematologic malignancy.

F Massa, A Zuppa, G Pesce, C Demichelis, M Bergamaschi, M Garnero, C Briani, S Ferrari, A Schenone, L Benedetti,

In chronic polyneuropathies associated with hematologic malignancy (HM) the optimal treatment management is primarily focused on the HM, but the parallel response of the neuropathy is still unclear. Rituximab is a recognized therapeutic choice in anti-MAG antibody polyneuropathy, that might be useful also in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with ... Read more >>

J. Neurol. Sci. (Journal of the neurological sciences)
[2020, 413:116777]

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Quantitative sudomotor test helps differentiate transthyretin familial amyloid polyneuropathy from chronic inflammatory demyelinating polyneuropathy.

E Fortanier, E Delmont, A Verschueren, S Attarian,

OBJECTIVE:Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an aggressive hereditary neuropathy characterized by sensory and autonomic dysfunction. There are numerous reports of TTR-FAP misdiagnosed and treated as chronic inflammatory demyelinating polyneuropathy (CIDP), leading to delayed diagnosis, risk of iatrogenic adverse events and increased socio-economic costs. Quantitative sudomotor function measured by electrochemical ... Read more >>

Clin Neurophysiol (Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology)
[2020, 131(5):1129-1133]

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Deciphering immune mechanisms in chronic inflammatory demyelinating polyneuropathies.

Jolien Wolbert, Mandy I Cheng, Gerd Meyer Zu Horste, Maureen A Su,

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nerves that presents with either chronic progression or relapsing disease. Recent studies in samples from patients with CIDP and mouse models have delineated how defects in central (thymic) and peripheral (extrathymic) immune tolerance mechanisms can cause PNS autoimmunity. ... Read more >>

JCI Insight (JCI insight)
[2020, 5(3):]

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Ultrastructural Lesions of Nodo-Paranodopathies in Peripheral Neuropathies.

Jean-Michel Vallat, Laurent Magy, Philippe Corcia, Jean-Marc Boulesteix, Antonino Uncini, Stéphane Mathis,

Whatever the cause of myelin damage of the peripheral nervous system, the initial attack on myelin by a dysimmune process may begin either at the internodal area or in the paranodal and nodal regions. The term "nodo-paranodopathy" was first applied to some "axonal Guillain-Barré syndrome" subtypes, then extended to cases ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(3):247-255]

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Epidemiology of chronic inflammatory demyelinating polyneuropathy in the South-Eastern area of Santiago, Chile.

Gabriel Cea, Juan Francisco Idiáquez, Rodrigo Salinas, José Manuel Matamala, Roque Villagra, Andrés Stuardo,

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy. It usually has an insidious onset, progressive course and heterogeneous clinical features. As far as we know, there is no epidemiological information on CIDP in South America and the Caribbean. Our aim was to estimate the frequency of CIDP in the ... Read more >>

J Clin Neurosci (Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia)
[2020, :]

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Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins.

Ludivine Kouton, José Boucraut, Jérome Devaux, Yusuf A Rajabally, David Adams, Jean Christophe Antoine, Frédéric Bourdain, Alexandre Brodovitch, Jean-Philippe Camdessanché, Cécile Cauquil, Jonathan Ciron, Thierry Dubard, Andoni Echaniz-Laguna, Aude-Marie Grapperon, Raul Juntas-Morales, Laurent Kremer, Thierry Kuntzer, Céline Labeyrie, Luca Lanfranco, Jean-Marc Léger, Thierry Maisonobe, Nicolas Mavroudakis, Sylvie Mecharles-Darrigol, Philippe Merle, Jean-Baptiste Noury, Violaine Rouaud, Céline Tard, Marie Théaudin, Jean-Michel Vallat, Karine Viala, Shahram Attarian, Emilien Delmont,

OBJECTIVE:Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) with antibodies against neurofascin 155 (Nfasc155) or contactin-1 (CNTN1) have distinctive clinical features. Knowledge on their electrophysiological characteristics is still scarce. In this study, we are investigating whether these patients have specific electrophysiological characteristics. METHODS:The electrophysiological data from 13 patients with anti-Nfasc155 IgG4 antibodies, 9 ... Read more >>

Clin Neurophysiol (Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology)
[2020, 131(4):921-927]

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Patients with chronic autoimmune demyelinating polyneuropathies exhibit cognitive deficits which might be associated with CSF evidence of blood-brain barrier disturbance.

Yavor Yalachkov, Valerie Uhlmann, Johannes Bergmann, Dilara Soydaş, Stefan Frisch, Marion Behrens, Christian Foerch, Johannes Gehrig,

BACKGROUND:Chronic autoimmune demyelinating polyneuropathies (CADP) result in impaired sensorimotor function. However, anecdotal clinical observations suggest the development of cognitive deficits during the course of disease. METHODS:We tested 16 patients with CADP (11 patients with chronic inflammatory demyelinating polyneuropathy, 4 patients with multifocal motor neuropathy and 1 patient with multifocal acquired ... Read more >>

PLoS ONE (PloS one)
[2020, 15(2):e0228679]

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Treatment-related fluctuations in subacute inflammatory demyelinating polyneuropathy.

Young Gi Min, Yoon-Ho Hong,

Treatment-related fluctuation (TRF), only defined in Guillain-Barre syndrome (GBS), refer to the deterioration of symptoms following treatment-induced improvement, and implies disease activity lasting beyond the effect of immunotherapy. Here, we first propose the concept of TRF in subacute inflammatory demyelinating polyneuropathy (SIDP) with description of a corresponding case. A 27-year-old ... Read more >>

eNeurologicalSci (eNeurologicalSci)
[2020, 18:100224]

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Early Aggressive Immunotherapy Improves Functional Outcome in Chronic Immune Sensory Polyradiculopathy.

Jasmine Shimin Koh, James Wei Min Tung, Genevieve Lynn Yu Tan-Yu, Thirugnanam Umapathi,

Chronic immune sensory polyradiculopathy (CISP) is an uncommon and treatable inflammatory disorder of the proximal sensory nerve roots. Patients typically present with severe sensory ataxia, normal motor examination, unsteady gait, and normal nerve conduction studies (NCS). We describe an elderly man who presented with a two-week history of progressive numbness ... Read more >>

Case Rep Neurol Med (Case reports in neurological medicine)
[2020, 2020:6595086]

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Electrophysiological Responsiveness to Long-Term Therapy in Chronic Inflammatory Demyelinating Polyneuropathy: Case Report.

Payam Khomand, Hans Katzberg, Mylan Ngo, Vera Bril,

Electrophysiological studies are essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP), but the utility of nerve conduction studies in monitoring outcomes in individual CIDP patients is controversial. Electrophysiological improvements after short-term treatment have been described in large cohorts of CIDP patients, but the magnitude of the changes is ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):40-44]

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Different distribution of demyelination in chronic inflammatory demyelinating polyneuropathy subtypes.

Kazumoto Shibuya, Atsuko Tsuneyama, Sonoko Misawa, Yukari Sekiguchi, Minako Beppu, Tomoki Suichi, Yo-Ichi Suzuki, Keigo Nakamura, Hiroki Kano, Satoshi Kuwabara,

In demyelinating polyneuropathies, distribution patterns of demyelination reflect underlying pathogenesis. Median and ulnar nerve conduction studies were reviewed in 85 typical chronic inflammatory demyelinating polyneuropathy (CIDP) patients and 29 multifocal acquired demyelinating sensory and motor neuropathy (MADSAM). Distal latencies were prolonged in typical CIDP and near normal in MADSAM. Abnormal ... Read more >>

J. Neuroimmunol. (Journal of neuroimmunology)
[2020, 341:577170]

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Diagnosis and Treatment of Hereditary Transthyretin Amyloidosis (hATTR) Polyneuropathy: Current Perspectives on Improving Patient Care.

Marco Luigetti, Angela Romano, Andrea Di Paolantonio, Giulia Bisogni, Mario Sabatelli,

Hereditary transthyretin amyloidosis (hATTR) with polyneuropathy (formerly known as Familial Amyloid Polyneuropathy) is a rare disease due to mutations in the gene encoding transthyretin (TTR) and characterized by multisystem extracellular deposition of amyloid, leading to dysfunction of different organs and tissues. hATTR amyloidosis represents a diagnostic challenge for neurologists considering ... Read more >>

Ther Clin Risk Manag (Therapeutics and clinical risk management)
[2020, 16:109-123]

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Low interrater reliability of brachial plexus MRI in chronic inflammatory neuropathies.

Marieke H J van Rosmalen, H Stephan Goedee, Anouk van der Gijp, Theo D Witkamp, Martijn Froeling, Jeroen Hendrikse, W Ludo van der Pol,

INTRODUCTION:Magnetic resonance imaging of the brachial plexus shows nerve thickening in approximately half of the patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). The reliability of qualitative evaluation of brachial plexus MRI has not been studied previously. METHODS:We performed an interrater study in a retrospective cohort ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, :]

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Sporadic hereditary neuropathies misdiagnosed as chronic inflammatory demyelinating polyradiculoneuropathy: Pitfalls and red flags.

Marta Campagnolo, Federica Taioli, Mario Cacciavillani, Marta Ruiz, Marco Luigetti, Alessandro Salvalaggio, Francesca Castellani, Silvia Testi, Moreno Ferrarini, Tiziana Cavallaro, Roberto Gasparotti, Gian Maria Fabrizi, Chiara Briani,

Hereditary neuropathies may be misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). A correct diagnosis is crucial for avoiding unnecessary therapies and access genetic counseling. We report on nine patients (seven men, mean age 49.2 ± 16.1) diagnosed with and treated as CIDP, in whom mutations or variants of unknown significance (VUS) in ... Read more >>

J. Peripher. Nerv. Syst. (Journal of the peripheral nervous system : JPNS)
[2020, 25(1):19-26]

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Boundaries of chronic inflammatory demyelinating polyradiculoneuropathy.

Peter Y K Van den Bergh, Pieter A van Doorn, Bart C Jacobs, Luis Querol, Carina Bunschoten, David R Cornblath,

J. Peripher. Nerv. Syst. (Journal of the peripheral nervous system : JPNS)
[2020, 25(1):4-8]

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Rituximab in refractory chronic inflammatory demyelinating polyneuropathy.

Suraj A Muley, Bill Jacobsen, Gareth Parry, Uzma Usman, Erik Ortega, David Walk, Jeff Allen, Mamatha Pasnoor, Matthew Varon, Mazen M Dimachkie,

INTRODUCTION:Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder in which early effective treatment is important to minimize disability from axonal degeneration. It has been suggested that some patients with CIDP may benefit from rituximab therapy, but there is no definitive evidence for this. METHODS:Baseline and post-rituximab-therapy neuromuscular Medical Research Council ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, :]

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Dose Adjustment of Subcutaneous IgG in Chronic Inflammatory Demyelinating Polyneuropathy.

Alanood Alsolaihim, Steven K. Baker,

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that is characterized by a slowly progressive sensory and motor involvement lasting at least 2 months. We present a CIDP patient on subcutaneous Ig (SCIg). Upon fine-tuning his dose from 24 to 28 g/week, this showed a dramatic improvement in both ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):73-77]

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MR neurography of lumbosacral nerve roots: Diagnostic value in chronic inflammatory demyelinating polyradiculoneuropathy and correlation with electrophysiological parameters.

Fei Wu, Weiwei Wang, Yanyin Zhao, Bingyou Liu, Yin Wang, Yang Yang, Yan Ren, Hanqiu Liu,

PURPOSE:MR neurography(MRN) is an advanced imaging technique to visualize peripheral nerves. Our aim was to determine the value of morphological features of lumbosacral nerve roots on MRN in diagnosing chronic inflammatory demyelinating polyradiculoneuropathy(CIDP) and analyze their correlations with electrophysiological parameters. METHODS:MRN of lumbosacral plexus was performed in 21 CIDP patients ... Read more >>

Eur J Radiol (European journal of radiology)
[2020, 124:108816]

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Dose Adjustment of Subcutaneous IgG in Chronic Inflammatory Demyelinating Polyneuropathy.

Alanood Alsolaihim, Steven K Baker,

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy that is characterized by a slowly progressive sensory and motor involvement lasting at least 2 months. We present a CIDP patient on subcutaneous Ig (SCIg). Upon fine-tuning his dose from 24 to 28 g/week, this showed a dramatic improvement in both ... Read more >>

Case Rep Neurol (Case reports in neurology)
[2020, 12(1):73-77]

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