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Spinal Nerve Root Hypertrophy as Etiology of Back Pain in CIDP (P1.072)
CONCLUSIONS: While the MRI report suggested the possibility of neurofibromata as the etiology of enlarged roots, the patient’s back pain resolved with plasmapheresis. His severely enlarged nerve roots were thus contributed to his onion bulb formation from his CIDP.Disclosure: Dr. Shaefer has nothing to disclose. Dr. Alsharabati has nothing to disclose. Dr. Claussen has nothing to disclose. Dr. Oh has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Shaefer, A., Alsharabati, M., Claussen, G., Oh, S. Tags: Neuromuscular Disease: Pathogenesis and Pathology Source Type: research

Pulsatile TH2 Shift Of Recovering TCR-V{beta} Expanded T-Helper Effector Cells After Initiation Of IVIG Treatment in CIDP (P1.074)
Conclusions: As IL-4 serum levels are found to be markedly high only in short intervals (2 weeks) after IVIg-administration, correlating with patients’ clinical improvement, and then decrease until the next, therapeutical benefits might be due to a pulsatile TH2 induction. This TH2 shift might lead to a short-term restitution of serum milieu and cellular immunehomeostasis, that, in contrast, cannot overcome pathological autoimmunity, necessitating maintenance therapy, as seen in clinical practice. To our knowledge, this TC subset characterization is the first to contribute to functional relevance of TCR repertoire re...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Russwurm, M., Putz, M., Hoft, T., Schlegel, K., Happel, M., Eienbroker, C., Seipelt, M., Tackenberg, B. Tags: Neuromuscular Disease: Pathogenesis and Pathology Source Type: research

Autologous haematopoietic stem cell transplantation: a viable treatment option for CIDP
Conclusions Our results though hampered by the limited number of patients and the lack of a control group suggest AHSCT to be efficacious in therapy-refractory CIDP, with a manageable complication profile. Confirmation of these results is necessary through randomised controlled trials.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 6, 2014 Category: Neurosurgery Authors: Press, R., Askmark, H., Svenningsson, A., Andersen, O., Axelson, H. W., Stromberg, U., Wahlin, A., Isaksson, C., Johansson, J.-E. J., Hagglund, H. Tags: Immunology (including allergy), Neuromuscular disease, Peripheral nerve disease Neuro-inflammation Source Type: research

Genetics of Guillain‐Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): current knowledge and future directions
Abstract Guillain‐Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are thought to be autoimmune diseases. There have been many attempts to find an HLA association with GBS and CIDP with little success. There have been studies of other plausible genes in GBS and CIDP and the role of these genes in GBS and CIDP and the data from these genetic studies is reviewed. Some of the genes that have been studied are immune related and some others have nervous system effects. The studies are limited by small numbers. Some of the genes show association with disease severity rather than diseas...
Source: Journal of the Peripheral Nervous System - March 1, 2014 Category: Neurology Authors: Stefan Blum, Pamela A. McCombe Tags: Review Source Type: research

Comparison of 2‐limb versus 3‐limb electrodiagnostic studies in the evaluation of CIDP
Conclusion: Three‐limb testing may increase diagnostic sensitivity of definite CIDP, especially in patients with atypical phenotypes. Larger prospective studies are needed to better assess the benefit of performing 3‐limb EDX studies. © 2014 Wiley Periodicals, Inc.
Source: Muscle and Nerve - August 1, 2014 Category: Internal Medicine Authors: Mary L. Vo, Aneliya Hanineva, Russell L. Chin, Bridget T. Carey, Norman Latov, Jennifer A. Langsdorf Tags: Research Article Source Type: research

IVIG regulates BAFF expression in patients with chronic inflammatory demyelinating polyneuropathy (CIDP)
Abstract: Recent studies indicate that the cytokine B-cell activating factor (BAFF) is involved in the pathogenesis of chronic inflammatory demyelinating polyneuropathy (CIDP). Intravenous immunoglobulin (IVIg) is standard treatment for CIDP and is known to rapidly modulate increased serum levels of pro-inflammatory cytokines. We evaluated the expression profile of BAFF and its corresponding BAFF-receptor in samples from CIDP patients, focusing on rapid changes before and after IVIg treatment. In CIDP patients BAFF serum concentrations were elevated compared to controls. Treatment with high-dose IVIg restored those elevate...
Source: Journal of Neuroimmunology - July 7, 2014 Category: Allergy & Immunology Authors: Christian Ritter, Dominik Förster, Philipp Albrecht, Hans-Peter Hartung, Bernd C. Kieseier, Helmar C. Lehmann Tags: Short Communications Source Type: research

Thrombocytosis distinguishes POEMS syndrome from CIDP
CONCLUSIONS: Thrombocytosis is a helpful indicator to prompt clinicians to consider the diagnosis of POEMS syndrome in patients who are thought to have CIDP, and it is an important reminder of the increased risk of thrombotic events in POEMS syndrome. This article is protected by copyright. All rights reserved.
Source: Muscle and Nerve - July 14, 2015 Category: Internal Medicine Authors: Elie Naddaf, Angela Dispenzieri, Jay Mandrekar, Michelle L. Mauermann Tags: Short Report Source Type: research

Frequent laboratory abnormalities in CIDP patients
Conclusion: Laboratory test abnormalities were found in most CIDP patients. The most common were paraproteinemia, higher than expected frequency of diabetes, and unexpected CK elevation. Additional abnormalities included anemia, high urate levels, and common biomarkers for vasculitic neuropathies. This article is protected by copyright. All rights reserved.
Source: Muscle and Nerve - November 17, 2015 Category: Internal Medicine Authors: Alon Abraham, Hana Albulaihe, Majed Alabdali, Mohammad Qrimli, Ari Breiner, Carolina Barnett, Hans D. Katzberg, Leif E. Lovblom, Bruce A. Perkins, Vera Bril Tags: Research Article Source Type: research

Evaluation of CIDP Diagnostic Criteria and Treatment Responses (P3.141)
CONCLUSION: While CIDP criteria can similarly predict IVIG treatment response, the EFNS 2010 criteria are more sensitive for the diagnosis of CIDP.Disclosure: Dr. Roach has nothing to disclose. Dr. Pasnoor has nothing to disclose. Dr. Statland has nothing to disclose. Dr. Dimachkie has received personal compensation for activities with Pfizer, Depomed, Merck, CSL-Behring, Nufactor, Biomarin, Baxter and Catalyst as a consultant, speaker, and advisory board member. Dr. Barohn has received personal compensation for activities with Grifols & Genzyme, Speakers Bureau, NuFactor as speaker and consultant. Dr. Veerapaneni has ...
Source: Neurology - April 3, 2016 Category: Neurology Authors: Roach, C., Pasnoor, M., Statland, J., Dimachkie, M., Barohn, R., Veerapaneni, K., Jawdat, O., Dick, A. Tags: Acquired Peripheral Neuropathies: Clinical and Pathological Aspects Source Type: research

Poems masquerading as treatment refractory cidp: a case series
Conclusions POEMS is rare difficult to diagnose but treatable condition that can mimic CIDP. Serum VEGF level is an important criterion to consider in diagnosis. Neurological recovery is fairly rapid and, in our experience, more promising than that quoted in the haematalogical literature.
Source: Journal of Neurology, Neurosurgery and Psychiatry - May 8, 2017 Category: Neurosurgery Authors: Nham, B., Brown, C., Khoo, L., Gibson, J., Spies, J., Jankelowitz, S. Tags: Abstracts Source Type: research

Pediatric CIDP: Clinical Features and Response to Treatment
Conclusions: Pulse oral corticosteroid therapy holds promise as an alternative treatment to IVIG in pediatric CIDP. Future multicenter studies are warranted to determine the comparative efficacy and safety of weekly pulse oral corticosteroids versus IVIg in pediatric CIDP.
Source: Journal of Clinical Neuromuscular Disease - November 21, 2017 Category: Neurology Tags: Original Article Source Type: research

Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype
Conclusions Our findings confirm previous data on the tight clinico-serological correlation between antibodies to nodal/paranodal proteins and CIDP. Despite the low prevalence, testing for their presence and isotype could ultimately be part of the diagnostic workup in suspected inflammatory demyelinating neuropathy to improve diagnostic accuracy and guide treatment. Classification of evidence This study provides Class III evidence that antibodies to nodal/paranodal proteins identify patients with CIDP (sensitivity 6%, specificity 100%).
Source: Neurology Neuroimmunology and Neuroinflammation - November 20, 2019 Category: Neurology Authors: Cortese, A., Lombardi, R., Briani, C., Callegari, I., Benedetti, L., Manganelli, F., Luigetti, M., Ferrari, S., Clerici, A. M., Marfia, G. A., Rigamonti, A., Carpo, M., Fazio, R., Corbo, M., Mazzeo, A., Giannini, F., Cosentino, G., Zardini, E., Curro, R., Tags: Autoimmune diseases, All Neuromuscular Disease, Peripheral neuropathy, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research

The Misdiagnosis of CIDP: A Review
AbstractThere 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 on to receive costly and potentially harmful treatments for a disease that they do not have, while at the same time missing an opportunity to treat their true ailment. The cost of misdiagnosis on patients and society is not trivial. Many factors contribute to misdiagnosis. Particular po...
Source: Neurology and Therapy - March 25, 2020 Category: Neurology Source Type: research

NK cell markers predict the efficacy of IV immunoglobulins in CIDP
Conclusions The correlation between the altered NK cell population and treatment efficiency suggests a crucial role for NK cells in the still speculative mode of action of IVIg treatment. Analyzing NK cell subsets after 24 hours of treatment initiation appeared as a predictive marker for IVIg responsiveness. Further studies are warranted investigating the potential of NK cell status as a routine parameter in patients with CIDP before IVIg therapy. Classification of evidence This study provides Class I evidence that NK cell markers predict clinical response to IVIg in patients with CIDP.
Source: Neurology Neuroimmunology and Neuroinflammation - October 1, 2020 Category: Neurology Authors: Mausberg, A. K., Heininger, M. K., Meyer Zu Horste, G., Cordes, S., Fleischer, M., Szepanowski, F., Kleinschnitz, C., Hartung, H.-P., Kieseier, B. C., Stettner, M. Tags: Autoimmune diseases, Chronic inflammatory demyelinating polyneuropathy Article Source Type: research