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Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years.
Authors: Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Atsuta N, Nakamura T, Hirayama M, Ogata H, Yamasaki R, Kira JI, Katsuno M, Sobue G Abstract We herein report a woman with chronic inflammatory demyelinating polyneuropathy (CIDP) in whom positivity for anti-neurofascin 155 antibodies was revealed 23 years after the onset of neuropathy. The patient initially reported numbness in the face at 50 years of age and subsequently manifested features compatible to typical CIDP. Steroid administration initiated at 54 years of age ameliorated her neuropathic symptoms. Although the nerve conduction indices at 59 year...
Source: Internal Medicine - March 3, 2018 Category: Internal Medicine Tags: Intern Med Source Type: research

FDA Approves Hizentra(R) (Immune Globulin Subcutaneous Human 20% Liquid) for the Treatment of Patients With Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
First and only subcutaneous immunoglobulin (SCIg) approved for the treatment of CIDP based on the largest controlled clinical study in CIDP KING OF PRUSSIA, Pa., March 16, 2018 -- (Healthcare Sales & Marketing Network) -- Global biotherapeutics leader ... Biopharmaceuticals, FDA CSL Behring, Hizentra, Immune Globulin, CIDP
Source: HSMN NewsFeed - March 16, 2018 Category: Pharmaceuticals Source Type: news

S6-4. CIDP syndrome with anti-NF155 and -CNTN1 IgG4 antibodies
In recent years, the specific autoantibodies for some CIDP patients groups have been found. Neurofascin155 (NF155) and contactin-1 (CNTN1), which are adhesion molecules at paranodal axo-glial junction, are the novel target antigens. The patients with anti-NF155 and-CNTN1 IgG4 antibodies might form specific clinical subgroups. In 2013, Querol et al. found anti-CNTN1 IgG4 antibodies in 3 of 46 patients with CIDP. Subsequent studies showed the positivity rate of anti-CNTN1 IgG4 antibodies in CIDP as 2.4 –7.5%.
Source: Clinical Neurophysiology - March 30, 2018 Category: Neuroscience Authors: Norito Kokubun Source Type: research

EP.116Genetic neuropathies presenting with CIDP-like features in childhood – 3 novel cases and review of the literature
Inherited neuropathies are amongst the most common neuromuscular disorders. Particularly in childhood, the distinction from much rarer chronic inflammatory demyelinating polyneuropathy (CIDP) may be challenging, considering that genetic neuropathies may show secondary inflammatory features, and that subacute CIDP with gradual onset may closely mimic the course of inherited disorders. The overlap between genetic neuropathies and CIDP is increasingly recognized in adults but has been rarely reported in childhood.
Source: Neuromuscular Disorders - September 30, 2019 Category: Neurology Authors: M. Fern ández-García, M. Kinali, A. Clarke, P. Fallon, V. Gowda, E. Wraige, H. Jungbluth Source Type: research

Immunoglobulin administration for the treatment of CIDP: IVIG or SCIG?
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired neurological disorder characterized clinically by weakness and impaired sensory function evolving over 2  months or more, loss or significant decrease in deep tendon reflexes, and by electrophysiological evidence of peripheral nerve demyelination. Expeditious diagnosis and treatment of CIDP early in the disease course is critical such that irreversible disability can be avoided. Intravenous immunoglo bulin (IVIG) is one first-line and maintenance therapy option for CIDP.
Source: Journal of the Neurological Sciences - November 8, 2019 Category: Neurology Authors: Jeffrey A. Allen, Deborah F. Gelinas, Miriam Freimer, M. Chris Runken, Gil I. Wolfe Source Type: research

Pronounced proximally accentuated changes in high-voltage stimulation and nerve ultrasound in long-standing therapy-naive CIDP variants.
Abstract The differential diagnosis of chronic demyelinating polyneuropathy particularly includes inflammatory (CIDP) and hereditary causes. Using the example of a 63-year-old patient, we show the diagnostic procedure with conventional electrophysiological diagnostics and additionally by the use of proximal nerve conduction studies with high-voltage stimulation (HVS) and the direct morphological examination by high-resolution nerve ultrasound. In the present case, the focal accentuation of the changes in HVS and the equally pronounced focal thickening of the most affected ulnar nerve in ultrasound confirmed the d...
Source: Der Nervenarzt - October 28, 2020 Category: Neurology Authors: Gardill K, Schüpbach M Tags: Nervenarzt Source Type: research

Clinical management of chronic inflammatory demyelinating polyneuropathy (CIDP) in Europe and India: An exploratory study
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disorder causing inflammatory demyelination of peripheral nerves and consecutive disability. Diagnostic criteria and treatments are well established, but it is unknown how clinical practice may differ in different geographical regions.In this multicentre study, clinical management of CIDP was compared in 44 patients from Germany, India and Norway regarding diagnostic and therapeutic procedures.All centres used EFNS/PNS diagnostic criteria for CIDP but diagnostic workup varied regarding screening for infectious diseases, genetic testing and nerve biopsy.
Source: Journal of the Neurological Sciences - May 20, 2021 Category: Neurology Authors: Martin K.R. Sva čina, Man Mohan Mehndiratta, Christian A. Vedeler, Yogesh Sharma, Ilja Bobylev, Alina Sprenger, Gina Remke, Hauke Wüstenberg, Ines Klein, Abhijeet Joshi, Helmar C. Lehmann Tags: Clinical short communication Source Type: research

CIDP and hemopathies, an underestimated association
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated and treatable disease that may be associated with various systemic conditions. Our objective is to describe the clinical, electrophysiological and pathological data of a series of patients with both CIDP and hemopathy. In this retrospective study, we analyzed 21 patients with CIDP and various hemopathies (malignant or not), consecutively observed for almost five years. In this particular context (with a risk of neurological complications of the hemopathy), a nerve biopsy was taken from each patient (after written consent).
Source: Journal of the Neurological Sciences - August 25, 2021 Category: Neurology Authors: Nathalie Deschamps, St éphane Mathis, Mathilde Duchesne, Karima Ghorab, Gaël Gallouedec, Laurence Richard, Jean-Marc Boulesteix, Philippe Corcia, Laurent Magy, Jean-Michel Vallat Source Type: research

Clinical outcome of CIDP one  year after start of treatment: a prospective cohort study
ConclusionsImprovement was seen in most patients. One year after the start of treatment, more than half of the patients were untreated and around one-third in remission. Residual symptoms and deficits were common regardless of treatment.
Source: Journal of Neurology - January 23, 2022 Category: Neurology Source Type: research

Autoantibody profile in a Malaysian cohort of chronic inflammatory demyelinating polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated acquired demyelinating neuropathy that is characterized by proximal and distal limb weakness, areflexia and sensory involvement [1]. In typical CIDP, clinical symptoms progress for more than eight weeks and follow a course of disease progression that is either progressive or relapsing-remitting. However, there are atypical presentations of CIDP including acute-onset CIDP [2], distal predominant subtype [distal acquired demyelinating symmetric neuropathy (DADS)], multifocal subtype [multifocal acquired demyelinating sensory and motor neuropathy (...
Source: Neuromuscular Disorders - January 22, 2022 Category: Neurology Authors: Cheng-Yin Tan, Khean-Jin Goh, Ai-Wen Oh, J érôme Devaux, Nortina Shahrizaila Tags: Research paper Source Type: research

Intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): mechanisms of action and clinical and genetic considerations
Expert Rev Neurother. 2023 Jan 16. doi: 10.1080/14737175.2022.2169134. Online ahead of print.ABSTRACTINTRODUCTION: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune peripheral nerve disorder that is characterized by subacute onset, progressive or relapsing weakness, and sensory deficits. Proven treatments include intravenous immunoglobulin (IVIg), Corticosteroids, and plasma exchange. This review focuses on the mechanisms of action, pharmacodynamics, genetic variations, and disease characteristics that can affect the efficacy of IVIg.AREAS COVERED: The proposed mechanisms of action of IVIg t...
Source: Expert Review of Neurotherapeutics - January 16, 2023 Category: Neurology Authors: Marinos C Dalakas Norman Latov Krista Kuitwaard Source Type: research