2,816 results match your criteria: "Chronic Inflammatory Demyelinating Polyradiculoneuropathy"

Introduction/aims: Somatosensory evoked potentials (SSEPs) are described as a supportive tool to diagnose chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); however, there is a lack of studies determining the effectiveness of SSEPs in monitoring the clinical course of individuals with this condition. The aims of this study are to evaluate the utility of SSEPs in monitoring patients with CIDP and to assess their association with clinical outcomes following immunomodulatory therapy.

Methods: This was a single-center retrospective observational study that included patients who met European Federation of Neurological Societies and Peripheral Nerve Society criteria for CIDP between 2018 and 2023.

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Autologous hematopoietic stem cell transplantation (HSCT) is associated with 5-year treatment-free remissions in approximately 80% of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed or were dependent on intravenous immunoglobulin and or plasmapheresis. Autologous HSCT was associated with significant improvement in strength, independent ambulation, quality of life, nerve conduction velocity, and compound muscle action potential amplitude. The results of HSCT are dependent on proper patient selection, i.

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Patients affected by chronic inflammatory demyelinating polyradiculoneuropathy require close follow up due to the neuronal demyelination along with axonal degeneration associated with the disease process, giving the opportunity to the medical team of adequating therapeutics and other medical interventions, according to the evolution of the symptoms, to prevent irreversible axonal degeneration.

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Immunoglobulin use in neurology: a practical approach.

Pract Neurol

August 2024

UCL Queen Square Institute of Neurology, Faculty of Brain Sciences, University College London, London, UK

Article Synopsis
  • Human immunoglobulin (IVIg) is used to treat various immune-mediated neurological disorders, focusing on its effects in both acute inflammatory diseases and chronic disease management.
  • The review examines how IVIg works and its evidence in treating conditions like Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in the UK and Australia.
  • It also discusses the pros and cons of IVIg, emphasizes practical aspects like informed consent and risk management, and addresses the careful use of this costly blood product.
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Proteomics analysis of immune response-related proteins in Guillain-Barré Syndrome (GBS) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).

J Neuroimmunol

September 2024

Department of Neurology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, China; Centre for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

The objective is to characterize differentially expressed proteins (DEPs) in Guillain-Barré Syndrome (GBS) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) through high-throughput analysis. Sera from 11 healthy controls (HCs), 21 GBS and 19 CIDP patients were subjected to Olink Proteomics Analysis. In the comparison between CIDP and GBS groups, up-regulation of ITM2A and down-regulation of NTF4 were observed.

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Article Synopsis
  • Autoimmune neuropathies are disorders where the immune system attacks peripheral nerves, with Guillain-Barré syndrome (GBS) as a common acute form, and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) as a chronic form.
  • Recent discoveries of specific antibodies associated with nerve damage have changed diagnostic and treatment approaches for GBS and CIDP, making testing for certain antibodies more common.
  • New subtypes of autoimmune neuropathies, defined by antibodies affecting the nodes of Ranvier, have emerged, allowing for better patient classification and targeted therapies, such as anti-CD20 treatment.
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Article Synopsis
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy characterized by prolonged clinical progression, with a subset known as acute-onset CIDP (A-CIDP) presenting rapid weakness similar to Guillain-Barré syndrome (GBS).
  • A case study of a 56-year-old woman highlighted the complexities of diagnosing A-CIDP, particularly when she exhibited anti-GM3 and anti-sulfatides antibodies, which are rarely seen in A-CIDP.
  • The findings emphasize the importance of identifying specific antibodies in understanding CIDP's pathogenesis and the need for careful monitoring of symptoms during treatment to prevent further complications.
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Background: Severe post-vaccination neurological complications are rare. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated polyneuropathy affecting the peripheral nerve roots, which is not well described as a post-vaccination side effect. Here, we present a rare complication of vaccination against SARS-CoV-2, reaching a diagnosis of CIDP.

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Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an inflammatory disease affecting the peripheral nerves and the most frequent autoimmune polyneuropathy. Given the lack of established biomarkers or risk factors for the development of CIDP and patients' treatment response, this research effort seeks to identify potential clinical factors that may influence disease progression and overall treatment efficacy.

Methods: In this multicenter, retrospective analysis, we have screened 197 CIDP patients who presented to the University Hospitals in Düsseldorf, Berlin, Cologne, Essen, Magdeburg and Munich between 2018 and 2022.

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Article Synopsis
  • The study explores the link between proteinuria and autoimmune nodopathies in patients with nephrotic syndrome, suggesting proteinuria may act as a biomarker.
  • Researchers analyzed urinalysis results and autoantibodies in 69 chronic inflammatory demyelinating polyneuropathy (CIDP) patients, finding that those with specific anti-CNTN1 and anti-NF155 antibodies often exhibited proteinuria.
  • The findings indicate that patients with nephrotic syndrome should be screened for these antibodies since proteinuria can help differentiate autoimmune nodopathies from CIDP.
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Background And Purpose: The amplitude, timing, and determinants of improvement with available treatments are uncertain in chronic inflammatory demyelinating polyneuropathy (CIDP). Our primary objective was to quantify categorized outcomes with routine care.

Methods: We retrospectively studied treatment response within 36 months from initiation in 112 consecutive subjects with CIDP.

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Distinct Clinical Courses and Shortened Lifespans in Childhood-Onset DNA Polymerase Gamma Deficiency.

Neurol Genet

August 2024

From the Université Paris Cité (A.R., M.S.), Institut Imagine, Génétique des maladies mitochondriales, INSERM UMR 1163; Centre de Référence des Maladies Mitochondriales (A.R., P.G., G.B., Z.A., C.-M.B., M.B., M.-T.A.-W., P.D.L., I.D., E.G., E.J., A.D.S.-M., N.B., A.M., M.S.), AP-HP, Hôpital Necker-Enfants Malades, Paris; Service de Biochimie (P.G.), AP-HP, Hôpital Bicêtre, Le Kremlin-Bicêtre; Service de médecine génomique des maladies rares (G.B., Z.A.), AP-HP, Hôpital Necker-Enfants Malades, Paris; Service de Génétique (M.B., D.B.), Centre Hospitalier Universitaire; Service de génétique clinique (L.D.), Centre de Compétences Maladies Héréditaires du Métabolisme, CHU de Rennes; Unité de Gastroentérologie (N.L., P.B.), Hépatologie, Nutrition, Diabétologie et Maladies Héréditaires du Métabolisme, Hôpital des Enfants, CHU de Toulouse; Service de Neuropédiatrie (M.-T.A.-W., A.D.S.-M.), CHU de Strasbourg; Service de Neurométabolisme pédiatrique (B.C.), CHU Timone, Marseille; Service et Centre de référence des maladies héréditaires du métabolisme (P.D.L., M.S.); Service de Neurophysiologie pédiatrique (I.D., C.G.), AP-HP, Hôpital Necker-Enfants Malades, Paris; Service de Génétique (A.G.), CHU de Rouen; Pediatric Hepatology and Pediatric Liver Transplant Unit (E.G., E.J.), AP-HP, CHU Bicêtre, Le Kremlin-Bicêtre; Laboratoire de Biochimie et Biologie Moléculaire (P.A.-B.), CHU d'Angers; Pédiatrie générale et maladies infectieuses (V.A.), AP-HP, Hôpital Necker-Enfants Malades, Paris; Service de médecine infantile (C.B.), CHU de Nancy; Service de Réanimation pédiatrique et néonatale (P.D.), AP-HP, Hôpital Bicêtre, Le Kremlin-Bicêtre; Centre de référence des maladies héréditaires du métabolisme (A.F.), Hospices civils de Lyon, CHU de Lyon; Service de génétique médicale (B.I.), CHU de Nantes; Service de Neurologie Pédiatrique (M.J.), AP-HP, Hôpital Robert Debré, Paris; Génétique Clinique et Oncogénétique (G.J.), CHU Amiens-Picardie; Service de Neurologie pédiatrie (H.M.), AP-HP, Hôpital Bicêtre, Le Kremlin-Bicêtre; Centre de référence des Maladies Héréditaires du métabolisme (K.M.), Hôpital Jeanne de Flandre, Lille; Service de Génétique Clinique (S.S.O., L.P.), CRMR anomalies du développement CLAD-Ouest, Rennes; Service de neurologie pédiatrique (C.R.-J.), Hospices civils de Lyon, CHU de Lyon; Imagerie pédiatrique (C.-J.R., N.B.), AP-HP, Hôpital Necker-Enfants Malades, Université Paris Cité; and Université Paris Cité (A.M.), Imagine Institute, INSERM UMR 1163, Paris, France.

Article Synopsis
  • - POLG deficiency is the most common cause of nuclear-encoded mitochondrial disorders, leading to a range of overlapping symptoms from infancy to adulthood, as seen in a study of 40 children with biallelic pathogenic variants.
  • - The study identified three main clinical patterns (neurologic, hepatic, gastrointestinal), with 24 patients requiring urgent care mainly due to severe neurologic issues like seizures and epilepsy.
  • - Most children with hepatic symptoms had the earliest onset and shortest survival rates, while those with gastrointestinal issues had milder symptoms and lived longer; overall, the prognosis was poor, with many fatalities occurring by age 10.
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Introduction/aims: Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with "supportive" evidence of CIDP on cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), ultrasound (US), or nerve biopsy but not on NCS, often receive immunomodulating therapy. We evaluated the treatment response of patients with clinical and supportive features of CIDP lacking NCS evidence of demyelination.

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Article Synopsis
  • Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a complex condition with unknown causes, and some cases have been linked to the COVID-19 mRNA vaccines, though no links to other vaccines have been reported.
  • A case study describes a 48-year-old woman who developed symptoms of CIDP shortly after receiving her second dose of the Moderna vaccine, following initial vaccination with the Pfizer-BioNTech vaccine.
  • Medical evaluations revealed significant neurological damage consistent with demyelination, leading to treatment with steroids and intravenous immunoglobulin therapy, suggesting a possible connection between vaccine variations and the onset of CIDP.
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Background: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presents significant treatment challenges due to its chronic nature, varied clinical presentations, and rarity. Subcutaneous immunoglobulin (SCIG) has emerged as a maintenance therapy, offering potential advantages in administration and patient experience over the previously recognized intravenous immunoglobulin (IVIG).

Methods: We included all clinical studies involving CIDP patients treated with SCIG from eleven databases up to March 2024.

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Article Synopsis
  • * The patient experienced symptoms like progressive weakness, tingling, and numbness, which led to nerve conduction studies that indicated demyelination.
  • * Following skin tests that confirmed leprosy, she received multidrug therapy and showed significant improvement, underscoring the need to consider leprosy in similar cases, especially in areas where it is more common.
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Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune disease of the peripheral nervous system. It is often difficult to diagnose, but severaly therapeutic options are nowadays available to reduce neurological deficits and to improve the disease course. This article exemplifies the management of CIDP by a typical case study.

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Article Synopsis
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-related disease affecting peripheral nerves, which the authors link to pulmonary nocardiosis in a specific patient case.
  • A 65-year-old man with CIDP experienced disease progression despite receiving treatment, and he ultimately developed pulmonary nocardiosis, which led to further health complications.
  • Despite attempts to manage both conditions with various therapies, including antibiotics and intravenous immunoglobulin, the patient’s condition deteriorated, and he ultimately died, highlighting the challenges in treating patients with coexisting rare diseases.
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Purpose Of Review: There is no diagnostic biomarker that can reliably detect Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnosis relies upon integrating key clinical characteristics and relevant supportive data. Consequently, misdiagnosis and delayed diagnosis are common.

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An immuno-DOT diagnostic assay for autoimmune nodopathy.

Clin Chem Lab Med

June 2024

131795 Institut de Génomique Fonctionnelle, Université de Montpellier, CNRS, INSERM , Montpellier, France.

Objectives: Autoimmune nodopathy (AN) is a life-threatening peripheral neuropathy mediated by four autoantibodies targeting axoglial cell adhesion molecules at the nodes of Ranvier: Neurofascin-155 (Nfasc155), PanNeurofascin (PanNfasc), Contactin-1 (CNTN1), and Contactin-associated protein 1 (CASPR1). Antibody detection is a strong biomarker for AN diagnosis and treatment monitoring. The aim of this study was to develop an immuno-dot assay (immuno-DOT) compatible with routine implementation in medical laboratories.

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, immune-mediated disorder in which an aberrant immune response causes demyelination and axonal damage of the peripheral nerves. Genetic contribution to CIDP is unclear and no genome-wide association study (GWAS) has been reported so far. In this study, we aimed to identify CIDP-related risk loci, genes, and pathways.

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Animal models of immune-mediated demyelinating polyneuropathies.

Autoimmunity

December 2024

Neuromuscular Immunopathology Research Laboratory, Division of Neuromuscular Disease, Department of Neurology, University of Alabama at Birmingham, Birmingham, AL, USA.

Immune-mediated demyelinating polyneuropathies (IMDPs) are rare disorders in which dysregulated adaptive immune responses cause peripheral nerve demyelinating inflammation and axonal injury in susceptible individuals. Despite significant advances in understanding IMDP pathogenesis guided by patient data and representative mammalian models, specific therapies are lacking. Significant knowledge gaps in IMDP pathogenesis still exist, e.

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