View clinical trials related to Polyradiculoneuropathy.
Filter by:CIDP, a rare disorder affecting young adults, causes gradual weakness of the limbs, areflexia and impaired sensory function. New CIDP phenotypes without antibodies but with modified cell profiles have been described. Treatments include corticotherapy, IVIg and plasmapheresis but the latter's action mechanisms remain unclear. Plasmapheresis supposedly removes toxic agents like antibodies from plasma but it is uncertain whether it has an immune-modulating effect. Also, the refining mechanisms of the two main plasmapheresis techniques - single plasma exchange and double filtration plasmapheresis (DFPP) - are different and unclear. This study aims to compare the evolution of peripheral lymphocyte profiles in patients with CIDP according to their treatment (single centrifugation plasmapheresis or DFPP) to better grasp the action mechanisms of both techniques.
Primary Objectives: - Part A: Efficacy of SAR445088 across three subpopulations of CIDP patients: standard of care (SOC)-Treated, SOC-Refractory and SOC-Naive - Part B:Long-term safety and tolerability of SAR445088 in CIDP Secondary Objectives: - Part A: - Safety and tolerability of SAR445088 in CIDP - Immunogenicity of SAR445088 - Efficacy of SAR445088 with overlapping SOC (SOC-Treated group) - Part B: - Durability of efficacy during long-term treatment with SAR445088 in CIDP - Long-term immunogenicity of SAR445088 in CIDP
Antibody-mediated idiopathic inflammatory diseases of the nervous system (also known as autoimmune diseases of the nervous system) are autoimmune diseases in which autoimmune cells and immune molecules attack the nervous system as the main pathogenic mechanism. In the immune response, pathogenic antibodies acting on autoantigens of the nervous system are collectively referred to as autoantibodies of the nervous system, and antibody-mediated idiopathic inflammatory diseases of the nervous system can occur in the central nervous system, peripheral nervous system, and neuromuscular junctions , and muscles. In this study, we will recruit three kinds of autoimmune diseases of nervous system including Neuromyelitis Optica Spectrum Disorder (NMOSD), Myasthenia Gravis (MG), Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and Immune-Mediated Necrotizing Myopathy (IMNM). B-cell maturation antigen (BCMA) is expressed on the surface of plasma cells, thus making it an ideal target for targeted therapies. Chimeric antigen receptor (CAR) T cells against BCMA offers another potential therapeutic option to eliminate plasma cells in patients with neurological autoimmune diseases driven by abnormal antibody who still suffer recurrent attacks from conventional treatments. In the current study, the safety and efficacy of a novel CAR-T cell therapy using CT103A cells, are evaluated in patients with relapsed/refractory antibody-mediated idiopathic inflammatory diseases.
Chronic inflammatory demyelinating polyradiculoneuropathy is a diffuse multifocal autoimmune disorder of the peripheral neuron, affecting 1 to 9 in 100,000 people. Its course is difficult to predict, and may be characterized by continuous progression, multiple relapses, or recovery after a few months. treatment. The predominantly motor form with 4 limbs represents the typical form, but the disease can take on other clinical forms (pure sensory impairment, ataxia, etc.). In addition to induction therapy, patients most often require long-term maintenance therapy. First-line therapies, with the same efficacy according to a 2013 Cochrane study, are glucocorticoid therapy, plasma exchanges and intravenous immunoglobulin injections. Glucocorticoids have a grade C recommendation level while a grade A has been assigned to intravenous immunoglobulins and plasma exchange. However, the latter have less tolerance and have a rebound effect which limits their long-term interest. Intravenous immunoglobulins are therefore the preferred treatment today. The effect of intravenous immunoglobulins, delivered as a bolus over a few days, lasts two to six weeks, with the number of people being cured of three to improve a person. A more recent study has also shown their advantage in reducing the relapse rate at 6 months. However, the response to intravenous immunoglobulins fluctuates in different patients and, for any given patient, changes over the course of the disease. The 2010 recommendations therefore recommend an adaptation of the doses and duration of intercourse (0.4 to 1.2 g / kg every 2 to 6 weeks) according to individual monitoring of the response to treatment. In order to embrace the diversity of symptoms of chronic inflammatory demyelinating polyradiculoneuropathy, several scores and scales are usually combined to ensure this follow-up in a cohort. Three clinical data are currently favored: the Inflammatory Rasch-built Overall Disability Scale (I-RODS), the INCAT Overall Neuropathy Limitations Scale (ONLS), the score of the Medical Research Council (MRC). However, none of them assess walking objectively. However, patients with chronic inflammatory demyelinating polyradiculoneuropathy sometimes report significant walking disturbances, which may result from both sensory disturbances or motor disturbances present in varying degrees depending on the patient. The alterations concerned, according to the studies, the walking speed, the temporal pattern of the step, with an impairment of the durations of the different phases (support and oscillation) or the angle and the angular speed of roll at the level of the trunk. Alterations in speed and phase duration of the step improve during treatment with intravenous immunoglobulin cures, with greater sensitivity compared to the ONLS and MRC scales. The power of the propulsive moment at the ankle during the last moments of the stance phase - the push-off - is another promising gait parameter that has made it possible to distinguish diabetic patients with polyneuropathy from those without diabetes. polyneuropathy and the intensity of the deficit is linked to the severity of the attack. Gait speed, as a reflection of the subject's gait performance, and the quality of gait including the timing of gait, trunk rotation movements and push-off, therefore seem to be potential response markers. for monitoring patients treated with intravenous immunoglobulins. InertiaLocoGraphy, quantification of gait by inertial measurement sensors, has proven its value in the evaluation of various pathologies in neurological practice, including chronic inflammatory demyelinating polyradiculoneuropathy. It gives access to the walking speed as well as to various walking quality criteria (vigor of the step, rhythmicity, regularity, symmetry, stability, fluidity, synchronization) including the times of the different walking phases and the rotational movements of the trunk, and a push-off substitute. InertiaLocoGraphie, non-invasive, easy and quick to set up, reflecting the patient's function, therefore potentially provides biomarkers of choice for monitoring the response to intravenous immunoglobulin cures in patients with chronic inflammatory polyradiculoneuropathy demyelinating. Its association with the traditional monitoring tools such as the ONLS score, the I-ROS, and the CRM therefore appears to be of key interest for this monitoring.
CIDP and MMN are part of a group of chronic inflammatory conditions that affect the peripheral nervous system. In CIDP, there is chronic inflammation of the peripheral nerves and nerve roots leading to demyelination. The myelin sheath is vital in the rapid propagation of nerve impulses between the central nervous system and the peripheral sensory receptors and muscles. By definition CIDP must progress over 8 or more weeks and can either have a slowly progressive disease course or a relapsing course with periods of improvement. Patients typically present with a non-length dependent neuropathy that affects motor (i.e. weakness of proximal or distal muscles, fatigue, swallowing difficulty, double vision, breathing difficulties etc) and sensory function. MMN is a similar condition to CIDP. It is an autoimmune demyelinating neuropathy that leads to slowly progressive asymmetrical weakness that worsens over years without treatment. IVIg is a recognised treatment for CIDP and MMN. A standard starting dose of 2 g/kg/course, spread over 2-5 days, has been widely used in both research and clinical practice. Due to the chronic nature of CIDP and MMN, most patients with these conditions require repeated doses to avoid relapse, but the frequency of courses and the total dose of IVIg per course required to achieve a steady state varies between patients. Given the modest risks involved with IVIg and its cost, the lowest possible dose and frequency of administration are preferred. Current strategies to reduce dose and frequency involve assessing clinical response to lower doses, but this is both time consuming and imprecise.
The nodes of Ranvier contain ion channels that enable the rapid propagation of the nerve impulse. Cell adhesion molecules and glycolipids play an important role in the formation of the nodes of Ranvier. Antibodies against glycolipids are detected in half of patients with Guillain-Barré syndrome, an acute inflammatory neuropathy affecting peripheral nerve. The investigators found that antibodies target cell adhesion molecules at nodes of Ranvier in 10% of patients with chronic inflammatory demyelinating neuropathy (CIDP), another disabling neuromuscular disease affecting peripheral nerves. In the majority of patients with GBS or CIDP, the mechanisms responsible for the neuromuscular disorders are unknown. Our goals are to identify novel targets of antibodies in patients, this in order to find novel bio-markers and to better understand the physiopathology of inflammatory neuropathies. This work will help patient diagnosis and treatment orientation.
The purpose of the study is to assess long-term safety and tolerability of weekly doses of rozanolixizumab in subjects with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
Chronic inflammatory demyelinating polyradiculoneuropathy (PIDC) is an acquired dysimmune polyneuropathy whose prevalence is estimated to be between 1 to 4 per 100,000 depending on the study, with an incidence of less than 1 per 100,000 per year. The clinical presentation of PIDC is heterogeneous, characterized by a symmetrical lesion predominating generally on large fibers, the most myelinated, responsible for ataxia and a motor deficit in the foreground. In the typical form, patients describe a proximal and distal motor or sensory deficit associated with isflexia that signifies a peripheral neurogenic syndrome. The physiopathological hypothesis is that of an inflammation responsible for demyelinating nerve fibers, which results in electroneuromyogram (ENMG) by conduction abnormalities and histologically when a neuromuscular biopsy is performed by segmental demyelination. Given the heterogeneity of the clinical presentation, electrical diagnostic criteria are proposed by the European Federation of Neurological Society in order to classify IPDCs into three categories: certain, probable and possible. In the absence of sufficient criteria to make the diagnosis of IPDC, it is also possible to use criteria of support, using a paraclinical report including the presence of an increase in protein (hyperproteinorachie) without cells for cerebrospinal fluid analysis, visualization of radicular inflammation on imaging (MRI of lumbar and / or brachial plexus), proximal peripheral involvement with somatosensory evoked potentials. Therapeutically, a joint management between rehabilitation and the introduction of a background treatment allows the clinical improvement of certain patients. To date, the treatments proposed in first intention are corticosteroids, intravenous immunoglobulins (IVIg) and plasma exchanges. In fact, the efficacy of intravenous immunoglobulins has been widely shown by controlled and randomized therapeutic trials. Efficacy studies of IVIg in the literature are most often based on an assessment of clinical response after 24 weeks, but in clinical practice the response to treatment and continuation of treatment is often evaluated after 3 courses of treatment with the help of a clinical evaluation and the realization of an electroneuromyogram. These are administered in day hospitalization or traditional hospitalization, every four weeks, to patients whose diagnosis of PIDC has been established by electroneuromyogram according to the EFNS criteria. Clinical prognostic factors for good response to IVIG therapy have been described in previous studies, including subacute disease, symmetrical involvement, and absence of amyotrophy. In order to optimize the management of IPDCs, it is important to identify patients who respond to IVIg. Thus, the objective of our study is to look at the electroneuromyogram, the presence of electrical predictors of good response to treatment with IVIg.
To evaluate the efficacy and safety of rituximab (genetical recombination) intravenously administered to CIDP patients with positive or negative IgG4 autoantibody.
The purpose of the study is to evaluate clinical efficacy of rozanolixizumab as a treatment for subjects with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).