Active clinical trials for "Polyradiculoneuropathy"

The objectives of this study are to determine the safety, tolerability and preliminary efficacy of alemtuzumab for infusion for the treatment of CIDP. Eligible subjects will be treated with alemtuzumab at the beginning of the study and then followed for three years. During the three year period, subjects will under go monthly safety evaluations consisting of blood and urine testing, symptom surveys and examination. Detailed neurological testing including nerve conduction testing, Rasch-built Overall Disability Scale (CIDP/RODS) and Overall Neuropathy Limitations Scale (ONLS) assessments will be performed every six months for three years. The study will also investigate and compare the responsiveness of the outcome measures being used.

The main objective of the study is to explore and map brain areas involved in processing and perception in patients suffering from neurological pathologies and condition. The investigators hypothesize for example, that a change (compare to healthy subjects) in the perceptual maps and body representation could be detected and characterize in patients suffering from impairments of peripheral nerve conduction.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder of peripheral nerves. Intravenous immunoglobulins (IVIg) are a first line therapy for CIDP. The investigators used a transcriptomic approach to compare the gene expression profiles in the peripheral blood of patients having a CIDP or autoimmune diseases, before and after IVIg treatment, in order to identify their mechanism of action in this condition, to lead to a better understanding of CIDP pathophysiology, and potentially determine factors associated with the response to the treatment.

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an acquired neuropathy characterized by an inflammatory multifocal segmental demyelination. Due to the clinical heterogeneity of this condition and the lack of specific marker that can reliably identify all patients, the diagnosis of CIDP remains difficult. Similarly, there are no clear factors predicting the evolution or the prognosis of the disease. Current treatments are the intravenous immunoglobulin (IVIg), corticoids and plasma exchange; IVIg therapy being the most commonly used. Responses of the patients to the treatments are variable. Thus, it is necessary to identify predictive markers of the therapeutic response of CIDP patients treated by IVIg. Several potential biomarkers have been proposed recently, but none of them has yet been validated as a predictive criterion for therapeutic response. It is therefore necessary to continue to investigate several biological parameters to identify a reliable biological marker. In electromyography, the Motor Unit Number Index (MUNIX) technique allows measuring the axonal loss by a precise count of functional motor units. This method, more sensitive than the measure of the Compound Muscle Action Potential (CMAP), is rarely used in CIDP. MUNIX might be a good tool to better characterize the patients and to follow the course of CIDP. It also might be a new sensitive and reliable marker predictive of the therapeutic response. Magnetic resonance Imaging (MRI) is increasingly used for the assessment of neuromuscular diseases. A recent study on CIDP patients reported a significant decrease of the muscle Magnetisation Transfer Ratio (MTR) compared to healthy subjects, correlated to clinical parameters. The use of advances MRI techniques could allow characterizing the structure and composition of muscle and nerve tissues of CIDP patients. It could also be a mean for identifying potential new markers, largely unexplored until now, that might be sensitive to disease course and/or IVIg response. The objective of this study is to identify predictive markers of the treatment response of CIDP patients receiving IVIg. This is a prospective observational exploratory study of a cohort of 30 CIDP patients treated with IVIg and followed-up during one year.

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.

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.

The main aim of this study is to evaluate the rates of adverse events of special interest (AESIs) (thrombotic events, acute kidney injury [AKI], and hemolytic events) among participants with CIDP initiating GGL compared with rates among participants with CIDP initiating comparator intravenous immunoglobulin (IVIG) products. No study medicines will be provided to participants in this study.

The use of nerve ultrasound for the diagnosis and monitoring of neuromuscular diseases is a promising growing field (1). Non-invasive and painless, ultrasound provides additional data electroneuromyography (EMG), with a spatial resolution at least as good as MRI, while being easily accessible and inexpensive.The polyradiculoneuritis Inflammatory Demyelinating Chronicles (IPDC), Neuropathies Motrices in Multifocal Conduction Blocks (NMMBC) and neuropathy associated with anti-MAG antibodies are among the major chronic inflammatory neuropathies with an autoimmune etiology. The diagnosis of these pathologies is based on the clinic, diagnostic tests and EMG. The interest not only in the diagnosis, but also for monitoring these pathologies using ultrasound analysis nerves has been demonstrated recently in several studies. However the limited resolution of current ultrasound images do not allow detailed study of the internal structure of the nerves which could later help deepen knowledge in this innovative field and can better understand the pathophysiological mechanism of the evolution of these pathologies . To do this, the investigators have available a UHF ultrasound in the ultrasound department of the Nice University Hospital Pasteur Hospital 2 The investigators realize a descriptive analysis study, pilot, mono-centric, multi and prospective on patients followed in the center with a diagnosis of IPDC, a NMMBC or neuropathy with anti-MAG antibodies and control subjects matched by age and sex. All the patients and controls in this study will receive a briefing and must sign an informed consent. They realize an ultrasound study of the nerve, using an ultra high frequency ultrasound system that will allow the assessment of anatomical structures of nerve (size, structure, vascularisation and assessment booklets). Ultrasound data will, in a second stage, compared with the data obtained with the EMG and clinical scores obtained using clinical rating scales. 40 subjects will be included, divided into four subgroups: 10 subjects with a diagnosis of IPDC (1), 10 subjects with a diagnosis of NMMBC (2), 10 subjects with a diagnosis of neuropathy antibody-anti MAG (3) and 10 control subjects with no signs of neuropathy at the EMG examination.

This study aims analyze the transcriptome to identify predictive biomarkers of IVIG in CIDP patients. 25 patients with a diagnosis of CIDP according to European criteria, naïve of treatment, will be included and followed for 1 year. Clinical assessment (RT-MRC ; Martin vigorimeter, RT-mISS,R-ODS,TW25, 9hole-peg test) will be performed at baseline and at 3, 6 and 12 months after the first IVIG course. Responder/No responder status will be defined at 3 month and confirmed at 12 months. Blood samples will be collected before the IVIG course at baseline, and at 2months and 6 months. At the end of the first IVIG course a blood sample will be collected to assess early changes of transcriptome. The CIDP diagnosis and responder/no responder status will be confirmed by an independent committee. The transcriptome of the patients will be individually analyzed and compared regarding Responder/Non responder status to a control groups of 20 healthy subjects

The aim of this study is to quantify the effect of IVIG treatment in a group of patients with chronic inflammatory demyelinating polyradiculoneuropathy(CIDP), who requires continues treatment of IVIG at regular intervals of 3-10 weeks: During continues treatment of IVIG at regular intervals of 3-10 weeks. During pause in treatment. Hypothesis: The disease activity in the patients are cyclical correlating to the treatment intervals. Pause in treatment will increase disease activity, which can be quantified with symptom scores, disability scales, and clinical test. Primary effect parameter is muscle strength quantified by isokinetic dynamometry. Added to the protocol there is an immunological study of inflammatory markers in blood samples of patients under treatment pause.