A Safety Study of GAMMAGARD LIQUID (GGL) in Participants With Chronic Inflammatory Demyelinating...
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)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.
Descriptive Analysis of Morphological Aspects of Nerve by Ultra-high Frequency Ultrasound (30-50MHZ)...
PolyradiculoneuropathyChronic Inflammatory DemyelinatingThe 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.
Prevalence of Decreased Corneal Sensation in Patients With Chronic Inflammatory Demyelinating Polyneuropathy...
Chronic Inflammatory Demyelinating PolyneuropathyChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating chronic progressive or relapsing neuropathy believed to be secondary to an autoimmune response against peripheral nerve antigens.5 We have observed two patients with CIDP with decreased corneal sensation who also suffered neurotrophic corneal ulcers and severe visual loss in the affected eyes. We want to explore the relationship of CIDP and corneal sensitivity. Our hypothesis is that people with CIDP have decreased corneal sensation compared to those without. We plan to perform a prospective study measuring corneal sensation in patients (proposed n=10) with CIDP and without to determine (1) if a difference exists in patients with CIDP compared to controls and (2) the magnitude of the difference. If a difference is detected in corneal sensitivity in patients with CIDP, this awareness amongst physicians and patients may help prevent blinding complications.
Biomark Study: Predict Intravenous Immunoglobulin Responders in Chronic Inflammatory Demyelinating...
CIDPThis 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
Study of CIDP Patients During IVIG Treatment
PolyradiculoneuropathyChronic Inflammatory DemyelinatingThe 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.
Immunoglobulin Dosage and Administration Form in CIDP and MMN
Chronic Inflammatory Demyelinating PolyneuropathyMultifocal Motor Neuropathy1 moreThe aim of this study is to evaluate development of hemolysis and the variation in isokinetic muscle strength in two groups of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) or multifocal motor neuropathy (MMN) Patients shifted from 3- or 6-weekly treatment with intravenous immunoglobulin (IVIG) to weekly treatment with subcutanoeus immunoglobulin (SCIG) Patients shifted from SCIG treatment with Subcuvia® or Hizentra® to Gammanorm®. Hypotheses During treatment with IVIG blood hemoglobin will fluctuate with a decline due to infusion, whereas it will remain stable during SCIG treatment without fluctuation Isokinetic muscle strength in affected muscle groups is more stable during treatment with SCIG than with IVIG Blood hemoglobin and changes in muscle strength is comparable during Subcuvia® or Hizentra® and Gammanorm® treatment
MRI in Diagnosing and Monitoring CIDP
Chronic Inflammatory Demyelinating PolyradiculoneuropathyWe want to study whether MRI can be useful in diagnosing and monitoring patients with CIDP in maintenance treatment with immunoglobulin
Diffusion Tensor Imaging in Chronic Inflammatory Demyelinating Polyneuropathy (PIDC)
CIDP (Chronic Inflammatory Demyelinating Polyradiculoneuropathy)CMT (Charcot Marie Tooth Disease)The main purpose of this study is to assess the clinical feasibility of diffusion tensor imaging (DTI) for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). For thar purpose, investigator will compare, fractional anisotropy (FA) obtained by diffusion tensor imaging (DTI) MRI 3T on brachial plexus and cervical spinal nerve roots between patients with defined Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP), according to the EFNS 2010 criteria, and healthy controls. The secondary outcomes will be to compare DTI parameters (FA, ADC or Apparent Diffusion Coefficient) between CIDP patients, healthy volunteers, and patients with Charcot Marie Tooth disease type 1a (CMT1a) and MRI morphological parameters (T1, STIR) between these groups. Moreover, investigator will investigate the possible relationship between MRI parameters, clinical indices, and electrophysiological measure.
An Expanded Access IND for the Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)...
Chronic Inflammatory Demyelinating PolyneuropathyThis is an Individual Patient Expanded Access IND to evaluate the safety and preliminary efficacy of autologous HB-adMSCs for treating a single patient Chronic Inflammatory Demyelinating Polyneuropathy, CIDP. The expanded access program will include a screening period of up to 28 days, a 44-week treatment period, a safety follow-up at 50, and a 52-week end-of-study visit.
sCD163 & CD19 as Candidate Biomarkers in CIDP and MMN
PolyradiculoneuropathyChronic Inflammatory DemyelinatingChronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are characterized by progressive deterioration in muscle strength, loss of sensibility, diminished or absent reflexes and impaired fine motor control. Often it is caused by demyelination which is suitable for treatment but damage to the axons may also occur especially in case of insufficient treatment. CIDP and MMN are immune mediated neuropathies in which first choice of treatment is intravenous immunoglobulin (IVIG), although the mechanisms underlying the effect of the IVIG is not yet clarified. The patients are diagnosed by electrophysiological examination and elevated level of protein in the cerebrospinal fluid. The diagnosis may be difficult to make due to great clinical variation and insensitive examinations methods including lack of biomarkers. The purpose of this study is to define if patients treated with SCIG and IVIG for CIDP and MMN have higher concentrations of sCD163 and CD19 in their cerebrospinal fluid and serum compared with symptomatic control subjects and is related to disease severity. Furthermore it is to define if patients newly diagnosed with CIDP or MMN have higher levels of sCD163 and CD19, than patients treated regularly with SCIG and IVIG.