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ABSTRACT Introduction/Aims Methods Results Discussion Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell dyscrasia. Polyneuropathy in POEMS syndrome may present as a subacute or chronic symmetric sensorimotor polyneuropathy, with electrophysiological features suggesting demyelination. Motor conduction block (CB), which is mostly seen in chronic inflammatory demyelinating polyneuropathy (CIDP), is considered an atypical electrophysiological feature in POEMS syndrome. We examined the frequency of motor CB in POEMS syndrome.Patients with POEMS syndrome from the database of our department who had been examined between August 2017 and December 2022 were included in this study. All of the patients' clinical and electrophysiological data were retrospectively collected and analyzed.We present the data of seven POEMS syndrome patients. Twenty‐eight upper extremity motor nerve conduction studies were performed on these patients, and partial CB was detected in seven upper extremity motor nerves (25%) of six of the patients. One patient had motor CB in both the median and ulnar nerves.The distinction between POEMS syndrome and CIDP is important since these conditions require different treatments. Motor CB in POEMS may be more common than has been generally believed. Clinicians should consider this when evaluating patients with demyelinating polyneuropathies and be meticulous in identifying CB. Data from much larger numbers of patients are needed. [ABSTRACT FROM AUTHOR]

Background and objectives: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired treatable autoimmune disorder. Due to limited availability and affordability of IV immunoglobulins and therapeutic plasma exchange in Pakistan, oral immunosuppressive drugs (ISDs) are used despite limited role in literature. The study aimed to determine the response to ISDs in CIDP patients by assessing the frequency of remission, reduction of disability using a neuropathy related disability score called Inflammatory Neuropathy Cause and Treatment score (or INCAT score), as well as reduction in steroid maintenance dose. Methods: The retrospective observational study of six months duration (May to October, 2020) was carried out in Aga Khan University Hospital, Karachi, Pakistan. Medical record of all the patients with idiopathic CIDP taking oral ISDs in last five years was selected which included bio-data, clinical signs and symptoms, medication details, and INCAT scores. Descriptive statistics were described i.e. frequency, percentages, mean/standard deviation using Microsoft Excel v.2021. Results: Out of thirteen patients, Azathioprine was used in nine, Mycophenolate mofetil in two and Cyclosporine in two, with remission (INCAT score improvement ≥ 1) achieved in eight, one and zero patients respectively. Duration of ISDs ranged from three to twenty-four months (average 15.8 months). Patients with monoclonal paraproteinemia and prior exposure to ISDs had a poor response to the introduction of subsequent ISDs. Conclusion: The study describes preliminary experience of the potential role of relatively cheaper and more convenient oral ISDs (especially Azathioprine) as an alternative or sparing agent to first line agents for CIDP and sets the stage for larger scale studies and randomized controlled trials. [ABSTRACT FROM AUTHOR]

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. We utilized the respective hospital information system and examined baseline data with clinical examination, medical letters, laboratory results, antibody status, nerve conduction studies, imaging and biopsy findings. Aside from clinical baseline data, we analyzed treatment outcomes using the Standard of Care (SOC) definition, as well as a comparison of an early (within the first 12 months after manifestation) versus late (more than 12 months after manifestation) onset of therapy. Results: In terms of treatment, most patients received intravenous immunoglobulin (56%) or prednisolone (39%) as their first therapy. Patients who started their initial treatment later experienced a worsening disease course, as reflected by a significant deterioration in their Inflammatory Neuropathy Cause and Treatment (INCAT) leg disability score. SOC-refractory patients had worse clinical outcomes than SOC-responders. Associated factors for SOC-refractory status included the presence of fatigue as a symptom and alcohol dependence. Conclusion: Timely diagnosis, prompt initiation of treatment and careful monitoring of treatment response are essential for the prevention of long-term disability in CIDP and suggest a "hit hard and early" treatment paradigm. [ABSTRACT FROM AUTHOR]

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a peripheral nerve disease with progressive or recurrent motor and sensory symptoms lasting over 2 months. The condition is autoimmune and is diagnosed through clinical, electrodiagnostic, and laboratory features. This narrative review aims to summarize the latest findings on fatigue in CIDP, including its prevalence, factors that influence its severity, origin, and management strategies. Until May 20th, 2023, the English medical literature was searched with the keywords: “CIDP,” and “fatigue.” Study design, patient group, clinical findings related to CIDP and fatigue, treatment, and current status information were extracted from each study. One reviewer conducted research and screening, and another conducted the review process. Studies in English, including original studies and case series involving a minimum of five patients, were included until May 20th, 2023. Fatigue is a common symptom in CIDP, although not specific to the disease, and profoundly impacts the patient’s quality of life. The mechanisms specific to fatigue in CIDP are still unknown. However, we suggest that fatigue is not solely a residual symptom and may be related to inflammatory conditions. Further research is needed, specifically on the central origin of fatigue. It is essential to analyze the novel treatment options for fatigue in CIDP because it is a significant contributing factor to morbidity.

Background and Aims: Charcot-Marie-Tooth (CMT) is a heterogeneous group of genetic neuropathies and is typically characterized by distal muscle weakness, sensory loss, pes cavus and areflexia. Herein we describe a case of CMT2CC presenting with proximal muscle weakness and equivocal electrophysiological features, that was misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Case report: A 30-year-old woman complained of proximal muscle weakness with difficulty climbing stairs. Neurological examination showed weakness in lower limb (LL) muscles, that was marked proximally and mild distally, and absence of deep tendon reflexes in the ankles. Nerve conduction studies (NCS) showed sensory-motor neuropathy with non-uniform NC velocity and a partial conduction block (CBs) in peroneal nerve and tibial nerves. Thus, a diagnosis of CIDP was entertained and the patient underwent ineffective treatment with intravenous immunoglobulins. At electrophysiological revaluation CB in peroneal nerve was undetectable as also distal CMAP had decreased whereas the CBs persisted in tibial nerves. Hypothesizing a hereditary neuropathy, we examined the proband's son, who presented mild weakness of distal and proximal muscles at lower limbs. Neurophysiological investigation showed findings consistent with an intermediate-axonal electrophysiological pattern. A targeted-NGS including 136 CMT genes showed the heterozygous frameshift mutation (c.3057dupG; p.K1020fs*43) in the NEFH gene, coding for the neurofilament heavy chain and causing CMT2CC. Interpretation: Diagnosis of a genetic neuropathy may be challenging when clinical features are atypical and/or electrophysiological features are misleading. The most common misdiagnosis is CIDP. Our report suggests that also CMT2CC patients with proximal muscle weakness and equivocal electrophysiological features might be misdiagnosed as CIDP. [ABSTRACT FROM AUTHOR]

Abstract Guillain–Barré syndrome and neuralgic amyotrophy have been associated with hepatitis E virus (HEV) genotype 3 infections, while myasthenia gravis (MG) has been associated with HEV genotype 4 infections. However, whether chronic inflammatory demyelinating polyneuropathy (CIDP) is associated with HEV infections has not been conclusively clarified yet. 102 CIDP patients, 102 age- and sex-matched blood donors, 61 peripheral neuropathy patients (non-CIDP patients), and 26 MG patients were tested for HEV and anti-HEV IgM and IgG. Sixty-five of the 102 (64%) CIDP patients tested positive for anti-HEV IgG and one (1%) for anti-HEV IgM. No other patient tested positive for ati-HEV IgM. In the subgroup of CIDP patients with initial diagnosis (without previous IVIG treatment), 30/54 (56%) tested positive for anti-HEV IgG. Anti-HEV rates were significantly lower in blood donors (28%), non-CIDP peripheral neuropathy patients (20%), and MG patients (12%). No subject tested positive for HEV viremia. CSF tested negative for in 61 CIDP patients (54 patients with primary diagnosis). The development of CIDP but not non-CIDP polyneuropathy may be triggered by HEV exposure in an HEV genotype 3 endemic region. The increased anti-HEV seroprevalence in CIDP patients is not a consequence of IVIG therapy.

Background: Therapeutic plasma exchange (TPE) is a highly effective rescue treatment for patients with acute exacerbation of neuroimmunological disease that removes circulating autoantibodies and inflammatory components from the bloodstream. The aims of this study are to explore the safety and the effectiveness of TPE in patients with autoimmune neurological disorders. Methods: We retrospectively evaluated the frequency of adverse events (AEs) and the effectiveness of TPE using the modified Ranking Scale (mRS) in patients with acute neurological flares who underwent TPE at the University Hospital of Palermo. Results: Of 59 patients, the majority underwent TPE due to multiple sclerosis (MS) relapse. In 23.7% of cases, TPE was performed before obtaining a definite diagnosis due to the severity of the clinical presentation. After TPE, the mRS score was globally reduced (p < 0.0001), and this effect was marked in patients with MS, Guillain–Barré syndrome, and myasthenia gravis crisis but not in those with paraneoplastic syndromes. Circulating pathogenetic antibodies, younger age, and the early use of TPE were factors strongly associated with TPE effectiveness. The overall safety profile of TPE was satisfactory with an AE frequency of 15%. Conclusions: These results highlight the early use of TPE in patients with circulating pathogenetic antibodies as well as its favorable safety profile.

Background: Therapeutic plasma exchange (TPE) is a highly effective rescue treatment for patients with acute exacerbation of neuroimmunological disease that removes circulating autoantibodies and inflammatory components from the bloodstream. The aims of this study are to explore the safety and the effectiveness of TPE in patients with autoimmune neurological disorders. Methods: We retrospectively evaluated the frequency of adverse events (AEs) and the effectiveness of TPE using the modified Ranking Scale (mRS) in patients with acute neurological flares who underwent TPE at the University Hospital of Palermo. Results: Of 59 patients, the majority underwent TPE due to multiple sclerosis (MS) relapse. In 23.7% of cases, TPE was performed before obtaining a definite diagnosis due to the severity of the clinical presentation. After TPE, the mRS score was globally reduced (p < 0.0001), and this effect was marked in patients with MS, Guillain–Barré syndrome, and myasthenia gravis crisis but not in those with paraneoplastic syndromes. Circulating pathogenetic antibodies, younger age, and the early use of TPE were factors strongly associated with TPE effectiveness. The overall safety profile of TPE was satisfactory with an AE frequency of 15%. Conclusions: These results highlight the early use of TPE in patients with circulating pathogenetic antibodies as well as its favorable safety profile. [ABSTRACT FROM AUTHOR]

Introduction: The value of a sural nerve biopsy for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is controversial. Evidence-based recommendations for its implementation are lacking. We investigated factors leading to biopsy and analyzed biopsy outcomes and consequences, assessed the predictability of biopsy outcomes through clinical parameters to avoid unnecessary biopsies, and compared results with electrophysiological and clinical severity to determine their prognostic value. Methods: 190 sural nerve biopsies were analyzed in two cohorts. One consisted of 163 biopsies and the second of 72 biopsies from the prospective Immune-mediated Neuropathies Biomaterial and Data registry (INHIBIT). Both have an intersection of 45 patients. 75 data sets from patients without biopsy were used. Analysis of nerve conduction studies, treatment, overall disability sum score (ODSS), biopsy outcomes, and diagnosis was performed. Results: 51% of biopsied patients received the diagnosis CIDP (77% fulfilled EFNS/PNS criteria), 21% were not CIDP typical, and 27% were unspecific. Biopsied patients responded less frequently to immunotherapies at time of biopsy than non-biopsied patients (p = 0.003). Immunotherapy was initiated more frequently after biopsy (p < 0.001) and more often with intravenous immunoglobulins (p < 0.0001). 76% of all biopsied patients met the electrophysiological criteria for CIDP. Sensory nerve action potential amplitudes of 0 µV still provide 73% of histological diagnostic value. Histologic signs of degeneration predicted ODSS worsening after 1 year (p = 0.028) but disease severity did not correlate with histological damage severity. Discussion: The main indication for nerve biopsy was the treatment of refractory cases of autoimmune neuropathies with the therapeutic consequence of treatment initiation or escalation. Sural biopsy also provided prognostic information. Even with extinguished sural SNAP, the biopsy can still have diagnostic value. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Whole‐body magnetic resonance neurography (MRN) is an imaging modality that shows peripheral nerve signal change in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We aimed to explore the diagnostic potential of whole‐body MRN and its potential as a monitoring tool after immunotherapy in treatment‐naïve CIDP patients. Methods: Whole‐body MRN using coronal 3‐dimensional short tau inversion recovery (STIR) sampling perfection with application‐optimized contrasts by using different flip angle evolution (SPACE) techniques was performed in patients being investigated for CIDP and in healthy controls. Baseline clinical neuropathy scales and electrophysiologic parameters were collected, and MRN findings were compared before and after CIDP treatment. Results: We found highly concordant symmetrical thickening and increased T2 signal intensities in the brachial/lumbosacral plexus, femoral, or sciatic nerves in five of the eight patients with a final diagnosis of CIDP and none of the healthy controls. There were no treatment‐related imaging changes in five patients with CIDP who completed a follow‐up study. Diffuse, symmetrical thickening, and increased T2 signal in root, plexus, and peripheral nerves were found in two patients ultimately excluded due to a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome in addition to signal changes in the muscles, bony lesions, organomegaly, and lymphadenopathy. Discussion: Whole‐body MRN imaging shows promise in detecting abnormalities in proximal nerve segments in patients with CIDP. Future studies evaluating the role of MRN in assessing treatment response should consider follow‐up scans after treatment durations of more than 4 months. [ABSTRACT FROM AUTHOR]

Neuromuscular disorders are a group of diseases which can result in severe development disorders. They can be demonstrated by many different symptoms. The case report presents the history of a patient who reported pain in the lower limbs and muscle weakness that made it difficult to move. Result of the EMG examination revealed neurogenic changes, suggesting the possibility of suffering from a motor variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), although not all the symptoms presented by the patient were characteristic. During subsequent hospitalizations, skin changes were noticed in the form of heliotropic erythema, which, based on a number of additional tests, finally led to the diagnosis of juvenile dermatomyositis. The occurrence of a neurological mask in the course of juvenile dermatomyositis indicates the need for a wide differential diagnosis. [ABSTRACT FROM AUTHOR]

Background: Contactin-1 (CNTN1) antibody-positive nodopathy is rare and exhibits distinct clinical symptoms such as tremors and ataxia. However, the mechanisms of these symptoms and the characteristics of the cerebral spinal fluid (CSF) remain unknown. Case presentation: Here, we report a case of recurrent CNTN1 antibody-positive nodopathy. Initially, a 45-year-old woman experiencing numbness in the upper limbs and weakness in the lower limbs was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Eleven years later, her symptoms worsened, and she began to experience tremors and ataxia. Tests for serum CNTN1, GT1a, and GQ1b antibodies returned positive. Subsequently, she was diagnosed with CNTN1 antibody-positive nodopathy and underwent plasmapheresis therapy, although the treatment's efficacy was limited. To gain a deeper understanding of the disease, we conducted a comprehensive literature review, identifying 52 cases of CNTN1 antibody-positive nodopathy to date, with a tremor prevalence of 26.9%. Additionally, we found that the average CSF protein level in CNTN1 antibody-positive nodopathy was 2.57 g/L, with 87% of patients exhibiting a CSF protein level above 1.5 g/L. Conclusion: We present a rare case of recurrent CNTN1 antibody-positive nodopathy. Our findings indicate a high prevalence of tremor (26.9%) and elevated CSF protein levels among patients with CNTN1 antibody-positive nodopathy. [ABSTRACT FROM AUTHOR]

Background & Objective: The existing practice in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is to initiate therapy with steroids, intravenous immune globulin (IVIg), or plasma exchange (PLEX) followed by period of immunosuppression. The objective of this study is to assess disability outcomes at 6 months after starting therapy. Methods: Patients who were diagnosed as having CIDP from the Army Hospital of Research and Referral, Delhi; who were initiated and maintained on therapy by treating neurologists with a six month follow up were included in this study. They were retrospectively divided into three groups based on initial therapy received. The primary outcome was comparison of the Inflammatory Neuropathy Cause and Treatment (INCAT) group overall disability sum score (INCAT-ODSS) at 6 months. Secondary outcomes were difference in score at 1 and 3 months, proportion with at least 20% response at 3 and 6 months ("responders") and proportion who needed "rescue" therapy during the 6 months. Results: Sixty patients (26 retrospective, 34 prospective) were included in this study. They were treated with IVIg (33), steroid (19) and PLEX (8). Baseline INCAT-ODSS score (±SD) was 7.2(2.2), 7.2(1.5) and 7.5(1.9) respectively. All received some form of oral immune suppression during follow up. Twenty one (35%) needed additional rescue therapy. There was an overall significant reduction in the mean INCAT-ODSS disability score from 7.2 to 3.1 (Mean difference 4.2; CI 3.6-4.8); p<0.01). Nearly 88% of patients (51/58) showed at least 20% improvement from baseline. Two were lost to follow up (1 IVIg, 1 steroid). There was no difference in the ODSS at 6 months [2.9(2.4), 3.5(2.7) and 2.7(1.3)] respectively. No difference in ODSS at 1 and 3 months. Proportion of responders at 6 months and proportion who needed rescue therapy were also similar. Conclusion: Irrespective of initial therapy and maintenance oral immunosuppression used, the overall disability reduction in treatment with IVIg, steroid or PLEX is significant; however the three modalities are comparable in terms of disability reduction at 6 months. At least a third may need additional rescue therapy. [ABSTRACT FROM AUTHOR]

Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare disease that targets the myelin sheath, while neuromyelitis optica spectrum disorder (NMOSD) with anti-aquaporin-4 antibodies (AQP4-Ab) affects astrocytes. We report a unique case of CIDP associated with NMOSD. Case presentation: A 49-year-old woman presented to the emergency department with recurrent episodes of vertigo and blurred vision. Brain magnetic resonance imaging (MRI) with contrast eight months before admission showed Dawson’s finger, and follow-up brain MRI showed a new hyperintense lesion. Visual evoked potential showed bilateral pre-chiasma lesions, and somatosensory evoked potential indicated lesions between the medulla and cerebral cortex. The patient tested positive for AQP4-Ab, and had ascending lower motor neuron weakness for the past 10 weeks. Electromyography revealed multiple demyelinating lesions suggestive of CIDP. The patient was intravenously administered corticosteroids, methotrexate, and azathioprine, resulting in clinical improvement. Conclusion: CIDP associated with NMOSD is a rare occurrence. In our patient, a combination of corticosteroids and immunosuppressants was effective. The mechanism of combined demyelination of the central and peripheral nervous systems is still not fully understood, and further immunological and pathological studies are needed.

Yusuf A Rajabally1,2 1Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Birmingham, B15 2TH, United Kingdom; 2Aston Medical School, Aston University, Birmingham, United KingdomCorrespondence: Yusuf A Rajabally, Inflammatory Neuropathy Clinic, Department of Neurology, University Hospitals Birmingham, Birmingham, B15 2TH, United Kingdom, Email y.rajabally@aston.ac.ukAbstract: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable autoimmune disorder, for which different treatment options are available. Current first-line evidence-based therapies for CIDP include intravenous and subcutaneous immunoglobulins, corticosteroids and plasma exchanges. Despite lack of evidence, cyclophosphamide, rituximab and mycophenolate mofetil are commonly used in circumstances of refractoriness and, more debatably, of perceived overdependence on first-line therapies. Rituximab is currently the object of a randomized controlled trial for CIDP. Based on case series, and although rarely considered, haematopoietic autologous stem cell transplants may be effective in refractory disease, with low mortality and high remission rates. A new therapeutic option has appeared with efgartigimod, a neonatal Fc receptor blocker, recently shown to significantly lower relapse rate versus placebo, after withdrawal from previous immunotherapy. Other neonatal Fc receptor blockers, nipocalimab and batoclimab, are under study. The C1 complement-inhibitor SAR445088, acting in the proximal portion of the classical complement system, is currently the subject of a new study in treatment-responsive, refractory and treatment-naïve subjects. Finally, Bruton Tyrosine Kinase inhibitors, which exert anti-B cell effects, may represent another future research avenue. The widening of the therapeutic armamentarium enhances the need for improved evaluation of treatment effects and reliable biomarkers in CIDP.Keywords: CIDP, immunoglobulins, corticosteroids, plasma exchange, efgartigimod

Iris N van Doorn,1 Filip Eftimov,1 Luuk Wieske,1,2 Ivo N van Schaik,1,3 Camiel Verhamme1 1Department of Neurology and Clinical Neurophysiology, Amsterdam Neuroscience and University of Amsterdam, Amsterdam UMC, location AMC, the Netherlands; 2Department of Clinical Neurophysiology, Sint Antonius Hospital, Nieuwegein, the Netherlands; 3Sanquin Blood Supply Foundation, Amsterdam, the NetherlandsCorrespondence: Camiel Verhamme, Department of Neurology and Clinical Neurophysiology, Amsterdam Neuroscience and University of Amsterdam, Meibergdreef 9, Amsterdam UMC, location AMC, 1105AZ, the Netherlands, Email c.verhamme@amsterdamumc.nlAbstract: Diagnosing Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) poses numerous challenges. The heterogeneous presentations of CIDP variants, its mimics, and the complexity of interpreting electrodiagnostic criteria are just a few of the many reasons for misdiagnoses. Early recognition and treatment are important to reduce the risk of irreversible axonal damage, which may lead to permanent disability. The diagnosis of CIDP is based on a combination of clinical symptoms, nerve conduction study findings that indicate demyelination, and other supportive criteria. In 2021, the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) published a revision on the most widely adopted guideline on the diagnosis and treatment of CIDP. This updated guideline now includes clinical and electrodiagnostic criteria for CIDP variants (previously termed atypical CIDP), updated supportive criteria, and sensory criteria as an integral part of the electrodiagnostic criteria. Due to its many rules and exceptions, this guideline is complex and misinterpretation of nerve conduction study findings remain common. CIDP is treatable with intravenous immunoglobulins, corticosteroids, and plasma exchange. The choice of therapy should be tailored to the individual patient’s situation, taking into account the severity of symptoms, potential side effects, patient autonomy, and past treatments. Treatment responses should be evaluated as objectively as possible using disability and impairment scales. Applying these outcome measures consistently in clinical practice aids in recognizing the effectiveness (or lack thereof) of a treatment and facilitates timely consideration of alternative diagnoses or treatments. This review provides an overview of the current perspectives on the diagnostic process and first-line treatments for managing the disease.Keywords: CIDP, treatment, diagnosis, NCS, imaging

• Upper limb neuropathic tremor is mediated by peripheral and central mechanisms, irrespective of the site of neuropathy. • Lower limb tremor is present in inflammatory neuropathy, is associated with greater disability, and leads to poorer balance. • These findings further contribute to our understanding of the disabling features of tremor and imbalance in neuropathy. The mechanisms underlying neuropathic tremor remain incompletely understood and a distinction has not been drawn between proximal and distal neuropathies. Lower limb tremor contributes to imbalance in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but this is unexplored in other neuropathies. We characterized upper and lower limb tremor in chronic immune sensory polyradiculopathy (CISP) and distal acquired demyelinating neuropathy with anti-MAG antibodies (DADS-MAG), contrasted to CIDP. This was a cross-sectional study of 38 patients (CIDP [n = 25], CISP [n = 7], DADS-MAG [n = 6]). Clinical assessment, tremor study recordings, nerve conduction studies, and somatosensory evoked potentials were performed. Balance was measured by force platform. Upper limb tremor was prevalent (CIDP 66%, CISP 70%, DADS-MAG 100%). Peak frequencies followed a gradient along the upper limb, unchanged by weight-loading. Lower limb tremor was also present (CIDP 32%, CISP 29%, DADS-MAG 66%) and associated with imbalance. Nerve conduction parameters correlated with upper limb tremor in DADS-MAG and CISP, and imbalance in CISP. Upper limb tremor is mediated by peripheral and central mechanisms regardless of distal or proximal pathology. Lower limb tremor correlates with peripheral nerve function and contributes to imbalance. This study contributes to the understanding of neuropathic tremor. Addressing lower limb tremor may be of therapeutic importance for neuropathy-associated imbalance. [ABSTRACT FROM AUTHOR]

Background and purpose: Although chronic inflammatory demyelinating polyneuropathy (CIDP) is understood as a disease affecting the peripheral nervous system, mild cognitive dysfunction, particularly in the executive domain, has been described to form part of the condition. Here our interest lay in CIDP‐related theory of mind (ToM) capacities as an aspect of social cognition relevant for many aspects of everyday life. Methods: Twenty‐nine patients with CIDP and 23 healthy controls participated in this study. They were subjected to overview cognitive testing, different executive function (EF) tasks, as well as to the Faux Pas Recognition Task (FPRT) for assessing cognitive ToM and the Reading the Mind in the Eyes Test (RMET) with respect to affective ToM. Results: Persons with CIDP and controls did not differ with respect to their overall cognitive state. However, in the German verbal fluency standard, the digit span forward and the digit span backward tests used as EF tasks patients performed significantly worse than controls. Further, performance was abnormally low in the FPRT, whilst the groups did not differ with respect to RMET results. The FPRT and digit span backward results correlated with each other. Conclusions: Patients with CIDP showed deficits in cognitive ToM performance together with EF dysfunction, whilst affective ToM was preserved. Altogether, the results suggest that low cognitive ToM capacities in patients with CIDP arise as a particular aspect of disease‐related executive dysfunction. [ABSTRACT FROM AUTHOR]

Background: The frequency of nodal–paranodal antibodies in HIV‐infected patients with chronic immune‐mediated radiculo‐neuropathies (IMRN) has not been previously described. Methods: HIV‐infected patients who met the inclusion criteria for chronic IMRN were screened for immunoglobulin G (IgG) antibodies directed against nodal (neurofascin (NF)186) and paranodal (NF155, contactin‐1 (CNTN1) and contactin‐associated protein(Caspr1)) cell adhesion molecules, using a live, cell‐based assay. To explore potential pathogenicity, binding of human IgG to myelinated co‐cultures was assessed by incubation with patients' sera positive for nodal or paranodal antibodies. Normal human serum was added as a source of complement to assess for complement activation as a mechanism for myelin injury. Results: Twenty‐four HIV‐infected patients with IMRN were included in the study, 15 with chronic inflammatory demyelinating polyneuropathy (CIDP), 4 with ventral root radiculopathies (VRR), and 5 with dorsal root ganglionopathies (DRG). Five patients with CIDP had combined central and peripheral demyelination (CCPD). Three patients (12.7%) tested positive for neurofascin IgG1 antibodies in the following categories: 1 patient with VRR was NF186 positive, and 2 patients were NF155 positive with DRG and mixed sensory‐motor demyelinating neuropathy with optic neuritis, respectively. Conclusion: The frequency of nodal–paranodal antibodies is similar among IMRN regardless of HIV status. Interpretation of the results in the context of HIV is challenging as there is uncertainty regarding pathogenicity of the antibodies, especially at low titres. Larger prospective immune studies are required to delineate pathogenicity in the context of HIV, and to establish a panel of antibodies to predict for a particular clinical phenotype. [ABSTRACT FROM AUTHOR]

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the world's most common treatable neuropathy which usually responds to immunosuppressive treatment. Autologous hematopoietic stem cell transplantation (aHSCT) is an intense way of inducing immunosuppression. Objective: We analyze the evolution of CIDP patients treated with aHSCT in our center. Methods: Between 2018 and 2023, persons with CIDP were prospectively autografted employing the "Mexican method" to conduct grafts on an outpatient basis, employing cyclophosphamide 200 mg/Kg and rituximab 1000 mg. The protocol is registered in ClinicalTrials.gov identifier NCT02674217. Results: In our center 21 autologous transplant cases were completed in 2018-2023. Seven patients provided data to assess the efficacy of the procedure. Positive responses (stabilization and/or improvement) were observed in all seven patients: Five reported improvements in the Inflammatory Neuropathy Cause and Treatment (1NCAT) score and one reported stabilization. In the Inflammatory Rasch-Built Overall Disability Scale (I-RODS) score. Median INCAT score was 5 (range 1-9), whereas median I-RODS score was 24 (range 11 -29). Five patients (71%) reported improvement in the INCAT score, one reported stabilization and one informed worsening; concerning the l-RODS score 5 (71%) informed improvement, whereas two reported stabilization. Conclusion: aHSCT conducted fully in an outpatient basis, employing the conditioning regimen of the "Mexican method" appears to be a feasible therapeutic option for persons with CIDP. Additional studies are needed to confirm these observations. [ABSTRACT FROM AUTHOR]

Introduction: Current guidelines for defining good outcomes in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) are predominately defined by experts. At present, we do not have a patient-anchored definition of what constitutes a "good" outcome. Our study aimed to assess the symptom burden of people living with CIDP, as well as satisfaction with treatments and clinical outcomes. Methods: We conducted an online-survey in CIDP patients registered with the US and Canadian GBS/CIDP foundations. Respondents answered general demographic and clinical questions, as well as satisfaction with current symptom burden and treatments, plus validated outcome measures. Results: A total of 318 individuals with self-reported CIDP completed the online survey, of whom 128 (40%) considered their current disease burden as satisfactory while 190 (60%) did not. Of 305 patients who answered the treatment satisfaction question, 222(74%) were satisfied with their treatments. Patients who were satisfied with their current symptoms had, on average, better scores in quality of life and disease severity scales, although regression modeling showed that only ability to walk, stable symptoms, and health utility scores were associated with symptom satisfaction. Treatment satisfaction was associated with stable symptoms, use of IVIG, and use of one versus no medication. Conclusions: A high proportion of members of the US and Canadian GBS/CIDP Foundations reporting a diagnosis of CIDP were unsatisfied with current symptoms, despite a high level of overall satisfaction with treatments. There is an unmet need for improving long-term outcomes in people with a diagnosis of CIDP, and for studying patient-centered long-term treatment goals. [ABSTRACT FROM AUTHOR]

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder characterised by the progressive demyelination of peripheral nerves, resulting in motor and sensory deficits. While much research has focused on clinical and electrophysiological aspects of CIDP, there is an emerging interest in exploring its impact on the visual system through visual evoked potentials (VEPs). This comprehensive review synthesises existing literature on VEP findings in CIDP patients, shedding light on their potential diagnostic and prognostic value. The review thoroughly examines studies spanning the last two decades, exploring VEP abnormalities in CIDP patients. Notably, VEP studies have consistently revealed prolonged latencies and reduced amplitudes in CIDP patients compared to healthy controls. These alterations in VEP parameters suggest that the demyelinating process extends beyond the peripheral nervous system to affect the central nervous system, particularly the optic nerve and its connections. The correlation between VEP abnormalities and clinical manifestations of CIDP, such as visual impairment and sensory deficits, underscores the clinical relevance of VEP assessment in CIDP management. Furthermore, this review addresses the potential utility of VEPs in aiding CIDP diagnosis and monitoring disease progression. VEP abnormalities may serve as valuable biomarkers for disease activity, helping clinicians make timely therapeutic decisions. Moreover, this review discusses the limitations and challenges associated with VEP assessment in CIDP, including variability in recording techniques and the need for standardised protocols. In conclusion, this review highlights the evolving role of VEPs as a non-invasive tool in CIDP evaluation. The consistent VEP abnormalities observed in CIDP patients suggest the involvement of the central nervous system in this demyelinating disorder. As our understanding of CIDP and its pathophysiology continues to evolve, further research in this area may lead to improved diagnostic accuracy and monitoring strategies, ultimately enhancing the clinical management of CIDP patients. [ABSTRACT FROM AUTHOR]

Introduction: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare but treatable immune-mediated chronic disorder of the peripheral nervous system. We describe a pediatric female patient with CIDP who presented with progressive muscle weakness in both upper and lower limbs. Case presentation: A 13-year-old female, belonging to an indigenous group, presented with the complaint of difficulty in walking for 4 months. The weakness was bilaterally symmetrical involving distal muscles with no sensory loss. Cranial nerve examination showed bilateral sensory neural hearing loss. Electromyography and Nerve Conduction Test were both suggestive of neuropathy but ruled out myopathy. After the diagnosis of CIDP, IV Methylprednisolone, and Azathioprine was given, which improved her symptoms. Conclusions: A 13-year-old female was diagnosed with CIPD. With timely intervention, the CIDP in children has a better prognosis than in adults. CIDP should be suspected in cases of muscle weakness even in cases of pediatric female patients in order to avoid delayed diagnosis and treatment, which could affect the prognosis.

Immune cells infiltrate the peripheral nervous system (PNS) after injury and with autoimmunity, but their net effect is divergent. After injury, immune cells are reparative, while in inflammatory neuropathies (e.g., Guillain Barré Syndrome and chronic inflammatory demyelinating polyneuropathy), immune cells are proinflammatory and promote autoimmune demyelination. An understanding of immune cell phenotypes that distinguish these conditions may, therefore, reveal new therapeutic targets for switching immune cells from an inflammatory role to a reparative state. In an autoimmune regulator (Aire)-deficient mouse model of inflammatory neuropathy, we used single-cell RNA sequencing of sciatic nerves to discover a transcriptionally heterogeneous cellular landscape, including multiple myeloid, innate lymphoid, and lymphoid cell types. Analysis of cell-cell ligand-receptor interactions uncovered a macrophage-mediated tumor necrosis factor-α (TNF-α) signaling axis that is induced by interferon-γ and required for initiation of autoimmune demyelination. Developmental trajectory visualization suggested that TNF-α signaling is associated with metabolic reprogramming of macrophages and polarization of macrophages from a reparative state in injury to a pathogenic, inflammatory state in autoimmunity. Autocrine TNF-α signaling induced macrophage expression of multiple genes (Clec4e, Marcksl1, Cxcl1, and Cxcl10) important in immune cell activation and recruitment. Genetic and antibody-based blockade of TNF-α/TNF-α signaling ameliorated clinical neuropathy, peripheral nerve infiltration, and demyelination, which provides preclinical evidence that the TNF-α axis may be effectively targeted to resolve inflammatory neuropathies.

Immunoglobulin G (IgG) therapy is an established long-term treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) that is commonly administered intravenously (IVIg). The subcutaneous immunoglobulin (SCIg) administration route is a safe and effective alternative option, approved by the United States Food and Drug Administration (FDA) in 2018, for maintenance treatment of adults with CIDP. Physicians and patients alike need to be aware of all their treatment options in order to make informed decisions and plan long-term treatment strategies. In this review, we collate the evidence for SCIg in CIDP from all published studies and discuss their implications and translation to clinical practice. We also provide guidance on the practicalities of how and when to transition patients from IVIg to SCIg and ongoing patient support. Evidence suggests that IVIg and SCIg have comparable long-term efficacy in CIDP. However, SCIg can provide additional benefits for some patients, including no requirement for venous access or premedication, and reduced frequency of systemic adverse events. Local-site reactions are more common with SCIg than IVIg, but these are mostly well-tolerated and abate with subsequent infusions. Data suggest that many patients prefer SCIg following transition from IVIg. SCIg preference may be a result of the independence and flexibility associated with self-infusion, whereas IVIg preference may be a result of familiarity and reliance on a healthcare professional for infusions. In practice, individualizing maintenance dosing based on disease behavior and determining the minimally effective IgG dose for individuals are key considerations irrespective of the administration route chosen.

Abstract Introduction Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the most common immune neuropathies leading to severe impairments in daily life. Current treatment options include intravenous immunoglobulins (IVIG), which are administered at intervals of 4–12 weeks. Determination of individual treatment intervals is challenging since existing clinical scores lack sensitivity to objectify small, partially fluctuating deficits in patients. End-of-dose phenomena described by patients, manifested by increased fatigue and worsening of (motor) symptoms, are currently difficult to detect. From a medical and socio-economic point of view, it is necessary to identify and validate new, more sensitive outcome measures for accurate mapping of disease progression and, thus, for interval finding. Digital health technologies such as wearables may be particularly useful for this purpose, as they record real-life data and consequently, in contrast to classic clinical 'snapshots’, can continuously depict the disease course. Methods In this prospective, observational, non-interventional, single-center, investigator-initiated study, CIDP patients treated with IVIG will be continuously monitored over a period of 6 months. Clinical scores and blood analyses will be assessed and collected during three visits (V1, V2, V3). Additionally, activity, sleep, and cardiac parameters will be recorded over the entire period using a wearable device. Further, patients' subjective disease development and quality of life will be recorded at various visits (read-outs). The usability of the smartwatch will be assessed at the end of the study. Perspective The study aims to evaluate different digital measurements obtained with the smartwatch and blood-based analyses for monitoring disease activity and progress in CIDP patients. In conjunction, both means of monitoring might offer detailed insights into behavioral and biological patterns associated with treatment-related fluctuations such as end-of-dose phenomena. Trial registration The study protocol was registered at ClinicalTrials.gov. Identifier: NCT05723848. Initially, the protocol was submitted prospectively on January 10, 2023. The trial was publicly released after formal improvements on February 13, 2023, after first patients were included according to the original protocol.

Inflammatory neuromuscular disorders encompass a diverse group of immune-mediated diseases with varying clinical manifestations and treatment responses. The identification of specific biomarkers has the potential to provide valuable insights into disease pathogenesis, aid in accurate diagnosis, predict disease course, and monitor treatment efficacy. However, the rarity and heterogeneity of these disorders pose significant challenges in the identification and implementation of reliable biomarkers. Here, we aim to provide a comprehensive review of biomarkers currently established in Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), myasthenia gravis (MG), and idiopathic inflammatory myopathy (IIM). It highlights the existing biomarkers in these disorders, including diagnostic, prognostic, predictive and monitoring biomarkers, while emphasizing the unmet need for additional specific biomarkers. The limitations and challenges associated with the current biomarkers are discussed, and the potential implications for disease management and personalized treatment strategies are explored. Collectively, biomarkers have the potential to improve the management of inflammatory neuromuscular disorders. However, novel strategies and further research are needed to establish clinically meaningful biomarkers. [ABSTRACT FROM AUTHOR]

Autoimmune neuromuscular diseases are a group of heterogenous pathologies secondary to the activation of the immune system that damage the structures of the peripheric nerve, the neuromuscular junction, or the skeleton muscle. The diagnosis of autoimmune neuromuscular disorders comprises a combination of data from clinical, laboratory, electromyography, imaging exam, and biopsy. Particularly, the whole-body MRI examination in the last two decades has been of great use in the assessment of neuromuscular disorders. MRI provides information about the structures involved and the status of activity of the disease. It can also be used as a biomarker, detect the pattern of specific muscle involvement, and is a useful tool for targeting the optimal muscle site for biopsy. In this work, we summarized the most used technical protocol of whole-body MRI and the role of this imaging technique in autoimmune neuromuscular disorders. [ABSTRACT FROM AUTHOR]

Introduction/Aims: Outcomes in chronic inflammatory demyelinating polyneuropathy (CIDP) have been reported in longitudinal and cross‐sectional studies. A considerable variation in long‐term disease outcome has appeared in those reports. To overcome this uncertainty, a systematic review and meta‐analysis was conducted on CIDP outcomes, including the parameters of case fatality rate, ambulation, physical ability, and remission. Methods: In accordance with the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guidelines, a systematic search was conducted in PubMed and EMBASE (OVID) for reports with at least 2 years of follow‐up on patients with active or previously active CIDP that were published no later than May 12, 2022. Studies were appraised for quality using the Joanna Briggs Institute Critical Appraisal Checklist for studies reporting prevalence data. Pooled analyses were conducted and the results were visualized using forest plots. The study protocol was registered prospectively on PROSPERO (CRD42021266903). Results: A total of 1290 titles were identified. Sixty‐nine full‐text articles were screened and 21 studies with 1199 patients were selected for the data analysis. The pooled case fatality rate was 3.3% (95% confidence interval [CI], 1.9% to 5.7%). The pooled fraction of nonambulatory patients was 8.2% (95% CI, 5.7% to 11.6%) and, overall, 47.1% (95% CI, 39.5% to 54.9%) of CIDP patients had a good outcome without disability. The pooled rate of remission was 40.8% (95% CI, 30.6% to 51.8%). Discussion: Future research is warranted on how to prevent long‐term impairment in CIDP. Care should be taken in developing clinical strategies to avoid immunomodulating therapy in the many patients in remission. See Editorial on pages 348–349 in this issue [ABSTRACT FROM AUTHOR]

Background: Intravenous immunoglobulins (IVIg) are the first-choice drugs for the treatment of certain neuroimmune diseases. The aim of this study was to evaluate the efficacy and safety of IVIg in patients with selected nervous system diseases. Methods: The study enrolled patients who received IVIg in programmes financed by the National Health Fund in Poland. The status of patients upon inclusion and during treatment was assessed using scales dedicated to specific neurological diseases. Results: The study enrolled 141 patients aged 56.28 ± 14.72 (51.77% female): 21 patients with myasthenia gravis (MG), 65 with chronic inflammatory demyelinating polyneuropathy (CIDP), 30 with Guillain–Barré syndrome (GBS), 12 with neuromyelitis optica spectrum disorder (NMOSD) and 13 patients with autoimmune encephalitis (AE). Neurological improvement was found in 14 (66.66%) MG patients (with a reduction of at least three points on the Quantitative Myasthenia Gravis Score (QMGS) within 14 days from the completion of the cycle), and in 34 (52.3%) GBS patients (with a reduction of at least one point on the Medical Research Council Scale within 14 days from the completion of the cycle). The parameters with the strongest effect on clinical improvement in MG patients were age [OR 1.033, CI 95% [0.09–1.09], p = 0.049] and baseline QMGS [OR 0.505; CI 95% [0.24–0.87], p = 0.038]. In the majority of CIDP patients (27, 97%) and NMOSD patients (6, 50%), neurological stabilisation was observed (without clinical improvement, defined for CIDP patients as an increase of at least two points on the Lovett Scale after three courses of IVIg were administered, and for NMOSD patients as an increase of at least one point on the Medical Research Council Scale and/or a shift of at least 0.3 logMAR after three courses of treatment). Deep-vein thrombosis was only one serious adverse event in the total group of patients treated with IVIg. Conclusions: The use of IVIg in patients with MG and GBS mostly results in neurological improvement, while in patients with NMOSD and CIDP, it mostly results in disease stabilisation. This could indicate the predominant anti-idiotypic antibody activity of IVIg in acute neuroimmune diseases or during exacerbations in chronic autoimmune diseases. The therapy of AE in comorbid neoplastic disease is burdened with an elevated risk of failure for IVIg. The results of our study confirm the improved safety of IVIg for selected neurological diseases. [ABSTRACT FROM AUTHOR]

The value of practice guidelines in the three most common autoimmune neuromuscular disorders, namely Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), Myasthenia Gravis (MG) and Autoimmune Inflammatory Myopathies (AIM), has been extensively debated regarding their usefulness in clinical practice, objectivity and universal value considering that guidelines are also established regionally in certain countries. This commentary highlights common concerns on how guidelines are presently generated, pointing out: (a) non-sufficient diversity among Task-Force members to identify and address not only routine clinical and electrophysiology issues but also immunology, imaging, pathology, biomarkers, epidemiology or treatment economics; (b) Task-Force being often comprised by the same or seemingly like-minded members conveying the erroneous impression that experts with opposing views might have been excluded, even if this is clearly not the case; and (c) relying on web-based registries or retrospective data collections from heterogeneous sources. As a result, the existing practice guidelines in CIDP, MG and AIM remain an unfinished business but an excellent base for further enhancement. Guidelines can be extremely helpful not only for clinical trials but also in clinical practice if viewed as a living document with continuously updated versions by experts even with opposing views with precise information on diagnostics, pathomechanisms, therapeutic schemes, evolving biomarkers and economics of new therapies with validation of the post-guidelines criteria. Geographic diversity should be taken into consideration because the availability of biomarker testing, and therapies differ among countries. Patient preferences need to be also considered in therapeutic guidelines because newly marketed drugs offer more options steadily changing the therapeutic algorithms in autoimmune neuromuscular diseases generating also questions as to whether they also influence decisions on insurance coverage. Collectively, these startup considerations are aimed to make practice guidelines more objective, widely acceptable worldwide and more practical or easier to follow in clinical practice. [ABSTRACT FROM AUTHOR]

Background and Aims: Imbalance is a prominent symptom of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Although upper limb tremor in CIDP is described, lower limb tremor has not been assessed. The aim of this study was to determine whether lower limb tremor was present in CIDP and assess potential relationships with imbalance. Methods: This was a cross‐sectional observational study of prospectively recruited consecutive patients with typical CIDP (N = 25). Clinical phenotyping, lower limb nerve conduction and tremor studies, and posturography analyses were performed. The Berg Balance Scale (BBS) divided CIDP patients into those with "good" and "poor" balance. Results: Lower limb tremor was evident in 32% of CIDP patients and associated with poor balance (BBSTremor 35 [23–46], BBSNo Tremor 52 [44–55], p =.035). Tremor frequency was 10.2–12.5 Hz with legs outstretched and on standing, apart from four patients with a lower frequency tremor (3.8–4.6 Hz) while standing. Posturography analysis revealed a high‐frequency spectral peak in the vertical axis in 44% of CIDP patients (16.0 ± 0.4 Hz). This was more likely in those with "good" balance (40% vs. 4%, p =.013). Interpretation: Lower limb tremor is present in one third of CIDP patients and is associated with poor balance. A high‐frequency peak on posturography is associated with better balance in CIDP. Lower limb tremor and posturography assessments could serve as important biomarkers of balance in a clinical setting. [ABSTRACT FROM AUTHOR]

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Abstract Introduction Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare progressive or relapsing inflammatory disease. Intravenous immunoglobulin (IVIG) is recommended as a first-line therapy. The aim of this study was to describe real-world treatment patterns and outcomes of patients with CIDP in the Define initiating IVIG treatment. Methods This cohort study used health insurance claims data from the Merative MarketScan Research Databases (2008–2018). Adult patients (≥ 18 years old) with CIDP without prior immunoglobulin treatment were identified using International Statistical Classification of Diseases and Related Health Problems (ICD) codes, and patients subsequently initiating IVIG were included in the analysis. Real-world IVIG treatment patterns and treatment and safety outcomes (assessed via ICD codes) were described. Results In total, 3975 patients (median age 58 years) with CIDP who initiated IVIG were identified. After the initial IVIG loading period, patients received IVIG at a median dosing interval of 21 days (quartile [Q]1, Q3: 7, 28), and continued treatment for a median of 129 days (Q1, Q3: 85, 271). After the 2-year follow-up period, 55% of patients had discontinued all IVIG treatment; more than one-half of these discontinuations occurred within 4 months. Diagnoses of impaired functional status were evident in more than 30% of patients at baseline, but at lower rates during follow-up. Rates of new-onset safety outcomes after IVIG treatment were low. Conclusion This real-world analysis of IVIG treatment patterns and treatment and safety outcomes of patients with CIDP who initiated IVIG highlights the unmet need for improved long-term management. Further research is needed to evaluate the use of functional status measures as endpoints for immunoglobulin treatment effectiveness.

Abstract Introduction/Aims Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patient’s ability to walk and perform activities of daily living independently. Furthermore, patients often report fatigue and depression which can affect their quality of life. These symptoms were assessed in CIDP patients receiving long-term intravenous immunoglobulin (IVIG) treatment. Methods GAMEDIS was a multi-center, prospective, non-interventional study in adult CIDP patients treated with IVIG (10%) and followed for two years. Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Hughes Disability Scale (HDS), Fatigue Severity Scale (FSS), Beck Depression Inventory II (BDI), Short Form-36 health survey (SF-36) and Work Productivity and Activity Impairment Score Attributable to General Health (WPAI-GH) were assessed at baseline and quarterly. Dosing and treatment intervals, changes in outcome parameters, and adverse events (AEs) were analyzed. Results 148 evaluable patients were followed for a mean of 83.3 weeks. The mean maintenance IVIG dose was 0.9 g/kg/cycle (mean cycle interval 38 days). Disability and fatigue remained stable throughout the study. Mean INCAT score: 2.4 ± 1.8 at baseline and 2.5 ± 1.9 at study end. HDS: 74.3% healthy/minor symptoms at baseline and 71.6% at study end. Mean FSS: 4.2 ± 1.6 at baseline and 4.1 ± 1.7 at study end. All patients reported minimal/no depression at baseline and throughout. SF-36 and WPAI-GH scores remained stable. Fifteen patients (9.5%) experienced potentially treatment-related AEs. There were no AEs in 99.3% of infusions. Discussion Long-term treatment of CIDP patients with IVIG 10% in real-world conditions maintained clinical stability on fatigue and depression over 96 weeks. This treatment was well-tolerated and safe.

Background: Intravenous immunoglobulin (IVIg) is efficient and one of very few treatment options for patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). However, finding the optimal dose of IVIg for individual CIDP patients remains challenging. The dose of IVIg needs to be adjusted individually. Considering the high healthcare costs of IVIg therapy, the overtreatment of some patients seen in placebo studies and the shortage of IVIg we recently experienced, as well as identifying factors associated with the required dose of IVIg in maintenance treatment, is extremely important. Thus, in this retrospective study, we analyze characteristics of patients with stable CIDP, which are associated with the required drug dose. Methods: 32 patients with stable CIDP treated with IVIg between July 2021 and July 2022 were identified from our database and included in this retrospective study. Patients’ characteristics were registered, and parameters were identified that were associated with the IVIg dose. Results: Age, cerebrospinal fluid protein elevation, disease duration, delay between symptom onset/diagnosis, Inflammatory Neuropathy Cause and Treatment (INCAT) score, and Medical Research Council Sum Score (MRC SS) were significantly associated with the required drug dose. In addition, an association of age, sex, elevated CSF protein, time interval between symptom onset and diagnosis, and the MRC SS with the required IVIg dose could be demonstrated in the multivariable regression analysis. Conclusions: Our model, which is based on routine parameters that are simple to address in the clinical practice, can be useful in adjusting the IVIg dose in patients with stable CIDP.

Abstract In many instances, the differential diagnosis between Guillain–Barre syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP) may be challenging. The aim of this letter to the editor is to elucidate comments and concerns raised, regarding our latest published article dealing with two patients that developed acute-onset CIDP after SARS-CoV-2 infection and Ad26.COV2.S vaccination, respectively.

Different types of solvents and adhesives are used in a shoe factory. The toxic chemicals can cause acute and chronic effects on the central and peripheral nervous system. Here, we report a case of a 25-year-old young man working as a coloring and gluing worker in a shoe factory, who presented with progressive quadriparesis following exposure of six months. Some of his coworkers also developed a similar illness. Nerve conduction velocity studies of the patient showed combined sensory and motor demyelinating and axonal polyneuropathy. Cerebrospinal fluid (CSF) analysis was normal. With conservative management and physiotherapy, there is a gradual improvement of the condition in the patient who can ambulate independently now. [ABSTRACT FROM AUTHOR]

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is one of the most common immune neuropathies leading to severe impairments in daily life. Current treatment options include intravenous immunoglobulins (IVIG), which are administered at intervals of 4–12 weeks. Determination of individual treatment intervals is challenging since existing clinical scores lack sensitivity to objectify small, partially fluctuating deficits in patients. End-of-dose phenomena described by patients, manifested by increased fatigue and worsening of (motor) symptoms, are currently difficult to detect. From a medical and socio-economic point of view, it is necessary to identify and validate new, more sensitive outcome measures for accurate mapping of disease progression and, thus, for interval finding. Digital health technologies such as wearables may be particularly useful for this purpose, as they record real-life data and consequently, in contrast to classic clinical 'snapshots', can continuously depict the disease course. Methods: In this prospective, observational, non-interventional, single-center, investigator-initiated study, CIDP patients treated with IVIG will be continuously monitored over a period of 6 months. Clinical scores and blood analyses will be assessed and collected during three visits (V1, V2, V3). Additionally, activity, sleep, and cardiac parameters will be recorded over the entire period using a wearable device. Further, patients' subjective disease development and quality of life will be recorded at various visits (read-outs). The usability of the smartwatch will be assessed at the end of the study. Perspective: The study aims to evaluate different digital measurements obtained with the smartwatch and blood-based analyses for monitoring disease activity and progress in CIDP patients. In conjunction, both means of monitoring might offer detailed insights into behavioral and biological patterns associated with treatment-related fluctuations such as end-of-dose phenomena. Trial registration: The study protocol was registered at ClinicalTrials.gov. Identifier: NCT05723848. Initially, the protocol was submitted prospectively on January 10, 2023. The trial was publicly released after formal improvements on February 13, 2023, after first patients were included according to the original protocol. [ABSTRACT FROM AUTHOR]

Background and purpose: Treatment options for chronic inflammatory demyelinating polyneuropathy (CIDP) are intravenous immunoglobulin, plasmapheresis, corticosteroids and immunosuppressive drugs. However, a substantial proportion of patients with CIDP remain refractory to treatment and develop severe functional disability. A systematic review and a meta‐analysis of the efficacy of hematopoietic stem cell transplantation (HSCT) treatment in refractory CIDP patients was performed. Methods: The study is based on queries in the PubMed, Cochrane Central Register of Controlled Trials, Embase, Web of Science and clinicaltrials.gov databases on 4 December 2022. Articles that met our eligibility criteria were included after screening. Patients' characteristics, treatment regime and outcome measures were extracted. Results: Eighty‐nine patients in 11 studies were included. The pooled estimate of responsiveness amongst the four included studies was 87.04% (95% confidence interval 66.7%–99.5%) and the pooled estimate of freedom of all immune modulating or suppressive drugs was 80.75% (95% confidence interval 71.2%–90.2%). Conclusion: This meta‐analysis and systematic review suggested that HSCT can be effective in the treatment of refractory CIDP. Whilst there are risks involved, HSCT may be a beneficial and viable therapy for refractory CIDP when carefully evaluated. [ABSTRACT FROM AUTHOR]

Introduction: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare progressive or relapsing inflammatory disease. Intravenous immunoglobulin (IVIG) is recommended as a first-line therapy. The aim of this study was to describe real-world treatment patterns and outcomes of patients with CIDP in the Define initiating IVIG treatment. Methods: This cohort study used health insurance claims data from the Merative MarketScan Research Databases (2008–2018). Adult patients (≥ 18 years old) with CIDP without prior immunoglobulin treatment were identified using International Statistical Classification of Diseases and Related Health Problems (ICD) codes, and patients subsequently initiating IVIG were included in the analysis. Real-world IVIG treatment patterns and treatment and safety outcomes (assessed via ICD codes) were described. Results: In total, 3975 patients (median age 58 years) with CIDP who initiated IVIG were identified. After the initial IVIG loading period, patients received IVIG at a median dosing interval of 21 days (quartile [Q]1, Q3: 7, 28), and continued treatment for a median of 129 days (Q1, Q3: 85, 271). After the 2-year follow-up period, 55% of patients had discontinued all IVIG treatment; more than one-half of these discontinuations occurred within 4 months. Diagnoses of impaired functional status were evident in more than 30% of patients at baseline, but at lower rates during follow-up. Rates of new-onset safety outcomes after IVIG treatment were low. Conclusion: This real-world analysis of IVIG treatment patterns and treatment and safety outcomes of patients with CIDP who initiated IVIG highlights the unmet need for improved long-term management. Further research is needed to evaluate the use of functional status measures as endpoints for immunoglobulin treatment effectiveness. Plain Language Summary: Chronic inflammatory demyelinating polyradiculoneuropathy, also called CIDP, is a rare disease that causes the body's immune system to attack its nerves. Treatments for CIDP include antibodies, which are also called immunoglobulins. Immunoglobulins may be given intravenously, meaning they are administered into a vein. Intravenous immunoglobulin, also called IVIG, is recommended as one of the first treatments that patients with CIDP receive in their therapy and involves giving antibodies through a drip into a vein. This study aimed to gather information on the day-to-day use of IVIG by patients with CIDP. Information from 2008 to 2018 was collected from a large health insurance database in the USA. Information was taken from the records of patients aged 18 years or older who had received IVIG during the information collection period. In total, records from 3975 patients with an average age of 58 years were included in the study. On average, patients received IVIG every 21 days for 129 days. By 2 years, 55% of patients had stopped receiving IVIG; most of those patients had stopped within 4 months of first receiving the treatment. In the 6 months before receiving IVIG, over 30% of patients experienced limitations owing to their CIDP that affected their daily lives, although this percentage became smaller once patients started to receive IVIG. In addition, a low number of patients experienced side effects because of their IVIG treatment. This study highlights that improved long-term care for patients with CIDP is needed. Further research into ways of measuring the impact of CIDP on patients' daily lives is required, which may help doctors to work out how effective IVIG is at treating CIDP. [ABSTRACT FROM AUTHOR]

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self‐reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies. Aim: To systematically assess the self‐reported limitations in activities and participation and determine associated factors. Methods: A survey study was conducted in 2021 in a cohort of patients with CIDP (n = 257) and MMN (n = 148) from a university hospital. The survey included the Rasch‐built Overall Disability Scale and the Utrecht Scale for Evaluation of Rehabilitation‐Participation, questions addressing personal and disease‐related factors and treatment. Multivariate linear regression analysis was used to determine associations with disease‐related and personal factors. Results: A total of 147 CIDP and 103 MMN patients responded. Limitations in activities were reported by 70.7% CIDP and 52.2% MMN patients with moderate to severe limitations in 22.4% and 5.9% patients, respectively. Participation restrictions were reported by 50% of CIDP and 40% of MMN patients, nevertheless satisfaction with participation was high. Fatigue, pain and resilience were independently associated with limitations in activities and satisfaction with participation in CIDP patients. Conclusions: Activity limitations and restrictions in participation are common in CIDP patients and to a lesser extent in MMN patients. Fatigue, pain and resilience independently contributed to perceived limitations in CIDP patients. Referral to a rehabilitation physician is warranted to address these limitations appropriately. [ABSTRACT FROM AUTHOR]

Background and Aims: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune‐mediated disease of the peripheral nerves, with significant unmet treatment needs. Clinical trials in CIDP are challenging; thus, new trial designs are needed. We present design of an open‐label phase 2 study (NCT04658472) evaluating efficacy and safety of SAR445088, a monoclonal antibody targeting complement C1s, in CIDP. Methods: This phase 2, proof‐of‐concept, multicenter, open‐label trial will evaluate the efficacy, and safety of SAR445088 in 90 patients with CIDP across three groups: (1) currently treated with standard‐of‐care (SOC) therapies, including immunoglobulin or corticosteroids (SOC‐Treated); (2) refractory to SOC (SOC‐Refractory); and (3) naïve to SOC (SOC‐Naïve). Enrolled participants undergo a 24‐week treatment period (part A), followed by an optional treatment extension for up to an additional 52 weeks (part B). In part A, the primary endpoint for the SOC‐Treated group is the percentage of participants with a relapse after switching from SOC to SAR445088. The primary endpoint for the SOC‐Refractory and SOC‐Naïve groups is the percentage of participants with a response, compared to baseline. Secondary endpoints include safety, tolerability, immunogenicity, and efficacy of SAR445088 during 12‐week overlapping period (SOC‐Treated). Part B evaluates long‐term safety and durability of efficacy. Data analysis will be performed using Bayesian statistics (predefined efficacy thresholds) and historical data‐based placebo assumptions to support program decision‐making. Interpretation: This innovative trial design based on patient groups and Bayesian statistics provides an efficient paradigm to evaluate new treatment candidates across the CIDP spectrum and can help accelerate development of new therapies. [ABSTRACT FROM AUTHOR]

Les nodopathies auto-immunes (NA) sont des pathologies démyélinisantes du système nerveux périphérique qui ont la particularité d'avoir une physiopathologie médiée par des anticorps majoritairement d'isotype IgG4. La présence de ces anticorps, l'installation aiguë ou subaiguë de la neuropathie, la mauvaise réponse aux immunoglobulines intraveineuses et la bonne sensibilité au rituximab (anticorps monoclonal thérapeutique anti-CD20) ont récemment conduit à exclure les NA du groupe des polyradiculoneuropathies inflammatoires démyélinisantes chroniques. À ce jour, quatre auto-anticorps ciblant des protéines axo-gliales au niveau des nœuds de Ranvier et des paranœuds ont été identifiés : les anti-pan-neurofascine (anti-pan-Nfasc) qui reconnaissent toutes les isoformes de neurofascine, les anti-neurofascine 155 (anti-Nfasc155), les anti-contactine 1 (anti-CNTN1) et les anti- contactin associated protein 1 (anti-CASPR1). Actuellement en France, l'identification biologique de ces anticorps s'effectue en cytométrie en flux sur cellules transfectées HEK dans le laboratoire d'immunologie de l'AP-HM, à l'Hôpital La Conception à Marseille. Les NA engagent le pronostic fonctionnel et parfois même le pronostic vital. Leur diagnostic doit donc être le plus rapide possible pour de ne pas retarder la prise en charge thérapeutique. Autoimmune nodopathy (AN) are demyelinating pathologies of the peripheral nervous system which have the particularity of having a pathophysiology mediated by antibodies mainly of IgG4 isotype. Specific autoantibodies, the acute or subacute onset of neuropathy, the poor response to intravenous immunoglobulins and the improvement after rituximab led to exclude AN from the group of chronic inflammatory demyelinating polyradiculoneuropathies. Currently, 4 auto-antibodies targeting axo-glial proteins at the nodes of Ranvier and paranodes have been identified: anti-pan-neurofascin (anti-pan-Nfasc) antibodies which recognize all neurofascin isoforms, anti-neurofascin 155 (anti-Nfasc155), anti-contactin 1 (anti-CNTNI) and anti-contactin associated protein 1 (anti-CASPRI). Currently, in France, the identification of these antibodies is processed by flow cytometry on transfected HEK cells in the Immunology laboratory at the hospital La Conception in Marseille. As AN can cause severe disability and sometimes be life-threatening, their diagnosis should be done as soon as possible in order to start promptly the treatment. [ABSTRACT FROM AUTHOR]

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