Diagnosis Of Chronic Inflammatory Demyelinating Polyneuropathy Research Articles

Charcot-Marie-Tooth type 2CC misdiagnosed as Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

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). 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. 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.

Pediatric Chronic Inflammatory Demyelinating Polyneuropathy: Challenges in Diagnosis andTherapeutic Strategies.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune neurological disorder seen in both pediatric and adult populations. CIDP typically presents with progressive and persistent weakness over at least 4 weeks in addition to sensory symptoms in the extremities. Although CIDP shares common clinical features between children and adults, it sometimes presents as a distinct clinical entity in children that requires close attention and recognition. A major caveat when diagnosing a child with CIDP is the clinical and diagnostic overlap with inherited neuropathies, most commonly Charcot-Marie-Tooth disease (CMT). Demyelinating CMT (dCMT) and CIDP might share similar clinical presentations, and sometimes it might be difficult to differentiate them on the basis of the electrodiagnostic findings or cerebrospinal fluid (CSF) albumino-cytological dissociation. This indeed merits early consideration for genetic testing in patients who do not respond to conventional CIDP therapies. Current treatment options for CIDP include intravenous immunoglobulins (IVIG), corticosteroids (CS), and plasmapheresis (PLEX). The need for novel therapies is essential in instances where patients continue to have symptoms despite the standard therapies or due to adverse effects of long-term use of standard therapies such as CS. This paper reviews the challenges in the diagnosis of CIDP in children and the current as well as novel therapies for CIDP.

Chronic inflammatory demyelinating polyneuropathy following COVID-19 vaccination: a case report and literature review

BackgroundSevere post-vaccination neurological complications are rare. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated polyneuropathy affecting the peripheral nerve roots, which is not well described as a post-vaccination side effect. Here, we present a rare complication of vaccination against SARS-CoV-2, reaching a diagnosis of CIDP.Case presentationA 67-year-old diabetic male presented with lower extremity paresthesia and weakness following the third dose of the Sinopharm (BBIBP-CorV) vaccine. Despite initial dismissal as a diabetic complication, symptoms escalated, affecting all extremities. Electromyography study revealed abnormal spontaneous activity with chronic reinnervation changes, which was more significant in the lower extremities. Based on the clinical course, radiographic imaging, and laboratory data, a diagnosis of CIDP with severe axonal demyelinating features was established. Treatment with intravenous immunoglobulin (IVIg), prednisolone, and azathioprine resulted in marked improvement of the upper extremities but limited recovery in distal lower extremity muscles.ConclusionAlthough CIDP is a rare complication following COVID-19 vaccination, it should be considered in the differential diagnosis. Timely diagnosis of vaccine-induced CIDP is challenging, and any delay can adversely affect treatment response in affected patients.

New chronic inflammatory demyelinating polyneuropathy/Guillain-Barré syndrome guidelines - impact on clinical practise.

There is no diagnostic biomarker that can reliably detect Guillain-Barré syndrome (GBS) or chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnosis relies upon integrating key clinical characteristics and relevant supportive data. Consequently, misdiagnosis and delayed diagnosis are common. Diagnostic criteria have proven valuable resources to improve diagnosis, but are underutilized during routine clinical care. In 2021, the EAN/PNS CIDP criteria was published, and were followed by the EAN/PNS GBS criteria in 2023. Both guidelines utilized GRADE methodology to formulate evidence-based recommendations that are intended to be used by adult and paediatric clinicians across diverse care settings to optimize diagnostic accuracy and improve patient outcomes during routine clinical care. The EAN/PNS GBS and CIDP criteria detail specific clinical, electrophysiological, and laboratory features that raise diagnostic confidence, and call attention to diagnostic mimics. The sensitivity of EAN/PNS and other modern criteria to detect GBS and CIDP is high, but utilization during clinical practice is low. Complexity is one factor limiting widespread application. Strategies are needed to optimize criteria adoption during routine clinical care such that GBS and CIDP diagnosis can be achieved with greater speed and accuracy.

Whole-body magnetic resonance neurography in patients with chronic inflammatory demyelinating polyneuropathy.

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. 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. 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. 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.

Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children

Analysis of demographic, clinical, laboratory, electrophysiological and neuroimaging data and pathogenetic therapy of pediatric patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients (n=30) were observed in a separate structural unit of the Russian Children's Clinical Hospital of the Russian National Research Medical University named after. N.I. Pirogova Ministry of Health of the Russian Federation in the period from 2006 to 2023. The examination was carried out in accordance with the recommendations of the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society on the Management of CIDP (2021). All patients received immunotherapy, including intravenous immunoglobulin (IVIG) (n=1), IVIG and glucocorticosteroids (GCS) (n=17, 56.7%), IVIG+GCS+plasmapheresis (n=12, 40.0%). Alternative therapy included cyclophosphamide (n=1), cyclophosphamide followed by mycophenolate mofetil (n=1), rituximab (n=2, 6.6%), azathioprine (n=3), mycophenolate mofetil (n=2, 6.6%). In all patients, there was a significant difference between scores on the MRCss and INCAT functional scales before and after treatment. At the moment, 11/30 (36.6%) patients are in clinical remission and are not receiving pathogenetic therapy. The median duration of remission is 48 months (30-84). The longest remission (84 months) was observed in a patient with the onset of CIDP at the age of 1 year 7 months. Early diagnosis of CIDP is important, since the disease is potentially curable; early administration of pathogenetic therapy provides a long-term favorable prognosis.

Chronic Inflammatory Demyelinating Polyneuropathy and Evaluation of the Visual Evoked Potentials: A Review of the Literature.

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.

Symptom and Treatment Satisfaction in Members of the US and Canadian GBS/CIDP Foundations with a Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy.

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. 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. 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. 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.

Electrodiagnostic profile of conduction slowing in amyotrophic lateral sclerosis

ObjectiveSince motor nerve conduction slowing can occur due to loss of large axons, we investigate the conduction slowing profile in amyotrophic lateral sclerosis (ALS) and identify the limits beyond which the diagnosis of exclusive axonal loss is unlikely. MethodsFirst, using linear regression analysis, we established the range of motor conduction slowing in 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DML), and F-wave latency (F) in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were subsequently validated in 38 additional CIDP patients. Then, the newly established demyelination confidence intervals were used to investigate the profile of conduction slowing in 95 ALS patients. ResultsCV slowing, prolonged DML, and abnormal F were observed in 22.2%, 19.6%, and 47.1% of the studied nerves respectively in ALS patients. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that CMAP amplitude dependent conduction slowing caused by an exclusive loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis. ConclusionsThe presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests the contribution of acquired demyelination or other additional mechanisms exist in the electrodiagnostic profile of ALS.

How I Treat Chronic Inflammatory Demyelinating Polyneuropathy Podcast.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated neuropathy that typically presents with progressive or relapsing, symmetric, proximal, and distal weakness of upper and lower limbs, sensory involvement of at least two limbs, and decreased or absent deep tendon reflexes. The symptoms of CIDP can be similar to those of other neuropathies, making diagnosis difficult, which can often lead to delays in correct diagnosis and treatment. The updated European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 guideline outlines a set of diagnostic criteria that help to identify CIDP with high accuracy and provides recommendations for the treatment of CIDP. The aim of this podcast, featuring Dr. Urvi Desai (Professor of Neurology, Wake Forest School of Medicine and Atrium Health Neurosciences Institute Wake Forest Baptist, Charlotte), is to discuss how the new guideline impacts diagnosis and treatment decisions in her everyday clinical practice. Using a patient case study example, the updated guideline recommends assessing a patient for clinical, electrophysiological, and supportive criteria for CIDP, enabling a more straightforward diagnosis of either typical CIDP, a CIDP variant, or an autoimmune nodopathy. A second patient case study highlights how the new guideline no longer considers autoimmune nodopathies as CIDP, as patients with these disorders do not meet hallmark CIDP criteria. This leaves an unmet need in terms of guidance on how to treat this subset of patients. Although the new guideline has not necessarily changed treatment preference in clinical practice, the addition of subcutaneous immunoglobulin (SCIG) into the guideline now better reflects clinical practice. The guideline helps to define and categorize CIDP more simply and consistently, allowing quicker and more accurate diagnosis, leading to a positive impact on treatment response and prognosis. These real-world insights into the diagnosis and management of patients with CIDP could help guide best clinical practice and help facilitate optimization of patient outcomes.

Motor Nerve Conduction Slowing in Amyotrophic Lateral Sclerosis (P10-8.006)

<h3>Objective:</h3> To determine the range of conduction values for motor nerve conduction slowing and further characterize the distribution of conduction slowing in ALS and characterize the limits of conduction slowing beyond which the diagnosis of ALS is uncertain. <h3>Background:</h3> Abnormal motor nerve conduction studies in ALS are attributed to loss of large axons and distal axonopathy, however conduction slowing can be the result of superimposed demyelinating polyneuropathy or an alternate diagnosis. <h3>Design/Methods:</h3> The electrodiagnostic data of 76 chronic inflammatory demyelinating polyneuropathy (CIDP) patients, identified using AAN research criteria was used to create 12 novel equations using regression analysis that determine a range of the expected slowing of a primary demyelinating polyneuropathy. Demyelinating range confidence intervals were defined by assessing conduction velocity (CV), distal latency (DL), and F latency in relation to distal compound muscle action potential (CMAP) amplitude of median, ulnar, fibular, and tibial nerves. Results were then validated in 38 additional CIDP patients. These developed equations were then used to evaluate conduction slowing in 95 patients with ALS. Transformed CMAP amplitude was used as an independent variable whereas transformed DL, CV, and F were used as dependent variables. <h3>Results:</h3> CV slowing, prolonged DL, and abnormal F latency were observed respectively in 22.2%, 19.6%, and 46.7% of the studied nerves. When slowing occurred, it affected more than one segment of the motor nerve, suggesting that loss of large axons is the main mechanism of slowing. No ALS patient had more than 2 nerves with CV slowing in the confidence interval defined by the regression equations or the American Academy of Neurology (AAN) research criteria for CIDP diagnosis. <h3>Conclusions:</h3> The presence of more than two motor nerves with CV slowing in the demyelinating range defined by the regression analysis or AAN criteria in ALS patients suggests an alternative diagnosis or superimposed demyelination. <b>Disclosure:</b> Mr. Mandava has nothing to disclose. Miss Shaikh has nothing to disclose. Mr. Jaffry has nothing to disclose. Mr. Jaffry has nothing to disclose. Mr. Ors has nothing to disclose. Mr. Trivedi has nothing to disclose. Ms. Faiz has nothing to disclose. Mr. Gandhi has nothing to disclose. Dr. Patel has nothing to disclose. Abu Nasar, MD has nothing to disclose. Ankit Pahwa has nothing to disclose. Dr. Sander has received personal compensation for serving as an employee of Grifols. Dr. Sander has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Grifols. Dr. Sander has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Law firm. Dr. Souayah has received publishing royalties from a publication relating to health care.

Prevalence and associated factors with peripheral neuropathies in the general population in the rural area of Adjohoun in Benin

BackgroundPeripheral neuropathies (PN) are a group of neurological conditions related to damage to the peripheral nervous system. Due to their wide diversity, few studies in sub-Saharan Africa have explored their epidemiology in general population. Our objective was to study the prevalence and associated factors with PN in general population in Adjohoun in Benin. MethodsA cross-sectional study has been conducted from February to March 2019 and included people aged ≥ 18 years old living in Adjohoun, Benin. Following a screening phase for PN (using World Health Organization questionnaire for major neurological diseases), a neurologist made a diagnosis after in-depth clinical examinations completed in some cases by electroneuromyography. The EFNS (European Federation of Neurological Societies) 2010 criteria was used for chronic inflammatory demyelinating polyneuropathy diagnosis. Data such as age, occupation, consanguinity, alcohol consumption, diabetes, hypertension were collected. Association between independent variables and PN were investigated using multivariable logistic regression models. ResultsIn total, 1 655 participants were included, mean age 41.4 ± 16.7 years; 64.8 % are female. The overall prevalence of PN was 6.9 % (95 %CI: 5.7 %-8.2 %). The main types of PN were: polyneuropathies 4.8 % (95 %CI: 3.8 %-5.9 %); polyradiculoneuropathies 1.6 % (95 %CI: 1.0 %-2.2 %). Factors independently associated with PN were age ≥ 40 years (adjusted Odds Ratio aOR = 19.6; 95 %CI: 8.2–46.3), diabetes (aOR = 1.8; 95 %CI: 1.08–2.99) and hypertension (aOR = 1.6; 95 %CI: 1.02–2.5). ConclusionThe prevalence of PN was high in the rural commune of Adjohoun in Benin. Actions on its modifiable associated factors such as diabetes and hypertension could help reduce the proportion of PN and their potential harmful consequences.

Diagnosis of chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic immune-mediated peripheral form of polyneuropathy. No reliable diagnostic biomarkers are available by which to make the diagnosis of CIDP. As a result, diagnosis of the condition can be challenging. Many patients are not recognized early in the disease course, and on the other end of the spectrum both establishing early and accurate diagnosis as well as avoiding misdiagnosis and overtreatment. Identification of the hallmark clinical, electrophysiological, and laboratory features of the disease are critical to facilitate rapid diagnosis, while an understanding of diagnostic pitfalls can help prevent misdiagnosis. Since the original description of CIDP in the 1970s, over 15 sets of diagnostic criteria have been proposed. The criteria published in 2021 by the European Academy of Neurology / Peripheral Nerve Society (EAN/PNS) were developed for use during routine clinical care and are available in the public domain. These criteria provide clinicians with an invaluable resource by which the data collected during the evaluation of the patient with possible CIDP can be interpreted. One point of importance that bridges diagnosis to treatment is objectification of the treatment response. Interpretation of how patients respond to treatment drives both long-term treatment paradigms and the diagnosis at which these treatments are aimed. Although no approach is perfect, utilization of strength impairment and disability outcomes in clinical practice can help unravel the difficulties in interpreting response to treatment. Just as improvement in these outcomes is considered diagnostically supportive, the absence of objective benefit argues against it and should prompt reconsideration of a CIDP diagnosis.

Factors Influencing the Diagnosis of Chronic Inflammatory Demyelinating

BACKGROUND: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a neurological disorder that leads to demyelination of peripheral nerves presenting with an array of symptoms. Symptoms of CIDP include but are not limited to loss of sensation, loss of reflexes, tingling and pain, and weakness. European Federation Neurological Society (EFNS) has developed guidelines for the diagnosis of this disorder. The objective of this study is to look at the relationship between the EFNS diagnostic criteria and whether patients that have the diagnosis of CIDP met this criteria. Data collection was completed on the patients diagnosed with CIDP and then the patients that were diagnosed but did not meet the criteria were analyzed to see what common outliers exist that led to the diagnosis.&#x0D; &#x0D; RESULTS: A total of 20 patients (13 males and 7 females) were included in the study. Eighty-three percent of patients that were correctly diagnosed using the EFNS/PNS guidelines displayed hyporeflexia at the time of their diagnosis. A large majority of the patients (83%) correctly diagnosed using the EFNS/PNS guidelines displayed distal weakness at the time of their diagnosis. At the time of their diagnosis, EMG showed that majority of those who did not meet the EFNS/PNS criteria had no nerves that displayed increased latency. Fifty-eight percent of those who did meet the criteria outlined by the EFNS/PNS guidelines had two or more nerves that presented with increased latency. Testing the velocity of patients displayed that all of the patients that did not meet the EFNS/PNS criteria did not present with nerves that had diminished velocity.&#x0D; &#x0D; CONCLUSION: CIDP misdiagnosis continues to be an issue leading to mismanagement of these patients. This study showed a preference of the clinical component for diagnosis of CIDP even if electrophysiological criteria was not met.

Optimizing chronic inflammatory demyelinating polyneuropathy care with subcutaneous immunoglobulin: The Polyneuropathy and Treatment with Hizentra Open-Label Extension (PATH OLE) study and beyond

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous, acquired autoimmune neurological disorder affecting peripheral nerves. CIDP is characterized by progressive weakness, reduced or absent tendon reflexes and impaired sensory function in the lower and upper limbs. CIDP diagnosis is mainly based on clinical, laboratory and electrophysiologic criteria and there are currently no diagnostic or prognostic biomarkers. First-line treatment options include corticosteroids, intravenous immunoglobulin (IVIg) and plasma exchange (PLEX). While IVIg and corticosteroids are the most common therapies administered for CIDP, there are challenges associated with their use, including systemic adverse events (AEs), some of which can be serious. Studies have shown that subcutaneous immunoglobulin (SCIg) may be associated with improved quality of life, which is attributed partially to the patients’ freedom to administer SCIg at home and at their convenience. While AEs with SCIg mostly consist of local site reactions, SCIg is associated with fewer systemic AEs compared with IVIg, and these are commonly mild, though severe reactions may rarely occur. A number of studies in the last decade have assessed SCIg in CIDP. One of these studies, the Polyneuropathy and Treatment with Hizentra® (PATH) study, was a global phase 3, double-blind, randomized, placebo-controlled trial that assessed the efficacy, safety, and tolerability of SCIg treatment in patients with CIDP. Based on the results of the PATH study, the US Food and Drug Administration (FDA) approved SCIg as a maintenance treatment for CIDP in 2018. This review summarizes and discusses the results of the PATH study and its open-label extension (OLE) study and provides an overview of the April 2021 update to the Hizentra® FDA-approved U.S. package insert based on findings from the PATH OLE. In addition, the review highlights key elements of the second revision of the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guideline for the diagnosis and treatment of CIDP. Finally, this review discusses the characteristics of patients with CIDP who may benefit from SCIg treatment.

COVID-19-Related Burden and Risk Perception in Individuals with Chronic Inflammatory Demyelinating Polyneuropathy and Multifocal Motor Neuropathy: A Cross-Sectional Study.

IntroductionThis study investigated the mental health burden of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) or multifocal motor neuropathy (MMN) during the COVID-19 pandemic in comparison to matched healthy controls.MethodsThe cross-sectional study included 59 patients with a diagnosis of either CIDP or MMN and 59 propensity score matched healthy controls. All participants completed a survey including demographics, distress (distress thermometer), depressive symptoms (PHQ-2), generalized anxiety (GAD-7), COVID-19-related fear, and risk perception. Additionally, patients with CIDP or MMN were asked about the frequency and type of infections since treatment initiation.ResultsPatients with either CIDP or MMN reported experiencing reduced frequency or no differences in infection frequency since immune medication was initiated. Regarding COVID-19, patients with CIDP or MMN rated their risk of infection similar to healthy controls, while they expected a higher probability of the occurrence of symptoms, severe course, and dying of COVID-19. They reported increased depressive symptoms, generalized anxiety, and COVID-19-related fear in comparison to healthy controls.ConclusionDespite their personal experience of reduced frequency of infection since immune medication was initiated, patients with CIDP or MMN reported increased risk perception and prevalence of depressive symptoms, generalized anxiety, and COVID-19-related fear in comparison to healthy controls. This highlights the need for evidence-driven strategies to protect the mental health of this vulnerable group.Supplementary InformationThe online version contains supplementary material available at 10.1007/s40120-022-00359-3.

Clinical and neurophysiological patterns of early presenting symptoms in acute onset chronic inflammatory demyelinating polyradiculoneuropathy

The phenotypes of chronic inflammatory demyelinating polyneuropathy (CIDP) include an acute-onset phenotype (A-CIDP) with an evolution time of less than eight weeks from the onset of symptoms. This entity can be confused with Guillain-Barre syndrome of the acute inflammatory demyelinating variety (AIDP), delaying the start of treatment. To analyze the clinical and electrophysiological differences between A-CIDP, classic CIDP and AIDP, in order to identify factors that may help in the early differential diagnosis. A cross-sectional study was carried out with patients seen at the neuromuscular disease clinic of the National Institute of Neurology and Neurosurgery with a diagnosis of CIDP according to the criteria of the European Federation of Neurological Societies and Peripheral Nerve Society. Patients with CIDP <8 weeks were categorized as A-CIDP and were compared with patients diagnosed with classic CIDP and AIDP. Clinical, paraclinical and electrophysiological variables were obtained and analyzed. Significant differences in history of infection, cranial nerve involvement and dysautonomia were observed between A-CIDP and AIDP. Electrophysiological recordings reported significant differences in motor nerve conduction velocity and sural nerve recordings, being lower in the A-CIDP group. A history of infection, cranial nerve involvement and dysautonomia are important parameters to take into account for the differential diagnosis of these entities. Electrophysiological analysis is similar between A-CIDP and CIDP. The differential diagnosis between these types of demyelinating polyneuropathy must be based on clinical assessment.

Inflammatory Neuropathy Cause and Treatment (INCAT) Scale for the assessment of disability level in patients with chronic inflammatory demyelinating polyneuropathy: linguocultural ratification in Russia

Background. Chronic inflammatory demyelinating polyneuropathy (CIDP) is a treatable dysimmune polyneuropathy. An objective response for pathogenic therapy is essential in diagnosis and management of CIDP. For proper assessment of patient’s complaints and evaluation of disease progression, it is recommended to use validated scales and questionnaires. The paper presents the results of the first step of Inflammatory Neuropathy Cause and Treatment (INCAT) validation in patients with CIDP.Objective: the development of the Russian version of the INCAT scale and its linguocultural ratification.Materials and methods. 15 patients with definite CIDP (according to EFNS/PNS criteria) were enrolled. Linguocultural ratification was conducted according to the standard protocol.Results. The Russian version of the INCAT scale was developed.Conclusion. We conducted the first stage of INCAT scale validation in patients with CIDP.

Is Fecal Calprotectin an Applicable Biomarker of Gut Immune System Activation in Chronic Inflammatory Demyelinating Polyneuropathy? - A Pilot Study.

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a complex autoimmune disease caused by dysregulated response to not fully recognized antigens. Some association between CIDP and inflammatory bowel disease (IBD) has been reported, but the exact pathophysiological links of these disorders are not well understood.Aim of the Study: To evaluate fecal calprotectin as a biomarker of gut inflammation in CIDP patients without IBD.Methods: Fifteen patients with CIDP and 15 healthy controls were included in the study. The CIDP diagnosis was based on the EFNS/PNS criteria. The occurrence of bowel symptoms was assessed based on a questionnaire. The quantitative evaluation of fecal calprotectin level was performed by the ELISA test.Results: The fecal calprotectin level (μg/g) expressed as median along with the lower and upper quartiles [25Q–75Q] was significantly higher in CIDP patients compared to the controls: 26.6 [17.5–109.0] vs 15.6 [7.1–24.1], p = 0.0066. Abnormal fecal calprotectin level (>50 μg/g) was found in 33% of all CIDP patients and in none of the control subjects. The patients with abnormal fecal calprotectin level did not differ from the rest of the study group regarding the neurological status. The most common bowel symptoms reported by CIDP patients included constipation (33%), feeling of incomplete evacuation (33%), bloating (27%), and alternating bowel movement pattern (27%).Conclusion: In one-third of CIDP patients the signs of gut immune system activation have been observed. This finding may be associated with CIDP pathogenesis and induction of autoimmune response as well as concomitant dysautonomia with gastrointestinal symptoms.

Focused Neuromuscular Ultrasound Approach for the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy.

Previous ultrasonographic studies of individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) have shown nerve enlargement at several sites. This prospective study compares only the bilateral median and ulnar nerves of individuals with CIDP with reference values to determine the clinical usefulness of this focused approach as a diagnostic tool. The cross-sectional area, echogenicity, and vascularity of the bilateral median and ulnar nerves of 25 subjects with CIDP were measured using ultrasound. Nineteen had typical CIDP based on the European Federation of Neurological Societies and the Peripheral Nerve Society guidelines, whereas six had atypical CIDP and were diagnosed based on clinical impression. Focal nerve enlargement was found in at least one segment in all subjects. Subjects with typical CIDP had larger cross-sectional areas compared with subjects with atypical CIDP. A focused ultrasound study, involving only the median and ulnar nerves, is sensitive for the detection of nerve enlargement in CIDP. Measuring the cross-sectional area of the median and ulnar nerves is clinically feasible and may help establish the diagnosis of CIDP.