Chronic Inflammatory Demyelinating Polyradiculoneuropathy Patients Research Articles

Prospective open-label trial with rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy not responding to conventional immune therapies

BackgroundTo evaluate the efficacy of rituximab in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients not responding to conventional immune therapies.MethodsAn open-label, prospective exploratory study was conducted with intravenous rituximab on 17...

Cytokines and chemokines in patients with chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy: A systematic review.

Advances in the understanding of cytokines have revolutionized mechanistic treatments for chronic inflammatory and autoimmune diseases, as exemplified by rheumatoid arthritis. We conducted a systematic literature review on the role of cytokines and chemokines in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN). Ovid Medline, EMBASE and Web of Science were searched until August 31, 2022 for human studies investigating cytokines levels in CIDP or MMN. Fifty-five articles on 1061 CIDP patients and 86 MMN patients were included, with a median of 18 patients per study (range 3-71). Studies differed in the inclusion criteria, type of assay, manufacturer, control subjects, and tested biological material. Only a minority of studies reported data on disease activity. Interleukin (IL)-6, IL-17, CXCL10, and tumor necrosis factor alpha (TNF-α), were elevated in CIDP compared to controls in most of the studies. IL-6 and TNF-α levels are also correlated with disability. In MMN patients, IL-1Ra was elevated in the majority of the reports. While acknowledging the challenges in comparing studies and the various limitations of the studies, including small patient numbers, particularly in MMN, our review suggests that IL-6, IL-17, CXCL10, and TNF-α might play a role in CIDP pathogenesis. Larger studies are needed in MMN.

Gait Assessment in Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Background and Aims. Gait impairment is a common manifestation of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). However, clinicians lack an effective monitoring tool, as no gait test has been validated for CIDP. The aim of this study was to determine the usefulness of three tests in monitoring the clinical course of patients with CIDP: Timed Up and Go (TUG), 10-Meter Walk Test (10MWT), and 30-Second Chair Stand (30SCS). Methods. This is a prospective, single-center observational study. We included newly diagnosed CIDP patients starting treatment or relapsed CIDP patients requiring new treatment. We monitored the clinical course using CIDP-validated clinical scales and correlated changes in clinical status with the results of the gait tests. A ROC curve was developed, and we chose the cut-off point on each scale with the best specificity and sensitivity to detect change in clinical status. Results. A total of 20 patients have been recruited. The 3 tests show a statistical correlation with objective clinical improvement. In patients who have showed clinical improvement during the follow-up examination, a mean reduction of 4.8 seconds in TUG and 2.6 in 10MWT and a gain of 3 repetitions in 30SCS have been observed. The optimal cut-off points for each test were TUG≤1 seconds, 10MWT≤1 seconds, and 30SCS≥1 repetition. The TUG test has the highest sensitivity (82.6%), and the 30SCS test has the highest specificity (100%) for detecting clinical improvement. Conclusions. The study found that the TUG and 30SCS tests could become effective tools for monitoring treatment response in CIDP patients.

Chronic inflammatory demyelinating polyneuropathy and HEV antibody status: A case-control study from Lazio, Italy

IntroductionFew studies have pointed to the possible role of infectious diseases in triggering Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP). Given the association of Hepatitis E Virus (HEV) with Guillain Barrè syndrome, we conducted a case-control study to determine the possible association of HEV infection with CIDP, analyzing possible risk factors for acquiring HEV infection in both CIDP patients and controls. Materials and methods82 CIDP and 260 from the general population have provided some personal information (demographics, anamnestic data and recognized risk factors for HEV infection) and underwent venipuncture blood sampling for virological assays testing for anti-HEV IgG and IgM with ELISA and RNA-HEV performing RT-PCR. ResultsAnti-HEV IgG seropositivity resulted in 32 CIDP patients (39.0%) and in 45 controls (17.3%), indicating a significant association between anti-HEV IgG positivity and CIDP (OR 3.04; 95% CI 1.70–5.43, p-value <0.001), but in multivariate logistic regression the only significant associations with anti-HEV positivity were eating pork liver sausages (OR 10.443, 95% CI 2.268–60.12, p-value 0.004) and IVIg/SCIg administration (OR 31.32, 95% CI 7.914–171.7, p-value <0.001). DiscussionThe higher prevalence of anti-HEV IgG in CIDP patients than in controls could be justified by chronically administering IVIg/SCIg with a passive acquisition of anti-HEV antibodies. Furthermore, all the 20 CIDP patients who underwent IVIg/SCIg administration reported HEV risk factors, so that they could have acquired the infection. ConclusionsFurther studies in a larger CIDP patient sample in treatment with therapy other than IVIg/SCIg are necessary to rule out the possible confounding effect of IVIg/SCIg.

Recurrent Guillian Barre’ Syndrome vs acute onset CIDP - study of 2 cases.

The most common acquired immune mediated polyneuropathies are acute inflammatory demyelinating polyradiculoneuropathy(AIDP), a form of Gullian Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy(CIDP). Sixteen percent of CIDP patients may present acutely like AIDP, developing in less than 8 weeks. Also GBS which is usually monophasic can have recurrences . Distinguishing Acute onset CIDP(A-CIDP) and recurrent AIDP may be difficult in early stages but may be crucial to guide the treatment strategies.

Serum Neurofilament and Free Light Chain Levels in Patients Undergoing Treatment for Chronic Inflammatory Demyelinating Polyneuropathy.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder affecting the peripheral nervous system. Despite the established diagnostic criteria, monitoring disease activity and treatment remains challenging. To address this limitation, we investigated serum neurofilament light chain (sNfL) and serum free light chains (sFLCs) as potential biomarkers. A total of 32 CIDP patients undergoing immunoglobulin therapy and 32 healthy controls enrolled in the present study, and agreed to have their blood plasma sNfL and sFLCs analyzed, while CIDP severity was assessed through the modified Rankin Scale (mRS) and the Overall Neuropathy Limitations Scale (ONLS). In line with the immunoglobulin treatment aimed at limiting neuronal damage administered to the majority of patients, sNfL levels did not exhibit significant differences between the two groups. However, CIDP patients showed significantly elevated sFLC and sFLC ratios, while the marker levels did not correlate with the clinical scores. The study confirms the potential of sFLCs as a sensitive biomarker of inflammatory processes in CIDP. Additionally, the present study results regarding neurofilaments strengthen the role of sNfL in monitoring CIDP treatments, confirming the effectiveness of immunoglobulin therapy. Overall, our results demonstrate how combining these markers can lead to better patient characterization for improved treatment.

Quantitative magnetic resonance neurography in chronic inflammatory demyelinating polyradiculoneuropathy: A longitudinal study over 6 years.

To evaluate magnetic resonance neurography (MRN) for the longitudinal assessment of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Prospective examination of twelve CIDP patients by neurological assessment, MRN, and nerve conduction studies in 2016 and 6 years later in 2022. Imaging parameters were compared with matched healthy controls and correlated with clinical and electrophysiological markers. The MRN protocol included T2-weighted imaging, diffusion tensor imaging (DTI), T2 relaxometry, and magnetization transfer imaging (MTI). Nerve cross-sectional area (CSA) was increased in CIDP patients compared to controls (plexus: p = 0.003; sciatic nerve: p < 0.001). Over 6 years, nerve CSA decreased in CIDP patients, most pronounced at the lumbosacral plexus (p = 0.015). Longitudinally, changes in CSA correlated with changes in the inflammatory neuropathy cause and treatment validated overall disability sum score (INCAT/ODSS) (p = 0.006). High initial nerve CSA was inversely correlated with changes in the INCAT/ODSS over 6 years (p < 0.05). The DTI parameter fractional anisotropy (FA) showed robust correlations with electrodiagnostic testing both cross-sectionally and longitudinally (p < 0.05). MTI as a newly added imaging technique revealed a significantly reduced magnetization transfer ratio (MTR) in CIDP patients (p < 0.01), suggesting underlying changes in macromolecular tissue composition, and correlated significantly with electrophysiological parameters of demyelination (p < 0.05). This study provides evidence that changes in nerve CSA and FA reflect the clinical and electrophysiological course of CIDP patients. Initial nerve hypertrophy might predict a rather benign course or better therapy response.

CSF CXCL13 is elevated in patients with CIDP and may reflect higher disease activity

To evaluate B-cell involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 11 patients with CIDP, 8 patients with Guillain–Barré syndrome and 13 patients with idiopathic normal pressure hydrocephalus (iNPH) were studied. CSF cytokine and chemokine (IL-10, IL-15, TNF-α, TGF-β1, GM-CSF, BAFF, CXCL10, and CXCL13) levels were measured by ELISA. The CSF CXCL13 level was significantly higher in patients with CIDP than in those with iNPH. The CSF CXCL13 level was significantly higher in CIDP patients with higher annualized relapse rates and higher modified Rankin scale scores. The CSF CXCL13 level is elevated in CIDP, especially in those with higher disease activity.

The distribution pattern of nerve enlargement in clinical subtypes of chronic inflammatory demyelinating polyneuropathy.

We aim to investigate nerve enlargement patterns and their correlation with clinical subtypes and treatment response using nerve ultrasound in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Between March 2015 and December 2021, 135 CIDP patients were recruited. Nerve ultrasound and electrophysiological studies were performed on the median and ulnar nerves. The responses to intravenous immunoglobulin (IVIg) or prednisone were evaluated with the disability score. There were 99 typical CIDP cases, 10 Lewis-Sumner syndrome (LSS) cases, 15 distal acquired demyelinating symmetric neuropathy (DADS) cases, nine pure motor CIDP cases, and two pure sensory CIDP cases. Sixty (61%) typical CIDP and seven (78%) pure motor CIDP patients had moderately increased or normal cross-sectional area (CSA), and 10 (67%) DADS and seven (70%) LSS patients had significantly increased CSA. The peripheral nerve showed a diffuse enlargement pattern in 46 (51%) typical CIDP, five (50%) LSS, three (25%) DADS, and three (33%) pure motor CIDP patients and a proximal regional enlargement pattern in 11 (12%) typical CIDP, one (10%) LSS, six (50%) DADS, and four (44%) pure motor CIDP patients. Patients with diffusely moderate enlargement patterns and those with proximal regional enlargement showed a higher response rate to glucocorticoids than to IVIg. Various distribution patterns of nerve enlargement existed in CIDP. Although almost all patterns could be detected in each CIDP subtype, diffusely moderate enlargement was more common in typical CIDP and LSS, while proximal regional enlargement was more common in DADS and pure motor CIDP. Different enlargement patterns might indicate different treatment responses.

Access to Immunoglobulin Treatment for CIDP Patients During the COVID-19 Pandemic

Background: Immunoglobulin supplies are limited; their access for patients diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) may have been difficult during the COVID-19 pandemic. Methods: A retrospective cross-sectional study was conducted with CIDP patients (n=16, 68.8% female, mean age 60.4±11.3) recruited from three Montreal tertiary care institutions. Inclusion criteria were patients over 18 years old who were receiving immunoglobulin treatment as of March 1st, 2020. Patients were asked to complete a questionnaire inquiring about changes in their immunoglobulin treatment during the pandemic and about their quality of life. Their charts were reviewed by an independent investigator. We used weighted chi-squared statistical tests and Cramer’s V correlation ratios to measure associations with treatment change. Results: Eighteen months after the pandemic started, 50% of patients were receiving the same treatment, 25% were receiving immunoglobulin treatment at a different frequency, 6.3% were receiving a different dose, 12.5% were receiving a different dose and frequency, and 6.3% were receiving a different treatment. Reasons associated with treatment change were worsening of neurological condition (18.8%; Cramer’s V=0.480; p-value=0.055), improvement of neurological condition (25%; Cramer’s V=0.577; p-value=0.021) and reduced availability of treatment (6.3%; Cramer’s V=0.258; p-value=0.302). There were no significant correlations between lower quality of life (p-value=0.323) or lower Rasch-built Overall Disability Scale score (p-value=0.574) and treatment change.  Conclusion: Difficulty accessing immunoglobulin treatment was infrequent and not significantly associated with treatment change for CIDP patients during the COVID-19 pandemic. A larger multicentre study across multiple sites might identify other treatment access problems resulting from the pandemic.

Serum neurofilament light chain does not detect self-reported treatment-related fluctuations in chronic inflammatory demyelinating polyneuropathy.

Serum neurofilament light chain (sNfL) is a marker for axonal degeneration. Patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often report a fluctuation of symptoms throughout one treatment cycle with intravenous immunoglobulins (IVIG). The aim of this study was to determine whether sNfL is suitable to quantify patient-reported symptom fluctuations. Twenty-nine patients with the diagnosis of CIDP or a CIDP-variant under treatment with IVIG were recruited in this study and underwent examination before IVIG infusion, in the middle of the treatment interval, and before their next IVIG infusion. Patients were surveyed regarding symptom fluctuations at the last visit and divided into two groups: those with and without fluctuations of symptoms. At the first visit, sociodemographic and disease-specific data were collected. Clinical scores were assessed at every examination. sNfL values were compared between both groups at the different time points after conversion into Z-scores-adjusted for age and body mass index. Patients with CIDP show elevated sNfL Z-scores (median at baseline: 2.14, IQR: 1.0). There was no significant change in sNfL Z-scores or questionnaire scores within the treatment cycle in either group. There was no significant difference in sNfL levels between the patients with and without symptom fluctuations. CIDP patients show elevated sNfL levels. However, sNfL is not suitable to reflect patient-reported fluctuations of symptoms. This indicates that symptom fluctuations during treatment with IVIG in patients with CIDP are not caused by a neuroaxonal injury. Furthermore, repeated sNfL measurements within one treatment cycle with IVIG seem to have no benefit for symptom monitoring.

The diagnostic value of quantitative assessment of MR neurography in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis.

The purpose of this study was to evaluate the diagnostic value of quantitative magnetic resonance neurography (MRN) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We also compared various MRN parameters and determined the best performing one. Through literature searches in PubMed, Embase, Cochrane, Ovid MEDLINE and ClinicalTtrials.gov until March 1, 2023, we selected studies with the diagnostic performance of MRN in CIDP patients. The pooled estimated sensitivity and specificity of quantitative MRN parameters were determined by a bivariate random-effects model. Subgroup analysis was performed to evaluate the proper quantitative parameters and nerve sites. A total of 14 quantitative MRN studies with 23 results gave a pooled sensitivity of 0.73 (95% CI 0.66-0.79) and a pooled specificity of 0.89 (95% CI 0.84-0.92). The area under the curve (AUC) was 0.89 (95%CI 0.86-0.92). Subgroup analysis of quantitative parameters showed the fractional anisotropy (FA) with the highest sensitivity of 0.85 (95% CI 0.77-0.90) and cross-sectional area (CSA) with the highest specificity of 0.95 (95% CI 0.85-0.99). The pooled correlation coefficient for interobserver agreements was 0.90 (95%CI 0.82-0.95). Quantitative MRN has considerable diagnostic value in CIDP patients with accuracy and reliability. FA and CSA can be promising parameters in the future diagnosis of CIDP patients. This is the first meta-analysis of quantitative MRN in the diagnosis of CIDP.We have selected reliable parameters with cut-off value and provided new insights for subsequent diagnosis of CIDP.

P.041 Guillain Barre syndrome could be a rare presenting finding of nodal and paranodal autoantibodies in immune-mediated neuropathies (IMN): A clinical utility of Cell based Assay

Background: Guillain Barre Syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are the two most common forms of treatable IMNs. Antibodies targeting proteins at paranodal cell-adhesion molecules such as contactin-1 (CNTN1), neurofascin-155 (NF155), contactin-associated protein 1 (CASPR1), and nodal neurofascins-NF140 and NF186, have been discovered in CIDP patients. Methods: Between August 2021 and January 2023, at BC Neuroimmunology laboratory, Vancouver we screened a total of 214 sera of patients for detecting nodal and paranodal antibodies with a fixed CBA. These patient sera were assayed for the presence of NF140, NF155, NF186, CNTN1, plus Caspr1 antibodies. The final diagnosis and response to therapy of positive cases were evaluated by a questionnaire requested from their physicians. Results: 10 cases were positive for nodal/paranodal antibodies by CBA (mean age 52.4 ± 15.4 years). Two cases were NF155 Ab positive CIDP with good response to conventional therapies. Three cases were double positive for NF140 and 186 Abs, three were double positive for CNTN1 and CASPR1 Abs. Interestingly, two cases were triple positive with GBS presentation. Conclusions: We identified a subgroup of nine patients with CIDP nodal and paranodal antibodies. Among them, two cases had triple positive antibodies with GBS presentation and poor response to plasma exchange and IVIg.

Presence of deep tendon reflexes may not exclude a diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) (P11-8.015)

<h3>Objective:</h3> To identify if there are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients who do not meet clinical criteria but satisfy electrodiagnostic criteria. Our hypothesis is that these patients do not differ from patients who meet criteria for CIDP and possible CIDP. <h3>Background:</h3> The diagnosis of CIDP remains challenging due to lack of a biomarker or a single diagnostic test. The European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) 2021 revision of the diagnostic criteria for CIDP advocate a stepwise approach to the diagnosis of CIDP which requires clinical criteria including absent or reduced deep tendon reflexes before the application of electrodiagnostic criteria. <h3>Design/Methods:</h3> We applied the 2021 EAN/PNS criteria to our cohort of 39 patients receiving intravenous immunoglobulin (IVIG) for CIDP. We compared clinical and electrodiagnostic criteria between those who fulfilled electrodiagnostic criteria but not clinical criteria and patients with CIDP or possible CIDP <h3>Results:</h3> Of our cohort 39-patients, 21 (53.8%) were classified as CIDP, 6 (15.3%) as possible CIDP and 12 patients didn’t meet criteria. Of the 10 patients who didn’t meet clinical criteria, 6/10 (60%) met electrodiagnostic criteria. These patients did not qualify as CIDP or Possible CIDP due to the presence of reflexes. There were no significant differences in the clinical phenotypes and demographics between these patients and CIDP or possible CIDP patients in our cohort. There was also no significant difference in the electrodiagnostic parameters between these patients and those with CIDP or possible CIDP. However, the patients with preserved reflexes tended to have more frequent conduction velocity slowing (p = 0.08) than patients with CIDP or possible CIDP. <h3>Conclusions:</h3> The presence of reflexes should not exclude the diagnosis of CIDP if the patient meets electrodiagnostic criteria. This could be indicative of a subset of patients with CIDP who have more involvement of the intermediate segments of nerve rather than proximal segments. <b>Disclosure:</b> Dr. Strathdee has nothing to disclose. The institution of Dr. Rodrigues has received research support from Australian Brain Foundation. The institution of Dr. Kapoor has received research support from Bethlehem Griffiths Research Foundation . Dr. Zhang has nothing to disclose. Dr. Faragher has stock in Argo P/L. Dr. Faragher has stock in Australian Foundation P/L. Dr. Faragher has stock in Argo Infrastructure P/L. Bruce Day has nothing to disclose. The institution of Dr. Hutton has received research support from Lundbeck. The institution of Dr. Hutton has received research support from Daewoong Pharmaceuticals . The institution of Dr. Hutton has received research support from Xalud. The institution of Dr. Hutton has received research support from Ipsen. The institution of Dr. Hutton has received research support from Abbvie. The institution of Dr. Hutton has received research support from Cerecin. Dr. Hutton has a non-compensated relationship as a Board Member with Brain Foundation (Vic) that is relevant to AAN interests or activities. Dr. Hutton has a non-compensated relationship as a President-elect with Australian and New Zealand Headache Society that is relevant to AAN interests or activities. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lundbeck. Dr. Stark has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Dr. Stark has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lilly. Dr. Stark has stock in CSL. Dr. Stark has stock in Multiple ASX listed (non-medical) companies. Dr. Stark has received personal compensation in the range of $10,000-$49,999 for serving as a TCMC Committee chair with AMA Victoria.

Risk of disease relapse, safety and tolerability of SARS-CoV-2 vaccination in patients with chronic inflammatory neuropathies.

The aim was to evaluate the risk of relapse after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, and its safety and tolerability, in patients with chronic inflammatory neuropathies. In this multicenter, cohort and case-crossover study, the risk of relapse associated with SARS-CoV-2 vaccination was assessed by comparing the frequency of relapse in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) patients who underwent or did not undergo vaccination. Frequency of relapse in the 3 months prior to and after vaccination, and safety and tolerability of SARS-CoV-2 vaccination, were also assessed. In all, 336 patients were included (278 CIDP, 58 MMN). Three hundred and seven (91%) patients underwent SARS-CoV-2 vaccination. Twenty-nine patients (9%) did not undergo vaccination. Mild and transient relapses were observed in 16 (5%) patients (13 CIDP, 3 MMN) after SARS-CoV-2 vaccination and in none of the patients who did not undergo vaccination (relative risk [RR] 3.21, 95% confidence interval [CI] 0.19-52.25). There was no increase in the specific risk of relapse associated with type of vaccine or diagnosis. Comparison with the 3-month control period preceding vaccination revealed an increased risk of relapse after vaccination (RR 4.00, 95% CI 1.35-11.82), which was restricted to CIDP patients (RR 3.25, 95% CI 1.07-9.84). The safety profile of SARS-CoV-2 vaccination was characterized by short-term, mild-to-moderate local and systemic adverse events. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in CIDP and MMN patients does not seem to be associated with an increased risk of relapse at the primary end-point, although a slightly increased risk in CIDP patients was found compared to the 3 months before vaccination.

Chunky yarn sign: A screening marker for brachial plexus hypertrophy on axial cervical spine MRI

AbstractBackgroundNerve hypertrophy is one of the characteristic features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The brachial plexus runs along the gap between the anterior and medial scalene muscles (interscalene gap), and its enlargement sometimes appears as widening of the interscalene gap on conventional axial cervical spine MRI.MethodsTo determine whether the screening of nerve hypertrophy in CIDP is available via conventional cervical spine MRI, we reviewed the medical records of 29 patients with CIDP who underwent cervical MRI, and imaging features were compared with 50 patients with cervical spondylosis who underwent nerve conduction study and were confirmed to have no neuropathies. The maximum diameter of the interscalene gap at the 6th or 7th cervical vertebra level on axial‐slice T2 weighted imaging was measured by two observers independently.ResultsThe maximum diameter of the interscalene gap was significantly larger in the CIDP patients (range, 1.7–11.58; median, 3.64 mm; interquartile range [IQR], 1.79) than in the cervical spondylosis patients (range, 0.87–4.71; median, 1.96 mm; IQR, 0.88) (p &lt; 0.001).ConclusionThe enlarged interscalene gaps were analogous to chunky yarn, with a continuous thick structure along the gaps. Attention to interscalene gaps on routine MRI can contribute to the screening of hypertrophic neuropathies without additional costs or procedures.

CIDP/autoimmune nodopathies with nephropathy: a case series study.

The co-morbidity of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)/autoimmune nodopathies with nephropathy has been gradually known in recent years. This study was intended to explore the clinical, serological and neuropathological features of seven patients with CIDP/autoimmune nodopathies and nephropathy. Among 83 CIDP patients, seven were identified with nephropathy. Their clinical, electrophysiological and laboratory examination data were collected. The nodal/paranodal antibodies were tested. The sural biopsies were performed in all the patients, and renal biopsies were operated in 6 patients. Six patients had chronic onsets and one had an acute onset. Four patients exhibited peripheral neuropathy preceding nephropathy while two showed concurrent onset of neuropathy and nephropathy, and one started with nephropathy. All the patients showed demyelination in electrophysiological examination. Nerve biopsies showed mild to moderate mixed neuropathies including demyelinating and axonal changes in all patients. Renal biopsies showed membranous nephropathy in all 6 patients. Immunotherapy was effective in all patients, with two patients showing good response to corticosteroid treatment alone. Four of the patients were positive to anti-CNTN1 antibody. Compared with anti-CNTN1 antibody-negative patients, antibody-positive patients had a higher proportion of ataxia (3/4 vs. 1/3), autonomic dysfunction (3/4 vs. 1/3), less frequent antecedent infections (1/4 vs. 2/3), higher cerebrospinal fluid proteins (3.2g/L vs. 1.69 g/L), more frequent conduction block on electrophysiological examination (3/4 vs. 1/3), higher myelinated nerve fiber density, and positive CNTN1 expression in the glomeruli of kidney tissues. Anti-CNTN1 antibody was the most frequent antibody in this group of patients with CIDP/autoimmune nodopathies and nephropathy. Our study suggested that there might be some clinical and pathological differences between the antibody positive and negative patients.

Parameters Associated with the Required Drug Dose of Intravenous Immunoglobulin in Stable Chronic Inflammatory Demyelinating Polyradiculoneuropathy

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. 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. 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. 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.

Repurposing MS immunotherapies for CIDP and other autoimmune neuropathies: unfulfilled promise or efficient strategy?

Despite advances in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and other common autoimmune neuropathies (AN), still-many patients with these diseases do not respond satisfactorily to the available treatments. Repurposing of disease-modifying therapies (DMTs) from other autoimmune conditions, particularly multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD), is a promising strategy that may accelerate the establishment of novel treatment choices for AN. This approach appears attractive due to homologies in the pathogenesis of these diseases and the extensive post-marketing experience that has been gathered from treating MS and NMOSD patients. The idea is also strengthened by a number of studies that explored the efficacy of DMTs in animal models of AN but also in some CIDP patients. We here review the available preclinical and clinical data of approved MS therapeutics in terms of their applicability to AN, especially CIDP. Promising therapeutic approaches appear to be B cell-directed and complement-targeting strategies, such as anti-CD20/anti-CD19 agents, Bruton's tyrosine kinase inhibitors and anti-C5 agents, as they exert their effects in the periphery. This is a major advantage because, in contrast to MS, their action in the periphery is sufficient to exert significant immunomodulation.

Compound muscle action potential duration ratio for differentiation between Charcot-Marie-Tooth disease and CIDP

ObjectiveTo elucidate the utility of the proximal to distal compound muscle action potential (CMAP) duration ratio to distinguish between demyelinating Charcot-Marie-Tooth disease (CMT) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) compared with nerve ultrasound. MethodsThirty-nine demyelinating CMT patients and 19 CIDP patients underwent nerve conduction studies (NCS) and nerve ultrasound. NCS parameters including CMAP duration ratio calculated by dividing the value at the proximal site by that at the distal site and nerve cross-sectional area (CSA) measured by ultrasound were compared between the two groups. The diagnostic sensitivity and specificity of each parameter were analysed. ResultsCMT patients showed a significantly lower CMAP duration ratio than CIDP patients (p < 0.05). The area under the curve (AUC) value of the CMAP duration ratio exceeded 0.95 when CMT was considered “positive”, and a cut-off value of 1.13 resulted in high diagnostic sensitivity and specificity (84.6 and 100 % for median nerve, 97.4 and 85.7 % for ulnar nerve, respectively), whereas the AUC value of nerve CSA ranged from 0.70 to 0.81. ConclusionsThe CMAP duration ratio could effectively distinguish between demyelinating CMT and CIDP. SignificanceAdding the CMAP duration ratio to a routine NCS may improve the accuracy of the diagnosis of demyelinating CMT.