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Abstract Background Severe 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 presentation A 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. Conclusion Although 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.

Background: Severe 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 presentation: A 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. Conclusion: Although 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. [ABSTRACT FROM AUTHOR]

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.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nerves that presents with either chronic progression or relapsing disease. Recent studies in samples from patients with CIDP and mouse models have delineated how defects in central (thymic) and peripheral (extrathymic) immune tolerance mechanisms can cause PNS autoimmunity. Notably, nerve parenchymal cells actively contribute to local autoimmunity and also control disease outcome. Here, we outline how emerging technologies increasingly enable an integrated view of how immune cells and PNS parenchymal cells communicate in CIDP. We also relate the known heterogeneity of clinical presentation with specific underlying mechanisms. For example, a severe subtype of CIDP with tremor is associated with pathogenic IgG4 autoantibodies against nodal and paranodal proteins. An improved understanding of pathogenic mechanisms in CIDP will form the basis for more effective mechanism-based therapies.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy, rarely reported as being associated with systemic lupus erythematous (SLE). We report the case of 53-year-old women with a long history of SLE, diagnosed with CIDP during a lupus flare beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. She received treatment with steroids, intravenous immunoglobulins and anifrolumab and showed clinical and immunological improvement. It is important to take into account the presence of peripheral neurological disorders in patients with SLE, considering CIDP as an uncommon manifestation. Controlling severe active disease is an imperative issue to manage this neurological manifestation which has a serious impact on health-related quality of life and physical function. [ABSTRACT FROM AUTHOR]

Chronic inflammatory demyelinating polyneuropathy (CIDP) and Guillain-Barre syndrome (GBS) are inflammatory neuropathies that affect humans and are characterized by peripheral nerve myelin destruction and macrophage-containing immune infiltrates. In contrast to the traditional view that the peripheral nerve is simply the target of autoimmunity, we report here that peripheral nerve Schwann cells exacerbate the autoimmune process through extracellular matrix (ECM) protein induction. In a spontaneous autoimmune peripheral polyneuropathy (SAPP) mouse model of inflammatory neuropathy and CIDP nerve biopsies, the ECM protein periostin (POSTN) was upregulated in affected sciatic nerves and was primarily expressed by Schwann cells. Postn deficiency delayed the onset and reduced the extent of neuropathy, as well as decreased the number of macrophages infiltrating the sciatic nerve. In an in vitro assay, POSTN promoted macrophage chemotaxis in an integrin-AM (ITGAM) and ITGAV-dependent manner. The PNS-infiltrating macrophages in SAPP-affected nerves were pathogenic, since depletion of macrophages protected against the development of neuropathy. Our findings show that Schwann cells promote macrophage infiltration by upregulating Postn and suggest that POSTN is a novel target for the treatment of macrophage-associated inflammatory neuropathies.

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and challenging demyelinating disorder. It is necessary to increase our understanding of potential connections between imaging, electromyography, and clinical characteristics. Objective: The aim of this study was to evaluate the relationships between multisequence magnetic resonance neurography (MRN) findings, electrophysiological parameters, and clinical characteristics in CIDP patients. Design: A cross-sectional study. Methods: Overall, 51 CIDP patients underwent MRN of the brachial and lumbosacral plexus, and nerve conduction studies. The inflammatory Rasch-built overall disability scale (I-RODS) questionnaire, CIDP disease activity status (CADS) scale, and muscle strength scores were evaluated by two neurologists. Electrophysiological parameters, clinical information, and multiparameter-MRN were analyzed for correlations. Multiparameter-MRN includes diameter, nerve-to-muscle T2 signal intensity ratio (nT2), contrast-enhanced ratio (CR), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) of bilateral plexus nerve roots. Results: Electrophysiological parameters that were not elicited were significantly higher in the lower extremities than in the upper extremities, and those were higher in sensory nerve conduction than in motor. There were moderate correlations between motor nerve conduction velocity and distal motor latency in nerve diameter, nT2, FA, and ADC, respectively (| r |, 0.45–0.64, p < 0.05). The correlations between CR and sensory nerve conduction velocity and peak latency were moderate, and ADC had a positive correlation with compound motor action potential amplitude (| r |, 0.45–0.63). FA correlated negatively with the course (r = −0.62) and cerebrospinal fluid (CSF) protein (r = −0.41), whereas ADC had correlated positively with CSF protein (r = 0.34). Only CR had a moderately negative correlation with CADS (r's = −0.57). Muscle strength in all extremities was positively correlated with FA (r 's range, 0.41–0.49). There was no significant correlation between I-RODS scores and multiparameter-MR. Conclusion: MRN-derived multiparameter [nerve size, nT2, and diffusion tensor imaging (DTI) parameters] could serve as quantitative biomarkers of myelin sheath integrity in CIDP. DTI parameters and CR correlated with clinical characteristics better than morphological parameters-MR for CIDP patients. [ABSTRACT FROM AUTHOR]

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and challenging demyelinating disorder. It is necessary to increase our understanding of potential connections between imaging, electromyography, and clinical characteristics. Objective: The aim of this study was to evaluate the relationships between multisequence magnetic resonance neurography (MRN) findings, electrophysiological parameters, and clinical characteristics in CIDP patients. Design: A cross-sectional study. Methods: Overall, 51 CIDP patients underwent MRN of the brachial and lumbosacral plexus, and nerve conduction studies. The inflammatory Rasch-built overall disability scale (I-RODS) questionnaire, CIDP disease activity status (CADS) scale, and muscle strength scores were evaluated by two neurologists. Electrophysiological parameters, clinical information, and multiparameter-MRN were analyzed for correlations. Multiparameter-MRN includes diameter, nerve-to-muscle T2 signal intensity ratio (nT2), contrast-enhanced ratio (CR), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) of bilateral plexus nerve roots. Results: Electrophysiological parameters that were not elicited were significantly higher in the lower extremities than in the upper extremities, and those were higher in sensory nerve conduction than in motor. There were moderate correlations between motor nerve conduction velocity and distal motor latency in nerve diameter, nT2, FA, and ADC, respectively (|r|, 0.45--0.64, p < 0.05). The correlations between CR and sensory nerve conduction velocity and peak latency were moderate, and ADC had a positive correlation with compound motor action potential amplitude (|r|, 0.45--0.63). FA correlated negatively with the course (r = -0.62) and cerebrospinal fluid (CSF) protein (r = -0.41), whereas ADC had correlated positively with CSF protein (r = 0.34). Only CR had a moderately negative correlation with CADS (r's = -0.57). Muscle strength in all extremities was positively correlated with FA (r's range, 0.41-0.49). There was no significant correlation between I-RODS scores and multiparameter-MR. Conclusion: MRN-derived multiparameter [nerve size, nT2, and diffusion tensor imaging (DTI) parameters] could serve as quantitative biomarkers of myelin sheath integrity in CIDP. DTI parameters and CR correlated with clinical characteristics better than morphological parameters-MR for CIDP patients. [ABSTRACT FROM AUTHOR]

Introduction/Aims: A model for predicting responsiveness to immunotherapy in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) has not been well established. We aimed to establish a new classifier for CIDP patients based on clinical characteristics, laboratory findings, and electrophysiological features. Methods: The clinical, laboratory, and electrophysiological features of 172 treatment‐naïve patients with CIDP between 2003 and 2019 were analyzed using an unsupervised hierarchical clustering. The identified pivotal features were used to establish simple classifications using a tree‐based model. Results: Three clusters were identified: 1, n = 65; 2, n = 70; and 3, n = 37. Patients in Cluster 1 scored lower on the disability assessment score before treatment. More patients in Clusters 2 (90.0%) fulfilled demyelinating criteria than patients in Cluster 1 (30.8%, p <.001). Cluster 3 had more patients with chronic kidney disease (CKD) (27.0%) and hypoalbuminemia (3.40 g/dL) than did Cluster 2 (CKD: 0%, p <.001; hypoalbuminemia: 4.09 g/dL, p <.001). The responsiveness to pulse steroid therapy was higher in Cluster 2 (70.0%) than in Clusters 1 (31.8%; p =.043) and 3 (25.0%; p =.014). A tree‐based model with four pivotal features classified patients in our cohort into new clusters with high accuracy (89.5%). Discussion: The established hierarchical clustering with the tree‐based model identified key features contributing to differences in disease severity and response to pulse steroid therapy. This classification system could assist clinicians in the selection of treatments and could also help researchers by clustering patients for clinical treatment trials. [ABSTRACT FROM AUTHOR]

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Objective To summarize the clinical, laboratory, imaging, electrophysiological and histopathological features and therapeutic responses of patients with paranodopathy with positive anti - neurofascin 155 (NF155) antibody. Methods and Results Five patients who visited Xuanwu Hospital, Capital Medical University in January 2017 to August 2020 with positive anti -NF155 antibody, were men with onset ages 10 to 61 years old. The clinical manifestations were subacute onset, gradual progress, distal limb weakness, numbness, tremor and ataxia. The distal limbs were more severe than the proximal and lower limbs were more severe than the upper. Five patients were positive NF155-IgG4. Cerebrospinal fluid protein was significant increased. There were cervical and lumbosacral nerve roots enlargement. The distal latency of compound muscle action potential (CMAP) prolonged, the conduction velocity decreased, and the F -wave latency extended. Sural nerve biopsy showed axonal degeneration, the Ranvier node widened, the transverse band disappeared, and the space between the myelin loop and axon increased without inflammatory cell infiltration and "onion bulb" structure. Glucocorticoid therapy and intravenous immunoglobulin (IVIg) were not effective or initially effective, then failed, while rituximab was effective. Conclusions Patients with positive anti -NF155 antibody paranodopathy showed heterogeneity in clinical characteristics and treatment. Early stage should do antibody examination and diagnosis. Early treatment could prevent myelin and axonal injury and preserve nerve function. [ABSTRACT FROM AUTHOR]

Background and Objectives: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered an immune-mediated heterogeneous disease that involves both cellular and humoral immunity. The advent of the new concept of node-paranodopathy in recent years has boosted the identification of more antibody-positive CIDP variants patients. Cases of Caspr1 autoantibodies are the least common. Here, we reported two patients with Caspr1 autoantibodies and summarized their clinical features and treatment responses. Methods: Do statistical analyses on the clinical manifestations and laboratory examinations obtained from two patients identified in this study, and eight patients with anti-Caspr1 antibodies reported in previous research. And based on the developed scoring standard, draw the radar charts and line graphs. Results: Similar to other studies, the two patients we mentioned had a subacute and severe onset, distal phenotype, sensory ataxia, and severe pain. Differently, they had severe pain accompanying cold sense and coarse tremor in both hands, which may be a typical symptom for the anti-Caspr1 positive patient in south China. And we drew the line and radar graph for two China patients based on five aspects, muscle strength, sensory nerve, cranial nerve, laboratory tests, and NCS examinations. The two visual data charts offered new complementary means for the diagnostic assessment of CIDP variants. Conclusion: Pain with cold sense, coarse tremor in hands, and CSF protein levels greater than 3g/L may be the source of the distinct symptoms observed in patients with anti-Caspr1 autoantibodies in south China. [ABSTRACT FROM AUTHOR]

Background and ObjectivesChronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered an immune-mediated heterogeneous disease that involves both cellular and humoral immunity. The advent of the new concept of node-paranodopathy in recent years has boosted the identification of more antibody-positive CIDP variants patients. Cases of Caspr1 autoantibodies are the least common. Here, we reported two patients with Caspr1 autoantibodies and summarized their clinical features and treatment responses.MethodsDo statistical analyses on the clinical manifestations and laboratory examinations obtained from two patients identified in this study, and eight patients with anti-Caspr1 antibodies reported in previous research. And based on the developed scoring standard, draw the radar charts and line graphs.ResultsSimilar to other studies, the two patients we mentioned had a subacute and severe onset, distal phenotype, sensory ataxia, and severe pain. Differently, they had severe pain accompanying cold sense and coarse tremor in both hands, which may be a typical symptom for the anti-Caspr1 positive patient in south China. And we drew the line and radar graph for two China patients based on five aspects, muscle strength, sensory nerve, cranial nerve, laboratory tests, and NCS examinations. The two visual data charts offered new complementary means for the diagnostic assessment of CIDP variants.ConclusionPain with cold sense, coarse tremor in hands, and CSF protein levels greater than 3g/L may be the source of the distinct symptoms observed in patients with anti-Caspr1 autoantibodies in south China.

IntroductionSarcoid polyneuropathy is a rare and clinically heterogeneous disorder that may be the initial presentation of sarcoidosis.MethodsWe report the clinical, electrophysiological, and pathological findings of a patient who carried a diagnosis of sensory-predominant chronic inflammatory demyelinating polyneuropathy (CIDP) for over a decade but was ultimately found to have sarcoid polyneuropathy.ResultsA 36-year-old man presented with a several-week history of gait difficulty and muscle cramps. He had a diagnosis of CIDP but had not received lasting benefit from steroid-sparing immunosuppressive drugs. Electrodiagnostic studies were consistent with a chronic demyelinating polyradiculoneuropathy with conduction blocks. After he developed systemic symptoms, tissue biopsies revealed granulomatous disease. Symptoms improved with steroid therapy.ConclusionsSarcoid polyneuropathy presents a diagnostic challenge, but, in patients with atypical neuropathy, characteristic systemic symptoms, or a poor response to standard treatment, nerve and muscle biopsies can help diagnose this treatable disorder.

Currently, there is growing evidence in the literature warning of misdiagnosis involving amyloidosis and chronic inflammatory demyelinating polyneuropathy (CIDP). Although inducing clinical manifestations outside the peripheral nervous system, light chain and transthyretin amyloidosis may initially present with peripheral neuropathy, which can be indistinguishable from CIDP, leading to a delay in the correct diagnosis. Besides, the precise identification of the amyloid subtype is often challenging. This case report exemplifies clinical and laboratory pitfalls in diagnosing amyloidosis and subtyping amyloid, exposing the patient to potentially harmful procedures

Background and PurposeChronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon demyelinating disorder. Although treatable, it is difficult to diagnose. The purpose of this study was to evaluate the diagnostic performance and abnormalities of plexus via quantitative multisequence magnetic resonance neurography (MRN) for CIDP.MethodsBrachial and lumbosacral (LS) plexus of 37 CIDP patients and 37 age- and gender-matched controls were examined by using multisequence MRN. Nerve diameter, nerve-to-muscle T2 signal intensity ratio (nT2), contrast-enhanced ratio (CR), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) were determined in both plexus, and tractographies were performed. The disease histories and the Inflammatory Rasch-built Overall Disability Scale (I-RODS) questionnaire scores were documented before MRI scans.ResultsThe sizes of nerve roots were larger in CIDP (p < 0.01). CR, nT2, and ADC were significantly higher, while FA was lower in CIDP than in controls (p < 0.01). FA had the highest sensitivity (0.809) and area under the curve (AUC) (0.925), while the highest specificity was 0.961 for CR in single parameters. The combination of FA and CR has the highest sensitivity, specificity, accuracy, and AUC in the LS plexus. CR only had a weak correlation with nT2 (p < 0.05). ADC and diameter had a positive correlation with nT2, and the diameter and nT2 had a negative correlation with FA in CIDP (p < 0.05). FA had a negative correlation with the duration in the CIDP (r’s = −0.404, p < 0.05). There was no significant correlation between the I-RODS scores and MR multiparameters (p < 0.05).ConclusionMultisequence MRN possesses a high diagnostic performance in the LS plexus. Sampling perfection with application-optimized contrasts using different flip angle evolutions (SPACE) combined with DTI and contrast enhancement serves as a recommended composite protocol for CIDP.

Background and Purpose: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon demyelinating disorder. Although treatable, it is difficult to diagnose. The purpose of this study was to evaluate the diagnostic performance and abnormalities of plexus via quantitative multisequence magnetic resonance neurography (MRN) for CIDP. Methods: Brachial and lumbosacral (LS) plexus of 37 CIDP patients and 37 age- and gender-matched controls were examined by using multisequence MRN. Nerve diameter, nerve-to-muscle T2 signal intensity ratio (nT2), contrast-enhanced ratio (CR), fractional anisotropy (FA), and apparent diffusion coefficient (ADC) were determined in both plexus, and tractographies were performed. The disease histories and the Inflammatory Rasch-built Overall Disability Scale (I-RODS) questionnaire scores were documented before MRI scans. Results: The sizes of nerve roots were larger in CIDP (p < 0.01). CR, nT2, and ADC were significantly higher, while FA was lower in CIDP than in controls (p < 0.01). FA had the highest sensitivity (0.809) and area under the curve (AUC) (0.925), while the highest specificity was 0.961 for CR in single parameters. The combination of FA and CR has the highest sensitivity, specificity, accuracy, and AUC in the LS plexus. CR only had a weak correlation with nT2 (p < 0.05). ADC and diameter had a positive correlation with nT2, and the diameter and nT2 had a negative correlation with FA in CIDP (p < 0.05). FA had a negative correlation with the duration in the CIDP (r 's = −0.404, p < 0.05). There was no significant correlation between the I-RODS scores and MR multiparameters (p < 0.05). Conclusion: Multisequence MRN possesses a high diagnostic performance in the LS plexus. Sampling perfection with application-optimized contrasts using different flip angle evolutions (SPACE) combined with DTI and contrast enhancement serves as a recommended composite protocol for CIDP. [ABSTRACT FROM AUTHOR]

Background: Peripheral nerve ultrasound (US) has been used as a promising diagnosing technique for peripheral nerve disorders. This study aimed to compare the US findings of Guillain-Barre syndrome (GBS) with chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: This case-control study was done on 25 patients with GBS at 3 weeks after onset of disease and 25 patients with CIDP. Demographic information and US results of median nerve at 2 points, ulnar nerve at 3 points, and tibial and peroneal nerves were collected. Results: Left median nerve diameter in patients with CIDP with the mean of 0.141 ± 0.047 was more than GBS group with the mean of 0.095 ± 0.034 (P < 0.001). Both sides of median nerve diameter in patients with CIDP were higher than patients with GBS (P < 0.050), but in the left side, it was more in patients with CIDP (P = 0.003). Conclusion: The diameter and circumference of median, ulnar, and tibial nerves in forearm and elbow of patients with CIDP are more than patients with GBS; therefore, it may be possible to use US findings based on these differences in diagnosis and differentiation of the two diseases. [ABSTRACT FROM AUTHOR]

Background and purpose: High peak serum immunoglobulin G (IgG) levels may not be needed for maintenance intravenous immunoglobulin (IVIg) treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and such high levels may cause side effects. More frequent lower dosing may lead to more stable IgG levels and higher trough levels, which might improve efficacy. The aim of this trial is to investigate whether high frequent low dosage IVIg treatment is more effective than low frequent high dosage IVIg treatment. Methods: In this randomized placebo‐controlled crossover trial, we included patients with CIDP proven to be IVIg‐dependent and receiving an individually established stable dose and interval of IVIg maintenance treatment. In the control arm, patients received their individual IVIg dose and interval followed by a placebo infusion at half the interval. In the intervention arm, patients received half their individual dose at half the interval. After a wash‐out phase patients crossed over. The primary outcome measure was handgrip strength (assessed using a Martin Vigorimeter). Secondary outcome indicators were health‐related quality of life (36‐item Short‐Form Health Survey), disability (Inflammatory Rasch‐built Overall Disability Scale), fatigue (Rasch‐built Fatigue Severity Scale) and side effects. Results: Twenty‐five patients were included and were treated at baseline with individually adjusted dosages of IVIg ranging from 20 to 80 g and intervals ranging from 14 to 35 days. Three participants did not complete the trial; the main analysis was therefore based on the 22 patients completing both treatment periods. There was no significant difference in handgrip strength change from baseline between the two treatment regimens (coefficient −2.71, 95% CI −5.4, 0.01). Furthermore, there were no significant differences in any of the secondary outcomes or side effects. Conclusions: More frequent lower dosing does not further improve the efficacy of IVIg in stable IVIg‐dependent CIDP and does not result in fewer side effects. [ABSTRACT FROM AUTHOR]

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder characterized by patchy demyelination of nerve roots and distal nerves. The course may be monophasic progressive or relapsing-remitting. CIDP is less common in children than in adults. As in adults, children with CIDP present with proximal and distal weakness and loss of deep tendon reflexes. Children are most often brought to medical attention due to gait disturbance and falling. As in adults, immunomodulatory treatment is the mainstay of therapy. Based on the small number of case series available, children with CIDP seem have a more favorable long-term course than adults.

• This is the first epidemiological study of CIDP in South America and the Caribbean. • Epidemiology of CIDP in Chile is similar to other regions of the world. • Frequency of CIDP in Chile is marginally greater in men than in women. Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy. It usually has an insidious onset, progressive course and heterogeneous clinical features. As far as we know, there is no epidemiological information on CIDP in South America and the Caribbean. Our aim was to estimate the frequency of CIDP in the South-Eastern region of Santiago, where our hospital is based and the population number assigned is officially reported every year by the health authorities. Records of 581 patients registered with the diagnosis of neuropathy were found and all patients meeting the diagnostic criteria of the EFNS/PNS for definitive and possible CIDP were included. Data were collected using a data extraction protocol designed by the authors and which included demographic, clinical, laboratory and electrophysiological information. The estimated prevalence and incidence of CIDP were 2.95/100,000 and 0.46/100,000 respectively. Fifteen patients (8 men, 7 women) were classified as definitive or possible CIDP. Nine patients had typical CIDP and three also had diabetes mellitus. The prevalence and incidence rates were similar to those reported in other regions of the world. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP. Case Report: A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait. Laboratory investigations showed positive antinuclear antibodies (ANA), anti-(smooth muscle (SM) antibody, anti-RNP antibody, anti-SSA antibody, anti-ds-DNA antibody, and an erythrocyte sedimentation rate (ESR) of 75 mm/hr, low C4, leukopenia, and anemia. Electromyography (EMG) confirmed the diagnosis of CIDP. The patient's neuropathy and muscle weakness improved on treatment with intravenous immunoglobulin (IVIG) and high-dose steroids. Conclusions: The early clinical diagnosis of CIDP, supported by serological autoantibody profiles associated with SLE, can predict a good response to steroids. Most patients with CIDP are treated successfully with steroids if the diagnosis is made early. IVIG, plasmapheresis, or immunosuppressive therapy should be considered if there is no response to steroids. [ABSTRACT FROM AUTHOR]

Objective: Although various modalities of treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP) there are not any treatment protocol agreed. Materials and methods: We retrospectively evaluated the 20 CIDP patients (14 male, 6 female). Five patients were excluded from the study because they could not continue their treatments due to various problems during the treatment. The remaining 15 patients treated with monthly high dose intravenous methyl prednisolone for five years (IVMP) and followed up for 10 years. Results: The mean age of the patients was 48.1 ± 14.6 years. The mean duration of disease was 6.8 ± 3.1 years. We were found statistically significant difference between the pre-treatment and sixth month modified Rankin scores (p < 0.001). Similarly, significant improvement was observed at the end of first, fourth and fifth years of treatment. Statistically significant difference was found between baseline and tenth year modified Rankin scores of 12 patients who were treated with only IVMP during 10 years follow-up. Conclusion: Long-term monthly IVMP pulse therapy seems to be very effective in the treatment of CIDP. [ABSTRACT FROM AUTHOR]

Thirteen children with chronic inflammatory demyelinating polyneuropathy monitored between 1975 and 2005 are reported from Centre hospitalier universitaire Sainte-Justine, Montreal, Canada.

INTRODUCTION. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disorder of peripheral nerves and nerve roots that run continuously for 8 weeks or more. The classic form is characterized by symmetric sensory and motor involvement, weakness is present in both proximal and distal muscles and is characteristic in the electrophysiological study demyelination with albumin-cytological dissociation in cerebrospinal fluid (CSF). PRESENTATION OF THE CASE. 34-year-old woman with a history of depression in treatment, started about a year ago muscle weakness of all limbs, ascending and progressive, leading to the inability of the march. Was admitted with paraparesis, predominantly in lower extremities, hypoesthesia and absence of tendon reflexes. Electrophysiological study evidence severe demyelinating motor sensory polyneuropathy with secondary axonal degeneration. CSF protein concentration of 100 mg/dL and no cellularity. Starts treatment with corticosteroids and plasmapheresis, evolving with significant improvement in functionality and independent walking. DISCUSSION. The main therapeutic modalities in CIDP are intravenous immunoglobulin, corticosteroids and plasmapheresis. All these seem to be equally effective separately. The choice is influenced by patient preference, adverse effects, cost, duration and availability. The use of intravenous immunoglobulin is sometimes limited; corticosteroids are inexpensive, but with frequent and significant side effects; and plasmapheresis is expensive, invasive and only available at specialized centers. Most patients respond initially but relapses are frequent. [ABSTRACT FROM AUTHOR]

Purpose: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable, immune-mediated condition characterised by progressive or relapsing motor and sensory neurological deficits. The diagnosis is based on a combination of clinical, neurophysiological and supportive criteria, but can be challenging. In this study, we quantified the diameter and cross-sectional area of the lumbosacral nerve roots, and explored the imaging characteristics of the sciatic nerves, in patients with CIDP versus healthy controls using MRI.Methods: MRI of the lumbosacral plexus and both thighs was performed at 3 T. Orthogonal diameter and cross-sectional area of the lumbosacral nerve roots were measured, along with sciatic nerve cross-sectional area at the mid-thigh level. The MRI appearance of the sciatic nerves was also evaluated qualitatively. All measurements were performed by an observer blinded to the diagnosis.Results: 10 patients with CIDP and 10 healthy controls (age and sex-matched) were studied. Lumbosacral nerve root diameter and cross-sectional area were significantly increased in patients with CIDP compared to controls (mean diameter 6.0 ± 1.1 mm vs 4.8 ± 0.3 mm; p = 0.006), with a high sensitivity (89 %) and specificity (90 %) on ROC analysis. Sciatic nerve cross sectional area was also significantly increased in the CIDP group, and was accompanied by qualitative MRI changes.Conclusions: Quantitative MRI reveals significant hypertrophy of the lumbosacral nerve roots and sciatic nerves in patients with CIDP compared to controls. This study provides further evidence for the inclusion of lumbosacral nerve root and sciatic nerve hypertrophy on MRI as a supportive feature in the diagnostic criteria for CIDP. [ABSTRACT FROM AUTHOR]

• Case report of combined central and peripheral demyelinating disease in Zambia. • Access to disease modifying therapies is limited in the developing world. • A definitive diagnosis of MS can be a challenge in low-resource settings. • Low estimated prevalence in the region from under-recognition of disease. Overlap syndromes, where otherwise distinct autoimmune processes of the central and peripheral nervous systems are present in the same patient, are uncommon and have not been previously reported in sub-Saharan Africa. We present a case of a 32-year-old man who was found to have both clinically isolated syndrome and chronic inflammatory demyelinating polyneuropathy, highlighting the importance of continued efforts to establish the prevalence of demyelinating disease in the region given the limited treatment options currently available for autoimmune disease. [ABSTRACT FROM AUTHOR]