scholarly journals Fatigue in patients with chronic inflammatory demyelinating polyneuropathy

2021 ◽  
Vol 13 (1) ◽  
pp. 51-56
Author(s):  
R. A. Gapeshin ◽  
E. R. Barantsevich ◽  
D. I. Rudenko ◽  
T. R. Stuchevskaya ◽  
E. A. Gavrilova ◽  
...  
Keyword(s):  
Psychiatric Illness ◽  
Comparison Group ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
European Federation ◽  
Study Group ◽  
Motor Neuropathy ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Somatic Diseases ◽  
Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a peripheral neuropathy, predominantly motor neuropathy, with a progressive or relapse-remitting course. Fatigue is a condition characterized by a physical or mental feeling of lack of energy or lack of motivation for action, which is often present in patients with CIDP.Objective: to assess the severity of asthenia in CIDP patients.Patients and methods. Examinations were made in 34 inpatients treated for documented CIDP that met the international European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. A study group included patients with CIDP, whereas a comparison group consisted of volunteers without psychiatric illness, who were compensated for somatic diseases.Results and discussion. In the patients with CIDP, the level of fatigue was found to be much higher than normal. Approximately half of the CIDP patients had obvious asthenia. However, the level of fatigue did not correlate with the severity of the course of CIDP.Conclusion. The findings suggest that fatigue is important in patients with CIDP that should be taken into account in the treatment of these patients.

scholarly journals Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy

2020 ◽  
Author(s):  
Umair J. Chaudhary ◽  
Yusuf A. Rajabally
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Diagnostic Delay ◽  
Final Diagnosis ◽  
Demyelinating Polyneuropathy ◽  
European Federation ◽  
Delay In Diagnosis ◽  
Alternative Diagnosis ◽  
Inflammatory Neuropathy ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Made In

Abstract Background The frequency and causes of underdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) are uncertain. We aimed to assess the frequency and electroclinical features of pre-referral CIDP underdiagnosis and the duration of delay prior to diagnosis and treatment initiation in a tertiary specialist clinic. Methods We retrospectively investigated 60 consecutive patients attending our Inflammatory Neuropathy Service, between 2015 and 2019, with a final diagnosis of treatment-responsive definite/probable CIDP. We reviewed the clinical and electrophysiological data in light of European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines and determined the frequency, causes and delay in diagnosis of CIDP. Results An initial alternative diagnosis to that of CIDP had been made in 68.3% (41/60) of patients. The commonest alternative diagnosis was of Guillain–Barré syndrome (GBS) in 23.3% (14/60) patients. Non-GBS underdiagnoses (27/60; 45%) mainly consisted of genetic neuropathy (8/27; 29.6%), diabetic neuropathy (5/27; 18.5%) and chronic idiopathic axonal polyneuropathy (4/27; 14.8%). Non-GBS underdiagnoses were predominantly due to non-recognition of proximal weakness (70.4%), multifocal deficits (18.5%) or proprioceptive loss (7.4%). Electrophysiological misinterpretation was contributory to pre-referral non-GBS underdiagnoses of CIDP in 85% of patients. Mean diagnostic delay in patients with non-GBS underdiagnoses of CIDP was of 21.3 months (range 2–132 months). Conclusion Underdiagnosis of CIDP is frequent and may lead to significant diagnostic and treatment delay. We suggest that lack of comprehensive and precise attention to typical electroclinical features of CIDP and its diagnostic criteria at the time of initial evaluation are equally contributory to underdiagnoses.

scholarly journals Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

2019 ◽  
Vol 90 (9) ◽  
pp. 981-987 ◽  
Author(s):  
Helmar Christoph Lehmann ◽  
David Burke ◽  
Satoshi Kuwabara
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Motor Neuropathy ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Costly Treatment ◽  
Relative Rarity ◽  
Treatment Responses ◽  
Immunomodulating Drugs ◽  
Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

scholarly journals Intravenous Immunoglobulins (IVIG) in Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ): time to maximal recovery

2015 ◽  
Vol 42 (S1) ◽  
pp. S34-S34
Author(s):  
S Baker ◽  
A Opala
Keyword(s):  
Treatment Time ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Intravenous Immunoglobulins ◽  
Demyelinating Polyneuropathy ◽  
Secondary Outcome ◽  
Ivig Treatment ◽  
European Federation ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Changes Over Time ◽  
Over Time

Background: The response of Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ) to Intravenous Immunoglobulins (IVIG) treatment is well established. However, determination whether patients who do not respond to 2 IVIG treatments or those whose condition stabilizes (ICE Trial) would benefit from additional treatments remains unclear. We aim to identify time period required to reach maximal strength gains from IVIG treatment (plateau). Furthermore, we will assess nerve conduction studies (NCS) changes over time with IVIG treatment. This will help in establishing a time course for treatment of CIDP with IVIG to maximize recovery. Methods: We performed a retrospective chart review of 27 patients with CIDP, with diagnosis confirmed by European Federation of Neurological Societies/Peripheral Nerve Society Guidelines (EFNS/PNS). Each patient’s strength response including: grip strength, knee extension, elbow flexion and dorsiflexion (using JAMAR Dynamometer) and NCS changes over time during IVIG treatment were analyzed. The primary outcome is duration of IVIG treatment, in months, required to reach a plateau in strength. Secondary outcome is NCS change including: Terminal Latencies, Conduction Velocities, Compound Sensory and Motor action potentials in nerves of upper and lower extremities over treatment time (emerging trends). Results: Pending (available by April 2015) Conclusion: Pending (available by April 2015)

scholarly journals Latent Therapeutic Demand of Immunoglobulin therapies in neuropathies – chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain–Barré syndrome (GBS) and multifocal motor neuropathy (MMN) in the United States

2020 ◽  
Author(s):  
Megha Bansal ◽  
Albert Farrugia
Keyword(s):  
Sensitivity Analysis ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Demyelinating Polyneuropathy ◽  
Guillain Barre Syndrome ◽  
Motor Neuropathy ◽  
Loading Dose ◽  
Clinical Factors ◽  
The Us ◽  
Inflammatory Demyelinating Polyneuropathy

AbstractChronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder characterized by progressive weakness and impaired sensory function in the legs and arms. Guillain-Barré syndrome is a disorder in which the body’s immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs (NIH). Multifocal motor neuropathy is a progressive muscle disorder characterized by muscle weakness in the hands, with differences from one side of the body to the other in the specific muscles involved (NIH). We have modeled a latent therapeutic demand (LTD) of IVIg for CIDP and similar neuropathies in the US. We used the decision analysis methodology similar to the methods used by Stonebraker et al1 for modeling LTD of IVIg. The model is based on the relationships of the epidemiological and clinical factors. Most of the usage patterns and dosage level of albumin are according to the epidemiological studies and clinical trials. The model is built in Microsoft Excel. The analysis is conducted based on oneway sensitivity analysis and probabilistic sensitivity analysis. The demand in terms of grams per 1,000 inhabitants is calculated depending on the treatment schedule and the prevalence of the disease. The model for CIDP has eight variables including prevalence of CIDP, patients using IVIg, dosage and treatment patterns. The annual demand of IVIg is based on initial treatment of 24 weeks followed by a maintenance period, with lower dosage and frequency of treatment for another 24 weeks2. The model for GBS has eight variables with a loading dose for 3-6 days followed by a second dose in case of relapse. The model for MMN has nine variables. It has a loading dose followed by maintenance dose every 1-6 weeks depending on the clinical factors of the patient. On an average, IVIg use was calculated as 100 gms, 5.6 gms and 35 gms per 1,000 inhabitants for CIDP, GBS and MMN, respectively, in the US annually.

Evidence base for investigative and therapeutic modalities in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy

2022 ◽  
Author(s):  
Hendrik Stephan Goedee ◽  
Yusuf A Rajabally
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Management Strategies ◽  
Multifocal Motor Neuropathy ◽  
Evidence Base ◽  
Demyelinating Polyneuropathy ◽  
Motor Neuropathy ◽  
Success Rates ◽  
Therapeutic Modalities ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Correct Reading

Chronic inflammatory demyelinating polyneuropathy, its variants and multifocal motor neuropathy belong to a spectrum of peripheral nerve disorders with complex dysimmune disease mechanisms. Awareness of the unique clinical phenotypes but also heterogeneity between patients is vital to arrive at early suspicion and ordering appropriate tests. This includes requirements for optimal electrodiagnostic protocol, aimed to capture sufficient electrophysiologic evidence for relevant abnormalities, a case-based approach on the eventual need to further expand the diagnostic armamentarium and correct reading of their results. Considerable phenotypical variation, diverse combinations of abnormalities found on diagnostic tests and heterogeneity in disease course and treatment response, all contribute to widespread differences in success rates on timely diagnosis and optimal treatment. We aim to provide a practical overview and guidance on relevant diagnostic and management strategies, including pitfalls and present a summary of the relevant novel developments in this field.

scholarly journals Interleukin 8, a Biomarker to Differentiate Guillain-Barré Syndrome From CIDP

2021 ◽  
Vol 8 (5) ◽  
pp. e1031
Author(s):  
Gautier Breville ◽  
Agustina M. Lascano ◽  
Pascale Roux-Lombard ◽  
Nicolas Vuilleumier ◽  
Patrice H. Lalive
Keyword(s):  
Predictive Value ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Acute Onset ◽  
Demyelinating Polyneuropathy ◽  
Interleukin 8 ◽  
Guillain Barre Syndrome ◽  
European Federation ◽  
Guillain Barré Syndrome ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Guillain Barré

ObjectiveTo determine whether CSF interleukin 8 (IL-8) concentration can help to distinguish Guillain-Barré syndrome (GBS) from chronic inflammatory demyelinating polyneuropathy (CIDP) at the initial stage of the disease.MethodsWe performed retrospective immunoassay of IL-8 in CSF, collected at the University Hospitals of Geneva between 2010 and 2018, from patients diagnosed with GBS (n = 45) and with CIDP (n = 30) according to the Brighton and European Federation of Neurological Societies/Peripheral Nerve Society criteria by a physician blinded to biological results.ResultsCSF IL-8 was higher in GBS (median: 83.9 pg/mL) than in CIDP (41.0 pg/mL) (p < 0.001). Receiver operating characteristic analyses indicated that the optimal IL-8 cutoff was 70 pg/mL. Above this value, patients were more likely to present GBS than CIDP (specificity 96.7%, sensitivity 64.4%, positive predictive value [PPV] 96.7%, and negative predictive value [NPV] 64.4%). Among GBS subcategories, IL-8 was higher in acute inflammatory demyelinating polyneuropathy (AIDP, median: 101.8 pg/mL) than in other GBS variants (median: 53.7 pg/mL). In addition, with CSF IL-8 above 70 pg/mL, patients were more likely to present AIDP than acute-onset CIDP (p < 0.001; specificity 100%, sensitivity 78.8%, PPV 100%, and NPV 46.2%) or other CIDP with nonacute presentation (p < 0.0001; specificity 95.8%, sensitivity 78.8%, PPV 96.3%, and NPV 76.7%).ConclusionCSF IL-8 levels can help to differentiate AIDP variant of GBS from CIDP, including acute-onset CIDP, with high specificity and PPV. This may improve early and appropriate treatment.Classification of EvidenceThis study provides Class II evidence that CSF IL-8 levels accurately distinguish patients with GBS from those with CIDP.

scholarly journals E.08 Subcutaneous vs. intravenous immunoglobulins for chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy

2016 ◽  
Vol 43 (S2) ◽  
pp. S17-S18
Author(s):  
JM Racosta ◽  
LA Sposato ◽  
J Baker ◽  
K Kimpinski
Keyword(s):  
Fixed Effects ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Meta Analysis ◽  
Multifocal Motor Neuropathy ◽  
Demyelinating Polyneuropathy ◽  
Comparable Efficacy ◽  
High Dose ◽  
Motor Neuropathy ◽  
Suitable Alternative ◽  
Inflammatory Demyelinating Polyneuropathy

Background: Background: High-dose intravenous immunoglobulin (IV-Ig) is an evidence-based treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). Recently, subcutaneous Ig (SC-Ig) has received increasing attention. We performed a meta-analysis to assess the efficacy of SC-Ig versus IV-Ig. Methods: Methods: We searched PubMed, Embase, and Scopus from January, 1990 to December, 2015 for publications comparing IV-Ig vs. SC-Ig in patients with CIDP or MMN. We performed fixed-effects meta-analyses for strength changes as measured by the Medical Research Council sum score changes (MRC-SS). Results: Results: A total of 8 studies comprising 138 patients (88 with CIDP and 50 with MMN) were included in the meta-analysis. Considering the total population the use of SC-Ig showed slightly better results for MRC-SS (ES=-1.78, 95%CI=-3.45 to -0.11, I2<0.001%). However, when CIDP and MMN were compared separately, there were no differences between treatments (CIDP: ES=-0.28, 95%CI=-0.57 to 0.02, I2<0.001%; MMN: ES=-0.34, 95%CI=-3.99 to 3.31, I2<0.001%). Conclusions: Conclusions: We found comparable efficacy between SC and IV-Ig administrations for CIDP and MMN. These results suggest that SC-Ig is a suitable alternative treatment method, especially when other situations (e.g. convenience, safety profile) warrant its use. Further studies are needed to explore the efficacy of SC-Ig for CIDP and MMN.

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