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 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.

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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