CIDP and Related Variants and Overlap Disorders

2019 ◽  
pp. 208-244
Author(s):  
Jeffrey A. Allen
Keyword(s):  
Monoclonal Gammopathy ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Clinical Manifestations ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy ◽  
Peripheral Nerve Disorder ◽  
Diagnostic Data ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
History Of

This chapter begins with a history of chronic immunological neuropathies. It then looks in particular at chronic inflammatory demyelinating polyneuropathy (CIDP), which is an immune-mediated peripheral nerve disorder characterized by progressive or relapsing motor or sensory symptoms. It then considers the epidemiology, clinical manifestations, and electrophysiology of CIDP. The chapter examines diagnostic data and diagnostic criteria for CIDP. It then looks at other neuropathies with clinical and electrophysiologic features that are shared with CIDP. Particular attention is given to neuropathy associated with monoclonal gammopathy including IgM associated neuropathy and POEMS syndrome, polyneuropathies associated with specific autoantibodies including antibodies that target nodal and paranodal structures, and multifocal motor neuropathy. For each condition diagnostic data, pathophysiology and treatment are discussed.

scholarly journals How We Treat Autoimmune Small Fiber Polyneuropathy with Immunoglobulin Therapy

2018 ◽  
Vol 80 (5-6) ◽  
pp. 304-310 ◽  
Author(s):  
Jill R. Schofield ◽  
Kamal R. Chemali
Keyword(s):  
Intravenous Immunoglobulin ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Immunoglobulin Therapy ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy ◽  
Intravenous Immunoglobulin Therapy ◽  
Small Fiber ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Significant Efficacy

Intravenous immunoglobulin therapy is FDA approved for the immune-mediated peripheral nerve disorders Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. Immunoglobulin therapy has been used increasingly with significant efficacy in the treatment of patients with disabling autoimmune forms of dysautonomia, which are most often small fiber (autonomic and/or sensory) polyneuropathies. It is recognized by most who treat these disorders, however, that patients with autonomic dysfunction treated with intravenous immunoglobulin therapy develop aseptic meningitis or severe lingering headache more frequently than other patient populations when this therapy is dosed in the traditional fashion. We discuss our combined 27 years of experience with the use of immunoglobulin and other immune modulatory therapy in patients with autoimmune small fiber polyneuropathy.

Disorders of peripheral nerves and motor neuron disease

2016 ◽  
Author(s):  
Sathiji Nageshwaran ◽  
Heather C Wilson ◽  
Anthony Dickenson ◽  
David Ledingham
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Peripheral Nerves ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Evidence Based ◽  
Motor Neuropathy ◽  
Treatment Regimens ◽  
Peripheral Nerve Involvement ◽  
Inflammatory Demyelinating Polyneuropathy

This chapter discusses the clinical features and evidence-based drug treatment regimens of polyneuropathies (Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy, paraproteinaemic neuropathies, and vasculitic neuropathies), mononeuropathies (Bell’s palsy), systemic conditions with peripheral nerve involvement (Sjögren’s and sarcoidosis), and motor neuron disease (MND).

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 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.

004 Mechanisms of nerve dysfunction in inflammatory neuropathies

2018 ◽  
Vol 89 (6) ◽  
pp. A3.1-A3
Author(s):  
Nidhi Garg ◽  
Susanna B Park ◽  
James Howells ◽  
Con Yiannikas ◽  
Steve Vucic ◽  
...  
Keyword(s):  
Molecular Mechanisms ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Conduction Block ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy ◽  
Treatment Approaches ◽  
Immune Mediated ◽  
Marked Variability ◽  
Nerve Enlargement ◽  
Axonal Excitability

IntroductionImmune-mediated neuropathies are a cause of disability and an immense cost to the healthcare system. They include chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN) and the neuropathy associated with IgM antibodies against myelin-associated glycoprotein (MAG). CIDP is extremely heterogeneous with marked variability in treatment responsiveness. Patients with MMN often respond to treatment but progressive weakness and wasting typically ensues over time. No therapy has consistently proven effective in anti-MAG neuropathy. The present series of studies were undertaken to improve understanding of disease mechanisms in these neuropathies, a critical step before targeted treatment approaches can be developed.MethodsPatients fulfilling Peripheral Nerve Society criteria for CIDP or MMN and patients positive for anti-MAG IgM underwent comprehensive clinical assessments, neurophysiology, serological testing and structural assessments.ResultsThe patient cohort consisted of 80 patients (51 CIDP, 14 MMN, 15 MAG). 6% of CIDP patients tested positive for anti-neurofascin 155 (NF155) and 4% for anti-contactin 1 IgG4. Anti-NF155 neuropathy was characterised by diffuse nerve enlargement and an axonal excitability profile consistent with severe disruption of the paranodal seal. CIDP patients testing negative for IgG4 antibodies also demonstrated significant nerve enlargement compared with healthy subjects. Axonal excitability profiles differed in those with and without median nerve conduction block. MMN was characterised by patchy nerve enlargement, marked increases in super-excitability and enlarged motor unit size. In contrast, anti-MAG neuropathy patients demonstrated a proximal pattern of nerve enlargement and an axonal excitability profile characterised by reduced super-excitability consistent with increased juxta-paranodal fast potassium channel conductance.ConclusionPatterns of nerve enlargement and neurophysiological profiles differ in the immune-mediated neuropathies providing insights into molecular mechanisms. These results provide templates that can guide treatment approaches. The combination of directed autoantibody assays and measures of axonal function can be used to monitor disease progression and therapeutic response.

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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