scholarly journals P.060 Immunoglobulin Use For Neuromuscular Conditions: Updating British Columbia Provincial Guidelines Through Focused Literature Review

2021 ◽  
Vol 48 (s3) ◽  
pp. S35-S36
Author(s):  
EK Tse ◽  
CB Smith ◽  
KM Chapman ◽  
K Beadon
Keyword(s):  
British Columbia ◽  
Literature Review ◽  
Outcome Measures ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Evidence Based ◽  
Motor Neuropathy ◽  
Vasculitic Neuropathy ◽  
Neuromuscular Conditions ◽  
Objective Outcome

Background: Immune-mediated neuromuscular conditions often cause significant disability and may require ongoing immunomodulating therapies such as immunoglobulin (Ig). Ig use in several neuromuscular conditions such as Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is supported by robust evidence, however Ig is increasingly used for other disorders. In British Columbia (BC), Ig use has increased annually; last year, expenditure exceeded 51 million dollars, 35% relating to neurological disease. Within the context of the pandemic, Ig supply is at risk of shortages. Methods: A focused literature review was conducted of CIDP, Guillain-Barré Syndrome (GBS), Multifocal Motor Neuropathy (MMN), Myasthenia Gravis (MG), and other neuromuscular conditions to compare BC Ig guidelines with international best practices. Provincial recommendations for Ig use were updated accordingly. Results: Evidence-based practices include acute and chronic Ig use in CIDP and MMN, and acute or relapse-related treatment in GBS and MG. Ig may be beneficial in other treatment-refractory inflammatory disorders such as Lambert-Eaton Myasthenic Syndrome and vasculitic neuropathy. Objective outcome measures can optimize patient care and ensure appropriate resource utilization. Conclusions: Updated BC guidelines emphasize using established diagnostic criteria, objective outcome measures and minimum effective Ig doses for neuromuscular conditions. Periodic literature reviews on Ig use allow guidelines to remain evidence-based.

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 Fasciculation differences between ALS and non-ALS patients: an ultrasound study

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jingwen Liu ◽  
Yi Li ◽  
Jingwen Niu ◽  
Lei Zhang ◽  
Jing Fan ◽  
...  
Keyword(s):  
Distribution Pattern ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Firing Frequency ◽  
Low Grade ◽  
Motor Neuropathy ◽  
Detection Rates ◽  
Ultrasound Study ◽  
Muscle Groups ◽  
Als Patients

Abstract Background Fasciculation is an important sign for the diagnosis of amyotrophic lateral sclerosis (ALS). Our study aimed to analyze the difference in fasciculation detected with muscle ultrasonography (MUS) between ALS patients and non-ALS patients with symptoms resembling ALS. Methods Eighty-eight ALS patients and fifty-four non-ALS (eight multifocal motor neuropathy, 32 chronic inflammatory demyelinating polyneuropathy/Charcot-Marie-Tooth, and 14 cervical spondylopathy or lumbar spondylopathy) patients were recruited. MUS was performed on 19 muscle groups in cervical, lumbosacral, bulbar, and thoracic regions for each patient. The intensity of fasciculation was divided into five grades based on firing frequency and number in the involved muscle groups. Results The overall detection rates were 72.8% in ALS and 18% in non-ALS patients. The fasciculation grades (median [IQR]) were 2 (0–3) in ALS and 0 (0–0) in non-ALS patients (P < 0.001). Fasciculations were observed in four regions for ALS patients and primarily distributed in proximal limbs. Fasciculations in non-ALS patients were primarily low-grade and mostly distributed in distal limbs. Discussion The fasciculation grade was higher in ALS than non-ALS patients. The distribution pattern of fasciculation was different between ALS and non-ALS patients. Conclusions The fasciculation grade and distribution pattern detected with MUS could help distinguish ALS from non-ALS patients.

Disorders of single nerves, roots, and plexuses

2016 ◽  
Author(s):  
Kerry R. Mills
Keyword(s):  
Nerve Conduction ◽  
Cervical Radiculopathy ◽  
Multifocal Motor Neuropathy ◽  
Nerve Conduction Studies ◽  
Motor Neuropathy ◽  
Vasculitic Neuropathy ◽  
Brachial Neuritis ◽  
Pressure Palsies ◽  
Lateral Sclerosis

The role of electromyography (EMG) and nerve conduction studies in disorders of single nerve, root, and plexus lesions are discussed. The motor and sensory anatomy underpinning diagnosis is described and a scheme presented showing the key muscles to be examined using EMG to differentiate nerve, plexus, and root lesions. The main causes of mononeuritis multiplex, of either axonal degeneration or demyelinative pathology, are covered, including diabetic neuropathy, vasculitic neuropathy, multifocal motor neuropathy with block, and the Lewis–Sumner syndrome. The confirmatory role of EMG and nerve conduction studies in the investigation of cervical and lumbar radiculopathies is highlighted as is the use of transcranial magnetic stimulation to differentiate cervical radiculopathy with myelopathy from amyotrophic lateral sclerosis. The neurophysiological hallmarks of traumatic cervical plexus lesions, including obstetric causes, inherited and acquired brachial neuritis, hereditary liability to pressure palsies, the cervical rib syndrome, and radiation plexopathy are also covered.

Complications of Immunoglobulin Therapy and Implications for Treatment of Inflammatory Neuropathy: A Review

2019 ◽  
Vol 14 (1) ◽  
pp. 3-13 ◽  
Author(s):  
Ahmed Abbas ◽  
Yusuf A. Rajabally.
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Kidney Injury ◽  
Immunoglobulin Therapy ◽  
Multifocal Motor Neuropathy ◽  
Evidence Base ◽  
Motor Neuropathy ◽  
Inflammatory Neuropathy ◽  
Inflammatory Neuropathies ◽  
Delayed Complications ◽  
Associated Risk Factors

Background: Intravenous Immunoglobulin (IVIg) forms a cornerstone of effective treatment for acute and chronic inflammatory neuropathies, with a class I evidence base in Guillain-Barré Syndrome (GBS), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). It is generally considered to be a safe therapy however there are several recognised complications which are reviewed in this article. Discussion and Conclusion: Most adverse events are immediate and mild such as headache, fever and nausea although more serious immediate reactions such as anaphylaxis may rarely occur. Delayed complications are rare but may be serious, including thromboembolic events and acute kidney injury, and these and associated risk factors are also discussed. We emphasise the importance of safe IVIg administration and highlight practical measures to minimise complications of this therapy.

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 Involvement of serum HSP 70 in Guillain-Barré Syndrome: An exploratory study and a review of current literature

2015 ◽  
Vol 61 (01) ◽  
pp. 61-67
Author(s):  
Aida Loshaj-Shala ◽  
Ana Poceva Panovska ◽  
Katerina Brezovska ◽  
Giangiacomo Beretta ◽  
Ljubica Suturkova ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Multifocal Motor Neuropathy ◽  
Guillain Barre Syndrome ◽  
Motor Neuropathy ◽  
Hsp 70 ◽  
Guillain Barré Syndrome ◽  
Different Types ◽  
Guillain Barré ◽  
Shock Proteins ◽  
Altered Serum

The evolutionary conserved family of heat shock proteins (HSP) is responsible for protecting cells against different types of stress. Although the levels of HSP can be readily measured in serum, the levels of HSP 70 in patients Guillain-Barre Syndrome (GBS) have not been studied before. To this aim we investigate whether patients with GBS (n=21) had altered serum HSP 70 levels compared to healthy controls (HC, n=9) and to patients affected by other immune disorders such as multifocal motor neuropathy (MMN, n=4) and chronic inflammatory demyelinating polyneuropathy (CIDP, n=6). The highest HSP 70 value (15.78 ± 1.72 ng/mL) was found in one patient in the GBS group, although we have found that serum HSP70 levels were significantly higher in 2 out of the 21 GBS patients (9.5%). Hence, it is of interest to underline that the patient with the highest HSP70 level, had also the best recovery rate. Моrе extensive research is required in order to support the hypothesis that HSP 70 serum concentration may be a useful biomarker for the prediction of remission outcome for GBS patients.

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.

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.

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