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

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.

P.056 Optimizing IVIg Use for Neuromuscular Conditions in British Columbia, Canada – Targeting High and Chronic User Groups

Background: Neuromuscular conditions account for 1/3 of IVIg use in BC and costs over $10 million annually. Since 2013, the BC Neuromuscular Review Panel has developed diagnostic and treatment algorithms for the use of IVIg. A framework was created to review high dose and chronic users. Methods: Utilizing Central Transfusion Registry data, all patients treated with IVIg for approved neuromuscular conditions (CIDP, MG, MMN) since April 1, 2013 were identified. Annual cohorts for patients using higher than usual dose and chronic use (>3 years) were established, and evaluated annually. Patient specific recommendations were made. Results: The initial cohort identified 38 high users of 377 patients receiving IVIg. 27 appropriate, 9 “not appropriate”. Subsequent cohorts showed a decrease in number of patients receiving inappropriate IVIg doses. In BC there has been a 36% increase in neuromuscular patients treated with IVIg (377 in 2013/14 to 512 in 2016/17). Despite this, IVIg the program has effectively reduced the annual grams/patient from 516 gm/patient in 2013/14 to 489 gm/patient in 2016/17. Conclusions: The BC Neuromuscular IVIg Review confirms that the majority of IVIg use is appropriate. Following yearly cohorts of chronic and high dose users helps optimize IVIg use, which may lead to improved patient care.

Dynamic contraction and fatigue analysis in biceps brachii muscles using synchrosqueezed wavelet transform and singular value features

In this study, the dynamic contractions and the associated fatigue condition in biceps brachii muscle are analysed using Synchrosqueezed Wavelet Transform (SST) and singular value features of surface Electromyography (sEMG) signals. For this, the recorded signals are decomposed into time-frequency matrix using SST. Two analytic functions namely Morlet and Bump wavelets are utilised for the analysis. Singular Value Decomposition method is applied to this time-frequency matrix to derive the features such as Maximum Singular Value (MSV), Singular Value Entropy (SVEn) and Singular Value Energy (SVEr). The results show that both these wavelets are able to characterise nonstationary variations in sEMG signals during dynamic fatiguing contractions. Increase in values of MSV and SVEr with the progression of fatigue denotes the presence of nonstationarity in the sEMG signals. The lower values of SVEn with the progression of fatigue indicate the randomness in the signal. Thus, it appears that the proposed approach could be used to characterise dynamic muscle contractions under varied neuromuscular conditions.

Inpatient Diagnosis and Management of Neuromuscular Disorders

Although many neuromuscular conditions are evaluated on an outpatient basis owing to their chronic or progressive nature, more urgent evaluation and management is often required for the inpatient presenting with acute to subacute focal or generalized numbness or weakness. This review focuses on clinical pattern recognition and basic anatomic localization principles to aid in the identification of common, as well as some less frequently encountered, neuromuscular disorders in hospitalized patients. The characteristic clinical and diagnostic features, associated complications, and recommended treatments of key neuromuscular conditions with acute and subacute manifestations are discussed. These conditions can be life-threatening in some cases, such as in Guillain–Barré syndrome, owing to associated oropharyngeal weakness, respiratory failure, or marked dysautonomia. Prompt recognition of the clinical and pathologic features is therefore necessary to reduce associated morbidity and mortality.

Botulinum Toxin Services for Neurorehabiliation: Recommendations for Challenges and Opportunities during the COVID-19 Pandemic

The COVID-19 pandemic severely impacted the function of medical facilities and rehabilitation services worldwide, including toxin services delivering Botulinum toxin treatments for neuromuscular conditions such as spasticity, dystonia, and sialorrhea. The aim of this paper is to understand how toxin services have dealt with the situation and what strategies have been adopted to continue services. The recommendations are based on a virtual round table held with toxin services experts from different European countries who shared their experiences and discussed the best practices. The challenges for toxin services were reviewed based on the experts’ experiences and on relevant literature from 2020 and 2021. A set of recommendations and best practices were compiled, focusing firstly on guidance for clinical practice, including assessing patients’ health and risk status and the urgency of their treatment. Secondly, it was discussed how patients on botulinum toxin therapy can be cared for and supported during the pandemic, and how modern technology and tele-medicine platforms can be generally used to optimize effectiveness and safety of toxin treatments. The technological advances prompted by the COVID-19 crisis can result in better and more modern patient care in the future.

Neurological and neuromuscular conditions and symptoms

Conditions associated with the impaired nervous system are common in paediatric palliative care (PPC). And yet, many neurological conditions are themselves rare, thereby limiting knowledge about each condition and its expected course. Parents and providers often face significant uncertainty about the risks and benefits associated with treatment decisions as prognostic information and scientific evidence may be limited. Other challenges include difficult assessment for the presence and source of distressing symptoms and the lifelong nature of many of the symptoms these children experience. This chapter addresses several aspects of palliative care in the management of neurological and neuromuscular symptoms. The first section provides a palliative framework for addressing neurological and neuromuscular conditions and a brief overview of the major life-limiting conditions related to the nervous system. The second section reviews the associated symptoms of the impaired central nervous system (CNS) along with their treatment strategies. This chapter deliberately focuses on care for children with severe neurological impairment, as these children increasingly benefit from the specific expertise of palliative care teams.