Impact of SARS-CoV-2 Infection Among Non-Invasive Ventilated ALS Patients

Background: The impact of SARS-CoV-2 infection among neuromuscular diseases with respiratory involvement, including amyotrophic lateral sclerosis (ALS), is still to be elucidated. Objectives: We aim to characterize the clinical outcome of ALS patients non-invasive ventilated (NIV), following SARS-CoV-2 infection. Methods: We analyzed retrospectively our patients followed regularly at our ALS clinic, from the beginning of the COVID-19 pandemic (middle March 2020) to March 2021. We included patients on NIV with a documented SARS-CoV-2 infection. We recorded demographic and clinical data, including from the acute infectious illness. Results: Three men with spinal-onset ALS are described, mean age of onset was 55±9.1 years (45–61), and mean disease duration was 17.5±15.9 months (6.1–41). All of them were wheelchair-bounded, with a mean ALSFRS-R of 15.3±0.6 (15–16). One patient used NIV 15 hours/day, 2 between 4 to 7 hours/day, and all used assisted coughing twice daily. None had coexistent comorbidities. They were managed for SARS-CoV-2 infection as outpatients with fluticasone, bronchodilators, azithromycin and increasing frequency of assisted coughing. Supplemental oxygen (mean of 2 liters per minute) was needed in two patients, and one required NIV also during the daytime. Total recovery from SARS-CoV-2 infection was observed in all, despite being in an advanced stage of their disease, with severe respiratory involvement. Conclusions: Prompt medical treatment is recommended for ALS patients with severe disease infected by SARS-CoV-2.

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A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany

Brain Sciences ◽

10.3390/brainsci11030372 ◽

2021 ◽

Vol 11 (3) ◽

pp. 372

Author(s):

Tara Peseschkian ◽

Isabell Cordts ◽

René Günther ◽

Benjamin Stolte ◽

Daniel Zeller ◽

...

Keyword(s):

Quality Of Life ◽

Amyotrophic Lateral Sclerosis ◽

Rating Scale ◽

Severe Disease ◽

Disease Stage ◽

Individualized Care ◽

Als Patients ◽

The Impact ◽

Lateral Sclerosis

Improving quality of life (QoL) is central to amyotrophic lateral sclerosis (ALS) treatment. This Germany-wide, multicenter cross-sectional study analyses the impact of different symptom-specific treatments and ALS variants on QoL. Health-related QoL (HRQoL) in 325 ALS patients was assessed using the Amyotrophic Lateral Sclerosis Assessment Questionnaire 5 (ALSAQ-5) and EuroQol Five Dimension Five Level Scale (EQ-5D-5L), together with disease severity (captured by the revised ALS Functional Rating Scale (ALSFRS-R)) and the current care and therapies used by our cohort. At inclusion, the mean ALSAQ-5 total score was 56.93 (max. 100, best = 0) with a better QoL associated with a less severe disease status (β = −1.96 per increase of one point in the ALSFRS-R score, p < 0.001). “Limb-onset” ALS (lALS) was associated with a better QoL than “bulbar-onset” ALS (bALS) (mean ALSAQ-5 total score 55.46 versus 60.99, p = 0.040). Moreover, with the ALSFRS-R as a covariate, using a mobility aid (β = −7.60, p = 0.001), being tracheostomized (β = −14.80, p = 0.004) and using non-invasive ventilation (β = −5.71, p = 0.030) were associated with an improved QoL, compared to those at the same disease stage who did not use these aids. In contrast, antidepressant intake (β = 5.95, p = 0.007), and increasing age (β = 0.18, p = 0.023) were predictors of worse QoL. Our results showed that the ALSAQ-5 was better-suited for ALS patients than the EQ-5D-5L. Further, the early and symptom-specific clinical management and supply of assistive devices can significantly improve the individual HRQoL of ALS patients. Appropriate QoL questionnaires are needed to monitor the impact of treatment to provide the best possible and individualized care.

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How Loan Bank of Assistive Technology Impacts on Life of Persons with Amyotrophic Lateral Sclerosis and Neuromuscular Diseases: A Collaborative Initiative

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph18020763 ◽

2021 ◽

Vol 18 (2) ◽

pp. 763

Author(s):

Thais Pousada ◽

Jessica Garabal-Barbeira ◽

Cristina Martínez ◽

Betania Groba ◽

Laura Nieto-Riveiro ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Assistive Technology ◽

Positive Impact ◽

Outcome Measurement ◽

Neuromuscular Diseases ◽

Three Dimensions ◽

Assistive Device ◽

Cross Sectional ◽

The Impact ◽

Lateral Sclerosis

(1) Background: The study is focused on the implementation of outcome measurement tools to assess the impact of an assistive device from a loan bank in the lives of people with Amyotrophic Lateral Sclerosis and Neuromuscular Diseases. The secondary purpose is to analyse the correct matching between the person and technology, derived from the counselling of an occupational therapist. (2) Methods: Cross-sectional study. The sample was formed by 28 people with rare neurodegenerative disorders. A specific questionnaire, the Psychosocial Impact of Assistive Device Scale (PIADS), and the Matching Person and Technology (MPT) tool were applied to collect the data. (3) Results: The dimension of the PIADS with the best score was competence, and the variations according to gender were not remarkable. The three dimensions of the PIADS (competence, adaptability, and self-esteem) were correlated positively between them and with the mean score of the MPT tool (p < 0.01). The type of assistive technology (AT), diagnosis, and correct match between person–technology are the main factors that condition a positive impact. (4) Conclusions: The results noted the importance of assessing the needs, demands, and contexts of people with rare neurodegenerative diseases to prescribe the best AT. Loan banks of AT have to be considered a valid service that complements their lack in public health services.

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Antisense Transcription across Nucleotide Repeat Expansions in Neurodegenerative and Neuromuscular Diseases: Progress and Mysteries

Genes ◽

10.3390/genes11121418 ◽

2020 ◽

Vol 11 (12) ◽

pp. 1418

Author(s):

Ana F. Castro ◽

Joana R. Loureiro ◽

José Bessa ◽

Isabel Silveira

Keyword(s):

Gene Expression ◽

Neuromuscular Diseases ◽

Antisense Transcription ◽

Spinocerebellar Ataxia Type ◽

Spinocerebellar Ataxia Type 7 ◽

Repeat Expansions ◽

Cellular Pathways ◽

The Impact ◽

Lateral Sclerosis

Unstable repeat expansions and insertions cause more than 30 neurodegenerative and neuromuscular diseases. Remarkably, bidirectional transcription of repeat expansions has been identified in at least 14 of these diseases. More remarkably, a growing number of studies has been showing that both sense and antisense repeat RNAs are able to dysregulate important cellular pathways, contributing together to the observed clinical phenotype. Notably, antisense repeat RNAs from spinocerebellar ataxia type 7, myotonic dystrophy type 1, Huntington’s disease and frontotemporal dementia/amyotrophic lateral sclerosis associated genes have been implicated in transcriptional regulation of sense gene expression, acting either at a transcriptional or posttranscriptional level. The recent evidence that antisense repeat RNAs could modulate gene expression broadens our understanding of the pathogenic pathways and adds more complexity to the development of therapeutic strategies for these disorders. In this review, we cover the amazing progress made in the understanding of the pathogenic mechanisms associated with repeat expansion neurodegenerative and neuromuscular diseases with a focus on the impact of antisense repeat transcription in the development of efficient therapies.

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Incidence and Characteristics of Spinal Decompression Surgery after the Onset of Symptoms of Amyotrophic Lateral Sclerosis

Neurosurgery ◽

10.1227/01.neu.0000180028.64385.d3 ◽

2005 ◽

Vol 57 (5) ◽

pp. 984-989 ◽

Cited By ~ 18

Author(s):

Daniel Yoshor ◽

Arnett Klugh ◽

Stanley H. Appel ◽

Lanny J. Haverkamp

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Symptom Onset ◽

Spinal Surgery ◽

Foot Drop ◽

Spinal Decompression ◽

Sporadic Als ◽

Number Of Patients ◽

Als Patients ◽

The Impact ◽

Lateral Sclerosis

Abstract OBJECTIVE: The high incidence of spondylosis in patients at the mean age of onset (55.7 yr) of amyotrophic lateral sclerosis (ALS) can make recognition of ALS as a cause of weakness difficult. METHODS: To assess the impact of this diagnostic dilemma on neurosurgical practice, we performed a retrospective analysis of a database of more than 1500 patients with motor neuron disease. RESULTS: Of 1131 patients with typical, sporadic ALS, 47 (4.2%) underwent decompressive spinal surgery after the onset of retrospectively recognized symptoms of ALS. Among 55 operations in 47 ALS patients, 86% yielded no improvement, 9% produced minor improvement, and only 5% provided significant benefit. Cervical decompression was performed in 56%, lumbar in 42%, and thoracic in 2%. Foot drop was a symptom prompting surgery in 11 patients, and in 10, this finding was subsequently attributed solely to ALS. No differences between ALS patients who underwent spinal decompression and other ALS patients were noted regarding age at symptom onset, severity of impairment at time of diagnosis, or rate of disease progression. Not surprisingly, patients who had spinal surgery tended to have a longer interval between retrospectively recognized symptom onset and diagnosis of ALS. CONCLUSION: A small but significant number of patients with unrecognized ALS undergo spinal surgery that in retrospect may be inappropriate. The possibility of ALS must be considered in the evaluation of patients with weakness even in the presence of radiographic evidence of spondylosis and nerve root or spinal cord impingement.

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The COVID-19 Hospitalization Metric in the Pre- and Post-vaccination Eras as a Measure of Pandemic Severity: A Retrospective, Nationwide Cohort Study

10.21203/rs.3.rs-898254/v1 ◽

2021 ◽

Author(s):

Nathanael Fillmore ◽

Jennifer La ◽

Chunlei Zheng ◽

Shira Doron ◽

Nhan Do ◽

...

Keyword(s):

Disease Severity ◽

Severe Disease ◽

Supplemental Oxygen ◽

Case Definition ◽

Vaccination Status ◽

P Value ◽

Case Definitions ◽

Pandemic Severity ◽

Vaccine Availability ◽

The Impact

Abstract Importance: Since the early days of the pandemic, COVID-19 hospitalizations have been used as a measure of pandemic severity. However, case definitions do not include assessments of disease severity, which may be impacted by prior vaccination.Objective: To measure how the severity of respiratory disease changed among inpatients with documented SARS-CoV-2 infection and to measure the impact of vaccination status on these trends, in order to evaluate the accuracy of the metric of “hospitalization plus a positive SARS-CoV-2 test” for tracking pandemic severity.Design: Retrospective cohort of inpatients with laboratory-confirmed SARS-CoV-2. All data were obtained from electronic health records.Setting: Multi-center, nationwide study conducted in the healthcare system of the US Department of Veterans Affairs (VA) from March 1, 2020, through June 30, 2021.Participants: All VA patients admitted to a VA hospital with a laboratory-confirmed SARS-CoV-2 infection within the 14-days prior to admission or during the hospital admission.Main Outcome: Moderate-to-severe COVID-19 disease, defined by use of any supplemental oxygen or documented SpO2 <94%, during an inpatient hospitalization between one day before and two weeks after a positive SARS-CoV-2 test.Exposure: SARS-CoV-2 vaccination status at the time of hospitalization. Patients were regarded as fully vaccinated starting 14 days after receiving the second of a 2-dose regimen or 14 days after receipt of a single-dose vaccine.Results: Among 47,742 admissions in 38,508 unique patients with laboratory-confirmed SARS-CoV-2, N=28,731 met the criteria for moderate-to-severe COVID-19. The proportion with moderate-to-severe disease prior to widespread vaccine availability was 64.0% (95% CI, 63.1-64.9%) versus 52.0% in the later period (95% CI, 50.9-53.2%), p-value for non-constant effect, <0.001. Disease severity in the vaccine era among hospitalized patients was lower among both unvaccinated (55.0%, 95% CI, 53.7-56.4%) and vaccinated patients (42.6%, 95% CI, 40.6-44.8%).Conclusions and Relevance: The proportion of hospitalizations that are due to severe COVID-19 has changed with vaccine availability, thus, increasing proportions of mild and asymptomatic cases are included in hospitalization reporting metrics. The addition of simple measures of disease severity to the case definition of a SARS-CoV-2 hospitalization is a straightforward and objective change that should improve the value of the metric for tracking SARS-CoV-2 disease burden.

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Use of nasal pillows in amiotrophic lateral sclerosis (ALS) patients with high non invasive ventilation (NIV) dependence

10.1183/13993003.congress-2018.pa2381 ◽

2018 ◽

Author(s):

Luana Focaraccio ◽

Giulia Rovere ◽

Roberta Di Tria ◽

Renza Ambrosanio ◽

Elisa Clivati ◽

...

Keyword(s):

Invasive Ventilation ◽

Non Invasive ◽

Non Invasive Ventilation ◽

Als Patients ◽

Lateral Sclerosis

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NIPPV: Prevalence, Approach and Barriers to Use at Canadian ALS Centres

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100009653 ◽

2010 ◽

Vol 37 (1) ◽

pp. 54-60 ◽

Cited By ~ 24

Author(s):

Benjamin R. Ritsma ◽

Michael J. Berger ◽

David A. Charland ◽

Michael A. Khoury ◽

Joel T. Phillips ◽

...

Keyword(s):

Positive Pressure Ventilation ◽

Invasive Mechanical Ventilation ◽

Positive Pressure ◽

Optimal Care ◽

Non Invasive ◽

Morning Headache ◽

Barriers To Use ◽

Als Patients ◽

Descriptive Survey ◽

Lateral Sclerosis

Objective:The purpose of this study was to evaluate Canadian amyotrophic lateral sclerosis (ALS) centres with respect to: 1) the prevalence of Non-invasive positive pressure ventilation (NIPPV) and invasive mechanical ventilation via tracheostomy (TV) utilization, 2) the approach to NIPPV use, focusing upon the currently employed initiation criteria and 3) the barriers influencing NIPPV administration.Methods:A descriptive survey research design aimed to obtain quantitative data and open-ended responses from an active physician at each of the 15 multidisciplinary Canadian ALS centres.Results:The principal findings of this study were: 1) NIPPV and TV are used in 18.3% and 1.5% of patients at Canadian ALS centres, respectively, 2) symptoms of respiratory insufficiency, namely orthopnea (clinical significance rated at 9.00/10 ± 1.48), dyspnea (8.27 ± 1.95) and morning headache (7.55 ± 1.21) are the most significant indicators for NIPPV initiation, 3) the primary barriers to NIPPV utilization are patient intolerance (70% of centres) and inaccessibility of respirologists and ventilation technologists (50% of centres).Conclusions:Variability in NIPPV use has an impact upon the management of Canadian ALS patients. The establishment of more definitive NIPPV initiation criteria, emphasizing respiratory symptoms, and the attenuation of barriers to NIPPV use should be targeted so as to ensure optimal care for all ALS patients.

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POS1233 USE OF BARICITINIB AND TOCILIZUMAB FOR THE TREATMENT OF MODERATE TO SEVERE COVID-19 IN HOSPITALIZED PATIENTS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3122 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 899.3-900

Author(s):

D. X. Xibille Friedmann ◽

S. M. Carrillo Vazquez

Keyword(s):

Hospital Stay ◽

Demographic Data ◽

Severe Disease ◽

Clinical Status ◽

Supplemental Oxygen ◽

Janus Kinase ◽

Oral Drug ◽

Assisted Mechanical Ventilation ◽

Non Invasive ◽

High Flow Oxygen

Background:SARS-CoV2 infection and COVID-19 associated pneumonia are associated to a dysregulated inflammatory response known as cytokine storm and the use of cytokine inhibitors, especially those targeting IL-6, has been proposed as a therapeutic alternative in these patients. Janus Kinase (JAK) inhibitors in combination to the antiviral Remdesivir have shown evidence of reducing recovery time and accelerating improvement in clinical status among patients with COVID-19, notably among those receiving high-flow oxygen or noninvasive ventilation1.Objectives:To describe the outcomes associated with the use of Tocilizumab (TCZ) and Baricitinib (Bari) in patients hospitalized for COVID-19.Methods:Descriptive study nested in a cohort. Patients from the General Hospital of Cuernavaca who were hospitalized due to COVID-19 associated pneumonia and/or respiratory failure requiring supplemental oxygen or invasive/non-invasive assisted mechanical ventilation (AMV) were analyzed. All patients had a positive RT-PCR upon hospital admission, sampled under standardized conditions. The clinical and demographic data of the patients upon hospitalization were obtained from the instrument on a specific platform (SISVER) used on a national basis. Severe disease was considered as the need for any type of AMV. TCZ was used at 400-600 mg in two infusions (baseline and at 72 hours), Bari at 4 mg daily for 14 days. The outcomes were the need for AMV, death, or discharge. A p≤ 0.05 was considered statistically significant.Results:Data from 404 SARS-CoV2 positive patients were analyzed. 269 (65.9%) were men. The mean age of the patients was 57.5 years (18-94; SD 15.3). 59.6% of the patients had one or more comorbidities (Diabetes and Hypertension in most cases [31.8%]). 22.8% of the patients had a history of current or previous smoking. Mean hospital stay was 7.4 days (1-36; SD 5.9). 15.9% of the patients required invasive or non-invasive AMV. 11 patients were treated with TCZ and 30 patients received treatment with Baricitinib. 5 of the 11 patients with TCZ required AMV (all but one were intubated), but only 6 of the 30 (20%) with Bari required AMV (all except one received CPAP). The use of Baricitinib was correlated with a reduction in the use of AMV (p 0.01). 6 patients with TCZ were discharged due to improvement and 5 died. In the case of Bari, 19 improved and were discharged and 11 died, significantly correlating with lower mortality (p 0.05). The use TCZ was not associated with a reduced hospitalization (50% remained hospitalized for more than 10 days) but patients receiving Baricitinib had significantly shorter hospital stays (86.6% had a hospital stay of less than 10 days) than those receiving TCZ (p<0.0001).Conclusion:The patients that were treated with Baricitinib and required AMV most often improved with non-invasive CPAP while the majority of the patients under AMV receiving TCZ were treated with an invasive mode, although this was due in part to the fact that TCZ can be employed intravenously and is more practical for intubated patients in whom oral drug administration is limited. Baricitinib was significantly associated with a better outcome (hospital discharge due to improvement) and a significantly shorter hospital stay.References:[1]Kalil, A.C.; Patterson, T.F.; Mehta, A.K.; Tomashek, K.M.; Wolfe, C.R.; Ghazaryan, V.; Marconi, V.C.; Ruiz-Palacios, G.M.; Hsieh, L.; Kline, S.; et al. Baricitinib plus Remdesivir for Hospitalized Adults with Covid-19. N. Engl. J. Med. 2020.Acknowledgements:Thanks to B. Flores and M. Hernandez for their help with data collection.Disclosure of Interests:Daniel Xavier Xibille Friedmann Speakers bureau: Lilly, Abbvie, Paid instructor for: Lilly, Abbvie, Consultant of: Pfizer, Lilly, Sandra Miriam Carrillo Vazquez Speakers bureau: Roche, Novartis, Paid instructor for: Roche, Janssen, Consultant of: Roche, Janssen

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Atopic dermatitis: clinical phenotypes, persistence and comorbidity

CHILD`S HEALTH ◽

10.22141/2224-0551.16.4.2021.236903 ◽

2021 ◽

Vol 16 (4) ◽

pp. 265-269

Author(s):

O.P. Volosovets ◽

S.P. Kryvopustov ◽

O.V. Mozyrskaya

Keyword(s):

Atopic Dermatitis ◽

Family History ◽

High Risk ◽

Age Of Onset ◽

Severe Disease ◽

Respiratory Allergy ◽

Severe Atopic Dermatitis ◽

Respiratory Allergies ◽

Duration Of Breastfeeding ◽

The Impact

Background. Atopic dermatitis is the most common skin disease among children. Its natural history is heterogeneous with a difference in age of manifestation, localization of lesions, severity, sensitization profiles, presence of comorbid atopic conditions, and longitudinal trajectories of disease progression. The study aimed to assess the impact of factors such as the onset of the disease, atopic family history, presence of concomitant allergic pathology, duration of breastfeeding on the course of the disease and the development of respiratory allergies, and the impact of environmental factors that aggravate the disease. Materials and methods. Children with atopic dermatitis (n = 88) were included in the study from September 2020 to April 2021 in Kyiv, Ukraine. Symptoms of respiratory allergy in the history of patients have been documented as comorbidity. Results. Total of 88 patients at the time of examination presented with manifestations of atopic dermatitis in the form of a rash, dry skin, scaling, itching, lichenization. The disease severity was significantly related to the age of onset: children with early phenotype had more severe disease (OR = 16.261; 2.056–127.911). There was no statistically significant association of early phenotype of atopic dermatitis with the development of concomitant allergic diseases (OR = 1.813; 0.415–7.916). A severe course was observed in the group of children with atopic family history (OR = 2.750; 1.123–6.735). Children with severe atopic dermatitis had a high risk of concomitant respiratory allergy (OR = 5.604; 1.863–16.863). The duration of breastfeeding did not impact the severity of atopic dermatitis (OR = 0.778; 0.119–5.100) and the risk of concomitant atopic diseases (OR = 1.417; 0.444–4.521). The course of atopic dermatitis was influenced by seasonality in 76 children (86.4 %): the condition of the skin deteriorated in the winter. Contact with the animal was associated with exacerbation of atopic dermatitis in 4 (4.5 %) children, food ingestion — in 20 children (25 %), pollen trees and grasses caused exacerbation in 5 children (5.7 %). Conclusions. Children with a severe phenotype of atopic dermatitis are at risk of developing respiratory allergies. Patients with atopic dermatitis who develop symptoms at the age of under 2 years, as well as those with an atopic family history, are at high risk of developing a severe disease phenotype.

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Epidemiology of Amyotrophic Lateral Sclerosis in the Republic of Cyprus: A 25-Year Retrospective Study

Neuroepidemiology ◽

10.1159/000477126 ◽

2017 ◽

Vol 48 (1-2) ◽

pp. 79-85 ◽

Cited By ~ 5

Author(s):

Christiana A. Demetriou ◽

Petros M. Hadjivasiliou ◽

Kleopas A. Kleopa ◽

Yiolanda P. Christou ◽

Eleni Leonidou ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Age Of Onset ◽

Age At Onset ◽

Positive Family History ◽

Temporal Trends ◽

Male Predominance ◽

The Republic ◽

Republic Of Cyprus ◽

Als Patients ◽

Lateral Sclerosis

Introduction: Amyotrophic lateral sclerosis (ALS) is a rare, rapidly progressive neurodegenerative disease. Despite wide variability in the incidence and prevalence of ALS, there is evidence of positive temporal trends and an increase in incidence with age. The aim of this study was to conduct a detailed epidemiological investigation of ALS in Cyprus. Methods: All registered Cypriot ALS patients in the Republic of Cyprus from January 1985 until December 2014 were included. Socio-demographic information was extracted from patient files. Results: The study identified 179 ALS patients, of whom 7 had a positive family history. The mean age at onset was 58.6 years and a slight male predominance was observed. Average annual crude incidence was 1.26 cases/100,000 person-years and at the beginning of 2015, prevalence of ALS was 7.9 cases/100,000 population. Both incidence and prevalence displayed an increasing trend, even after age-standardization of incidence rates. Conclusions: Incidence, prevalence and main socio-demographic characteristics of ALS in Cyprus were similar to those of other European countries, without any geographic clustering of the disease. Additionally, an increased incidence through the years was confirmed. However, observations such as a higher male prevalence and a younger mean age of onset compared to published literature require further investigation.

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