Multidisciplinary care in amyotrophic lateral sclerosis: a 4-year longitudinal observational study

Sukockienė, Eglė; Ferfoglia, Ruxandra Iancu; Truffert, André; Barras, Anne-Chantal Héritier; Genton, Laurence; Viatte, V.; Leuchter, Igor; Escher, Monica; Horie, Ninon; Poncet, Antoine; Pasquina, Patrick; Adler, Dan; Janssens, Jean–Paul

doi:10.4414/smw.2020.20258

Cited by 13 publications

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“…Considering that the most common cause of death in ALS is weakness of respiratory muscles, one limitation of the TUG might be its inability to capture respiratory dysfunction. Unfortunately, the design of our cohort study [14] and the management of respiratory failure in this cohort limited the possibility of exploring the links between functional respiratory measures, namely, forced vital capacity, maximal inspiratory pressure, or percutaneous oxygen saturation (SpO 2 ), and the results of the TUG. Indeed, the elective regular pulmonary assessments performed according to our study protocol aimed to initiate noninvasive ventilation electively, before patients developed overt respiratory failure, and thus before they became hypercapnic and hypoxemic during the daytime.…”

Section: Discussion/conclusionmentioning

confidence: 99%

Longitudinal Timed Up and Go Assessment in Amyotrophic Lateral Sclerosis: A Pilot Study

et al. 2021

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Progressive loss of walking ability in amyotrophic lateral sclerosis (ALS) has been scarcely studied as a potential predictive factor for survival in motor neuron disease. We aimed to assess the progression of gait decline and its association with mortality in ALS using the Timed Up and Go test (TUG). Patients were followed up prospectively at the Centre for ALS and Related Disorders in Geneva University Hospitals between 2012 and 2016. The TUG was performed at baseline and subsequent evaluations occurred every 3 months. At inclusion, patients were classified as unable to perform the TUG, “slow TUG” (>10.6 s), and “fast TUG” (≤10.6 s). In total, 68 patients with ALS (mean ± SD age: 68.6 ± 11.9 years; 50% female) were included. Baseline TUG was negatively correlated with the total ALSFRS-R score (r = −0.63, p < 0.001). At baseline, ALS patients with bulbar onset performed the TUG faster (9.9 ± 3.7 s) than the non-bulbar ones (17.3 ± 14.9 s, p = 0.008). Thirty of 68 (44%) patients died by the end of the follow-up period. The TUG performance at the first visit did not predict mortality. While we did not find any association with mortality in ALS and gait quantification, the TUG was feasible in a majority of ALS patients, was correlated with functional status, and could be of interest in the follow-up of non-bulbar ALS patients.

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Section: Discussion/conclusionmentioning

confidence: 99%

Longitudinal Timed Up and Go Assessment in Amyotrophic Lateral Sclerosis: A Pilot Study

et al. 2021

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“…This cross-sectional controlled cohort study included adults diagnosed with ALS and participating in the clinical multidisciplinary follow-up of the Geneva University Hospitals (HUG) [16]. They were included at the start of their follow-up at the Geneva University Hospitals, while they had no enteral nutrition nor parenteral nutrition.…”

Section: Methodsmentioning

confidence: 99%

Oral function in amyotrophic lateral sclerosis patients: A matched case–control study

et al. 2021

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Background & aims: Patients with amyotrophic lateral sclerosis (ALS) develop swallowing difficulties with the progression of the disease. The present study aimed at comparing oral function and body composition between ALS patients and healthy controls, and at evaluating which parameters are the most discriminant between both groups. Methods: We included ALS patients at the start of their multidisciplinary follow-up at the Geneva University Hospitals and healthy age-, gender-, and dental status-matched adults. We assessed the severity of the disease through the ALS Functional Rating Scale and the swallowing difficulties through the EAT-10 score. We performed an intraoral examination of the dental status, and measured chewing performance, bite, lip and tongue force, saliva weight, and body composition. Group comparisons were performed with t-tests or ManneWhitney tests as appropriate. Linear discriminant analysis was used to determine the most discriminant parameters between groups. Results: Twenty-six ALS patients (bulbar onset: n ¼ 7, median (IQR) ALS Functional Rating Scale: 37 (11)) were included. The ALS patients had a significantly lower chewing performance (p < 0.001), lip force (p < 0.001), tongue force (p ¼ 0.002), saliva weight (p < 0.004) and fat-free mass index (p < 0.001) as compared to the healthy individuals, and a higher EAT-10 score (p < 0.001). In ALS patients, a low chewing performance was correlated with a low bite (r ¼ À0.45, p < 0.05)) and tongue force (r ¼ À0.59, p < 0.05). The most discriminant parameters between both groups, by order of importance, were chewing performance, fat-free mass index and saliva weight and allowed the calculation of a discriminant function. Conclusion: Compared to healthy controls, ALS patients have significant alterations of oral function and body composition. The most discriminant parameters between both groups were chewing performance, fat-free mass index and saliva volume. It remains to be demonstrated whether oral parameters predict outcome. Clinical Trial registry: clinicaltrials.gov, identifier: NCT 01772888.

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“…There was no difference in survival between the clinic population and the non-clinic population; however, this does not preclude previously reported survival benefit of multidisciplinary care, as the non-clinic population may have received multidisciplinary care through local providers or home health organizations. [26][27][28] With gene therapies now being investigated in clinical trials, 29 the ALS Association could potentially serve as a platform to connect the familial ALS patients among the non-clinic population to the tertiary centers running clinical trials.…”

Section: Discussionmentioning

confidence: 99%

Demographics, clinical characteristics, and prognostic factors of amyotrophic lateral sclerosis in Midwest

et al. 2021

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Introduction/Aims: The Midwest has the highest regional prevalence of self-reported amyotrophic lateral sclerosis (ALS) in the United States, but with limited epidemiological studies. We aimed to explore the characteristics of patients with ALS in the Midwest.Methods: This was a retrospective cohort study of participants with ALS deceased between January, 2010, and September, 2020, registered with the ALS Association Mid-America Chapter. Demographics and clinical variables included gender, race/ethnicity, military status, site of onset, interventions (gastrostomy, non-invasive ventilation, tracheostomy), and visits to ALS Association-registered clinics. Disease characteristics were compared to the National ALS Registry, and survival analysis was performed followed by sample augmentation with historical data to estimate survival with hypothetical censoring. Results:The database included 1447 participants with a mean age at diagnosis of 65.7 ± 11.9 y (>60 y at diagnosis: 72%). The median survival from symptom onset was 28.0 mo (95% confidence limit: 26.3, 29.7); sample augmentation increased this to 41.0 mo (38.5, 43.5). Bulbar onset disease and older age at diagnosis were associated with shorter survival. Participants not followed in ALS-Association registered clinics were more frequently male, had familial onset and tracheostomy. Veterans (N = 298) were older at diagnosis but had similar survival after adjustment for age. Discussions: Our cohort had an older age at onset and more frequent bulbar onset than the National ALS Registry, perhaps reflecting ascertainment biases in each registry. Prospective cohort studies with more clinical and functional data are needed to better characterize ALS in Midwest, veterans, and non-clinic populations, and to optimize care.

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Multidisciplinary care in amyotrophic lateral sclerosis: a 4-year longitudinal observational study

Cited by 13 publications

References 0 publications

Longitudinal Timed Up and Go Assessment in Amyotrophic Lateral Sclerosis: A Pilot Study

Longitudinal Timed Up and Go Assessment in Amyotrophic Lateral Sclerosis: A Pilot Study

Oral function in amyotrophic lateral sclerosis patients: A matched case–control study

Demographics, clinical characteristics, and prognostic factors of amyotrophic lateral sclerosis in Midwest

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