Gait Performance and Use of Mental Imagery as a Measure of Disease Progression in Amyotrophic Lateral Sclerosis

Ferfoglia, Ruxandra Iancu; Barras, Anne-Chantal Héritier; Pollak, Pierre; Janssens, Jean–Paul; Allali, Gilles

doi:10.1159/000444052

Cited by 5 publications

(3 citation statements)

References 23 publications

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“…We used the TUG throughout the course of follow-up visits, and it was feasible to perform among the majority of ALS patients (54/68; 79%), regardless of whether they were bulbar or non-bulbar ALS patients. As a reliable marker of gait monitoring, the TUG is appropriate for clinical trials in ALS [6] and shows a linear increase over the disease course that correlates with falls [7] and disability [5]. Deterioration of functional status, mainly driven by a decline in gait, is a key indicator of disease progression and mortality in ALS [8,9].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…We prospectively collected data on prevalent and incident ALS patients in the Centre for ALS and Related Disorders in Geneva University Hospitals between June 2012 and September 2016. All the patients with confirmed ALS according to the El Escorial criteria (definite, probable, or possible ALS) were recruited as previously described [5]. The exclusion criteria were the presence of other neurological or orthopedic disorders interfering with gait.…”

Section: Methodsmentioning

confidence: 99%

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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%

Section: Methodsmentioning

confidence: 99%

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

et al. 2021

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“…The TUG also shows high interrater, intrarater, and test-retest reliability, 7,19 and it has been used to evaluate functional mobility in elderly people and patients with various diseases. 20-23 The TUG has been shown to have high sensitivity and specificity for community-dwelling adults who are at risk of falls. 24 The TUG scores in patients with dizziness had a significantly positive correlation with age in the present study, consistent with previous studies.…”

Section: Discussionmentioning

confidence: 99%

Effects of Peripheral Vestibular Dysfunction on Dynamic Postural Stability Measured by the Functional Reach Test and Timed Up and Go Test

Nishi

Kamogashira

Fujimoto

et al. 2017

Ann Otol Rhinol Laryngol

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Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice

Hummel

Chan

Hoven

et al. 2018

The American Journal of Pathology

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Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal disease characterized by muscular atrophy because of loss of upper and lower motor neurons. Histopathologically, most patients with ALS have abnormal cytoplasmic accumulation and aggregation of the nuclear RNA-regulating protein TAR DNA-binding protein 43 (TDP-43). Pathogenic mutations in the TARDBP gene that encode TDP-43 have been identified in familial ALS. We have previously reported transgenic mice with neuronal expression of human TDP-43 carrying the pathogenic A315T mutation (iTDP-43 mice), presenting with early-onset motor deficits in adolescent animals. Here, we analyzed aged iTDP-43 mice, focusing on the spatiotemporal profile and progression of neurodegeneration in upper and lower motor neurons. Magnetic resonance imaging and histologic analysis revealed a differential loss of upper motor neurons in a hierarchical order as iTDP-43 mice aged. Furthermore, we report progressive gait problems, profound motor deficits, and muscle atrophy in aged iTDP-43 mice. Despite these deficits and TDP-43 pathologic disorders in lower motor neurons, stereological analysis did not show cell loss in spinal cords. Taken together, neuronal populations in aging iTDP-43 mice show differential susceptibility to the expression of human TDP-43.

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Gait Performance and Use of Mental Imagery as a Measure of Disease Progression in Amyotrophic Lateral Sclerosis

Cited by 5 publications

References 23 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

Effects of Peripheral Vestibular Dysfunction on Dynamic Postural Stability Measured by the Functional Reach Test and Timed Up and Go Test

Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice

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