Measures and markers in Amyotrophic Lateral Sclerosis

In ALS it would be expected that quality of life (QoL) would be decreased. However, studies to date show diverging results. Our study focuses on how ALS affects QoL on the different domains of the SF-36 cross-sectionally and during progression. The method used was a prospective cohort study, with assessments at baseline, at six months, and at one year. Patients were included with possible, probable or definite ALS according to the revised El Escorial criteria and were between 30 and 70 years of age. ALS functional rating scale was used to establish disease status, SF-36 as QoL scale. At baseline 73 completed ALSFRS forms were available and 62 completed SF-36 forms. The ALSFRS showed disease progression. SF-36 scores showed lower QoL scores of persons with ALS compared to the general population both in cross-sectional and longitudinal aspects in the domains of Physical Functioning, Role Physical, and Social Functioning, but similar compared with the general population in the Mental Health and Role Emotional domains. This study shows deteriorating physical health but stable mental health, thereby illustrating the diverging correlations between ALS severity and HRQoL. The diverging pattern of physical and mental health suggests a frame-shift in the experience of HRQoL.

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“…Reliability and validity of the ALSFRS are good (19,20) and it is the preferred measure for disease severity in ALS (21).…”

Section: Instrumentsmentioning

confidence: 99%

Cross‐sectional and longitudinal correlations between disease progression and different health‐related quality of life domains in persons with amyotrophic lateral sclerosis

Groot

Post

Heuveln

et al. 2007

Amyotrophic Lateral Sclerosis

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“…The study characteristics are summarized in Table I. Measures of disease course included survival and changes in functional measures including the Amyotrophic Lateral Functional Rating Scale (ALSFRS), percent predicted Vital Capacity (%VC) and Maximum Voluntary Isometric Contraction (MVIC) of limb muscle groups (10). Concomitant medications received by participants at study entry and at subsequent visit were recorded.…”

Section: Datasetsmentioning

confidence: 99%

Medications and laboratory parameters as prognostic factors in amyotrophic lateral sclerosis

Qureshi¹,

Shui

DiBernardo³

et al. 2008

Amyotrophic Lateral Sclerosis

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We sought to examine the influence of medication usage and laboratory measurements on disease progression in amyotrophic lateral sclerosis (ALS). A database of 596 volunteers with ALS was generated from three clinical trials and one observational study. Disease course was measured by survival and three functional measures: the ALS Functional Rating Scale (ALSFRS), Vital Capacity (VC) and Maximum Voluntary Isometric Contraction (MVIC). Survival modeling was performed using Cox proportional hazards regression. The association of medication or laboratory measurements with disease progression was determined using a random effects model. In the multivariate analysis, survival was shorter in participants who took aspirin (HR =1.93, p =0.046); NSAIDs (HR =1.51, p =0.054); had low blood chloride (HR =0.76, p =0.020) or high bicarbonate levels (HR =1.37, p =0.006). Individuals who took calcium had better survival (HR =0.37, p =0.008) and a slower rate of decline of MVIC arm megascore (p =0.033). Vital capacity declined faster in individuals with lower serum chloride (p<0.0001), or higher bicarbonate (p =0.002) levels and those taking paracetamol (acetaminophen) (p =0.035). We conclude that aspirin or NSAID use may shorten survival in ALS, while calcium use may prolong survival. Our results support a need to further explore the role of neuroinflammation in the pathogenesis of ALS.

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“…DMD: As might be expected, little compensatory change is EA size is needed. (15,61), considerable atrophy of many and often most fibers (1,61), led by profound downregulation (z-scores from over 11 to under -0.2, i.e., essentially off): ACTA1 (skeletal actin), TNNC2 (troponin C, fast), MYLPF (myosin light chain, fast) and TTN (titin). Also notable are all classic troponins, both fast and slow isoforms, consistent with ACTA1 loss.…”

Section: Exact Size Handlingmentioning

confidence: 99%

“…Indeed, a remarkably large number of hypotheses that 75 have been proposed (e.g., reviewed in 15), and successful biomarkers for ALS have been of 76 limited value, but include the hallmark gradual loss of motor units as measured by 77 electrodiagnosis, changes in several protein concentrations in cerebrospinal fluid (67), isometric 78 muscle-joint strength testing to document weakness using a representative collection of 79 strategic joints (15,61), muscle atrophy scales based on images from muscle biopsies (61), and 80…”

Section: Introduction 44mentioning

confidence: 99%

Functional classification of skeletal muscle networks. II. Applications to pathophysiology

Winters

Subramaniam

2012

Journal of Applied Physiology

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In our preceding companion paper (Wang Y, Winters J, Subramaniam S. J Appl Physiol. doi: 10.1152/japplphysiol.01514.2011), we used extensive expression profile data on normal human subjects, in combination with legacy knowledge to classify skeletal muscle function into four models, namely excitation-activation, mechanical, metabolic, and signaling-production model families. In this paper, we demonstrate how this classification can be applied to study two well-characterized myopathies: amyotrophic lateral sclerosis (ALS) and Duchenne muscular dystrophy (DMD). Using skeletal muscle profile data from ALS and DMD patients compared with that from normal subjects, normal young in the case of DMD, we delineate molecular mechanisms that are causative and consequential to skeletal muscle dysfunction. In ALS, our analysis establishes the metabolic role and specifically identifies the mechanisms of calcium dysregulation and defects in mitochondrial transport of materials as important for muscle dysfunction. In DMD, we illustrate how impaired mechanical function is strongly coordinated with other three functional networks, resulting in transformation of the skeletal muscle into hybrid forms as a compensatory mechanism. Our functional models also provide, in exquisite detail, the mechanistic role of myriad proteins in these four families in normal and disease function.

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Measures and markers in Amyotrophic Lateral Sclerosis

Cited by 15 publications

References 123 publications

Cross‐sectional and longitudinal correlations between disease progression and different health‐related quality of life domains in persons with amyotrophic lateral sclerosis

Cross‐sectional and longitudinal correlations between disease progression and different health‐related quality of life domains in persons with amyotrophic lateral sclerosis

Medications and laboratory parameters as prognostic factors in amyotrophic lateral sclerosis

Functional classification of skeletal muscle networks. II. Applications to pathophysiology

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