The ALSFRSr predicts survival time in an ALS clinic population

Kaufmann, Petra; Levy, Gilbero; Thompson, John L.P.; DelBene, M. L.; Battista, Vanessa; Gordon, Paul H.; Rowland, Lewis P.; Levin, B.; Mitsumoto, Hiroshi

doi:10.1212/01.wnl.0000148648.38313.64

Cited by 220 publications

(185 citation statements)

References 27 publications

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“…The change in body weight for values above the recommended, according to the age group and stature of the Brazilian population, has been growing in recent decades. When degenerative diseases affect individuals older than 45 years of age, being overweight and obese are commonlyobserved symptoms 16,19 . Another reason could be the inclusion of patients in the early stages of the disease where the respiratory functions were not affected; a fact that would help the preservation of body fat reserves.…”

Section: Discussionmentioning

confidence: 99%

Assessment and nutrition education in patients with amyotrophic lateral sclerosis

Almeida¹,

Stanich

Salvioni

et al. 2016

Arq. Neuro-Psiquiatr.

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Neurological patients with amyotrophic lateral sclerosis (ALS)often deteriorate to a worsening nutritional status. The aim of this study was to compare the nutritional status and food intake after nutrition education in patients with ALS. Clinical, anthropometric and functional variables were analyzed. Fifty-three patients were monitored at an early stage of the disease. The average score on the functionality scale was 33 points. Initially only 3.8% were classified as low body weight. After three months, 50% showed significant variation in anthropometric measures related to muscle mass and body fat reserves without association with clinical variables. After nutritional guidance, there was an increase in the intake of all food groups, especially the dairy group (p <0.05).The change of the nutritional status occurs early in patients with amyotrophic lateral sclerosis, even in those previously eutrophic or over weight. There was an increase in food intake after nutritional guidance according to the food guide adapted to the Brazilian population.

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Section: Discussionmentioning

confidence: 99%

Assessment and nutrition education in patients with amyotrophic lateral sclerosis

Almeida¹,

Stanich

Salvioni

et al. 2016

Arq. Neuro-Psiquiatr.

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“…The maximum score of 12 indicates intact bulbar function, whereas values below 12 indicate bulbar impairment. The ALSFRS-R has been used in clinical trials due to its sensitivity to disease progression (Kaufmann et al, 2007;Kimura et al, 2006;Kollewe et al, 2008) and survival (Gordon & Cheung, 2006;Kaufmann et al, 2005). However, this assessment tool is an ordinal scale and is likely insensitive to small differences in disease presentation (Rosenfeld & Jackson, 2013) or early stages of the disease (Voustianiouk et al, 2008).…”

mentioning

confidence: 99%

Speech Movement Measures as Markers of Bulbar Disease in Amyotrophic Lateral Sclerosis

Shellikeri

Green

Kulkarni

et al. 2016

J Speech Lang Hear Res

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Purpose: The goal of this study was to identify the effects of amyotrophic lateral sclerosis (ALS) on tongue and jaw control, both cross-sectionally and longitudinally. The data were examined in the context of their utility as a diagnostic marker of bulbar disease. Method: Tongue and jaw movements were recorded crosssectionally (n = 33 individuals with ALS, 13 controls) and longitudinally (n = 10 individuals with ALS) using a threedimensional electromagnetic articulography system during the production of the sentence Buy Bobby a puppy. The movements were examined for evidence of changes in size, speed, and duration and with respect to disease severity and time in the study.Results: Maximum speed of tongue movements and movement durations were significantly different only at an advanced stage of bulbar ALS compared with the healthy control group. The longitudinal analysis revealed a reduction in tongue movement size and speed with time at early stages of disease, which was not seen cross-sectionally. As speaking rate declined, tongue movements decreased in maximum speed, whereas jaw movements increased in maximum speed. Conclusions: Longitudinal analyses of sentence-level kinematic data show their sensitivity to early bulbar impairment. A change in articulatory kinematics can serve as a useful diagnostic marker for bulbar ALS and to track bulbar disease progression in a clinical setting.

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“…The site where stem cells were injected and probably the time the MRI-FA follow-up was performed may explain these positive changes in the FA only in internal capsule and corona radiata after the procedure. In the present series, clinical parameters in transplanted patients were followed up by the ALSFRS-R [51], since is the most widely used and extensively validated global scale for assessing motor function in ALS. The median baseline ALSFRS-R score was 29 points and at 6 months after transplantation, the median ALSFRS-R score was 28 points.…”

Section: Discussionmentioning

confidence: 99%

“…The ALS Functional Rating Scale Revised (ALSFRS-R) [51], which is the most widely used and extensively validated global scale for assessing motor function in ALS, was administered to all ALS patients. This scale is weighted toward limb and bulbar function, and gives a total severity score out of 48 possible points.…”

Section: Study Subjectsmentioning

confidence: 99%

Increase of Pyramidal Tract Fractional Anisotropy on MRI after Stem Cell Transplantation in ALS Patients

HR¹,

Garza²

2014

J Neurol Neurophysiol

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Background and Purpose: Amyotrophic lateral sclerosis (ALS) is characterized by the selective death of motor neurons. Cortical neuron loss is associated with axonal degeneration along specific white matter tracts. Fractional anisotropy (FA) determined by Magnetic Resonance (MR) might detect integrity or changes in brain white matter pathways. This study was aimed to analyze pyramidal tract changes in ALS patients submitted to stem cell transplantation into the frontal motor cortices.

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The ALSFRSr predicts survival time in an ALS clinic population

Abstract: In an ALS clinic population, the total Amyotrophic Lateral Sclerosis Functional Rating Scale-revised score at baseline is a strong predictor of death or tracheostomy independently of forced vital capacity and after adjustment for age at baseline, sex, and symptom duration.

Cited by 220 publications

References 27 publications

Assessment and nutrition education in patients with amyotrophic lateral sclerosis

Assessment and nutrition education in patients with amyotrophic lateral sclerosis

Speech Movement Measures as Markers of Bulbar Disease in Amyotrophic Lateral Sclerosis

Increase of Pyramidal Tract Fractional Anisotropy on MRI after Stem Cell Transplantation in ALS Patients

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