Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS

Kimura, F.; Fujimura, C.; Ishida, S.; Nakajima, H.; Furutama, D.; Uehara, H.; Shinoda, K.; Sugino, M.; Hanafusa, T.

doi:10.1212/01.wnl.0000194316.91908.8a

Cited by 448 publications

(389 citation statements)

References 9 publications

(6 reference statements)

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“…Other proposed clinical biomarkers for disease progression include electrical impedance myography, muscle ultrasound, and various imaging modalities (i.e., voxel-based morphometry, diffusion tensor imaging, T2 quantification, positron emission tomography) [83][84][85]. Biochemical markers of underlying neuropathology phosphorylated high molecular weight neurofilament protein (pNFH) or immunological markers are also promising [86].…”

Section: Biomarkersmentioning

confidence: 99%

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Rosenfeld¹,

Strong

2015

Neurotherapeutics

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With the acceleration in our understanding of ALS and the related motor neuron disease has come even greater challenges in reconciling all of the proposed pathogenic mechanisms and how this will translate into impactful treatments. Fundamental issues such as diagnostic definition(s) of the disease spectrum, relevant biomarkers, the impact of multiple novel genetic mutations and the significant effect of symptomatic treatments on disease progression are all areas of active investigation. In this review, we will focus on these key issues and highlight the challenges that confront both clinicians and basic science researchers.

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Section: Biomarkersmentioning

confidence: 99%

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Rosenfeld¹,

Strong

2015

Neurotherapeutics

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“…The rate of deterioration also correlates with survival [8]. An obvious advantage of such a measure is that it is very quick to perform and can even be completed over the telephone or online, making it simple to obtain regular data, even if the patient is unable to return to the clinic for evaluation [5].…”

Section: Introductionmentioning

confidence: 99%

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Rutkove

2015

Neurotherapeutics

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Progressive weakness remains the clinical hallmark of amyotrophic lateral sclerosis (ALS). Accordingly, a variety of tools has been developed to capture this disease feature, including questionnaires, such as the ALS-functional rating scale, strength testing, pulmonary function tests, electrophysiologic measures, including motor unit number estimation, and imaging techniques. Despite this plethora of approaches, there is little agreement as to what measures to use in a given clinical trial or in the clinic during routine patient care. Part of the reason for this uncertainty is that ALS is a remarkably protean disease. Some individuals progress rapidly, others slowly; some patients have considerable upper motor neuron dysfunction, whereas others have little; and there is considerable variation in the sequence of body regions affected, in some the disease beginning in the bulbar musculature and in others in one arm or one leg. Here, I present a variety of basic and more complex clinical measures for potential use in therapeutic trials with the aim of offering a balanced and practical set of recommendations, as well as considerations for future studies.

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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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Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS

Cited by 448 publications

References 9 publications

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

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

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