Journal
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
Volume 89, Issue 10, Pages 1016-1023Publisher
BMJ PUBLISHING GROUP
DOI: 10.1136/jnnp-2017-317887
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Funding
- Motor Neurone Disease Research Institute of Australia
- University of Queensland [UQECR1719385]
- Australian National Health and Medical Research Council [1078901, 1113400]
- Netherlands ALS Foundation (MEASURE study)
- MND and Me Foundation
- Royal Brisbane and Women's Hospital Foundation
- Queensland Brain Institute
- Australian Institute for Bioengineering and Nanotechnology at the University of Queensland
- Australian and New Zealand Association of Neurologists Education
- Research Foundation/National Health and Medical Research Council Postgraduate Scholarship
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Objective To determine the prevalence of hypermetabolism, relative to body composition, in amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and survival. Methods Fifty-eight patients with clinically definite or probable ALS as defined by El Escorial criteria, and 58 age and sex-matched control participants underwent assessment of energy expenditure. Our primary outcome was the prevalence of hypermetabolism in cases and controls. Longitudinal changes in clinical parameters between hypermetabolic and normometabolic patients with ALS were determined for up to 12 months following metabolic assessment. Survival was monitored over a 30-month period following metabolic assessment. Results Hypermetabolism was more prevalent in patients with ALS than controls (41% vs 12%, adjusted OR=5.4; p<0.01). Change in body weight, body mass index and fat mass (%) was similar between normometabolic and hypermetabolic patients with ALS. Mean lower motor neuron score (SD) was greater in hypermetabolic patients when compared with normometabolic patients (4 (0.3) vs 3 (0.7); p=0.04). In the 12 months following metabolic assessment, there was a greater change in Revised ALS Functional Rating Scale score in hypermetabolic patients when compared with normometabolic patients (-0.68 points/month vs -0.39 points/month; p=0.01). Hypermetabolism was inversely associated with survival. Overall, hypermetabolism increased the risk of death during follow-up to 220% (HR 3.2, 95% CI 1.1 to 9.4, p=0.03). Conclusions and relevance Hypermetabolic patients with ALS have a greater level of lower motor neuron involvement, faster rate of functional decline and shorter survival. The metabolic index could be important for informing prognosis in ALS.
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