Body composition in amyotrophic lateral sclerosis subjects and its effect on disease progression and survival

Tandan, Rup; Levy, E.; Howard, Diantha B.; Hiser, John; Kokinda, Nathan; Dey, Swatee; Kasarskis, Edward J.

doi:10.1093/ajcn/nqac016

Cited by 10 publications

(13 citation statements)

References 48 publications

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“…On the other hand, ultrasound muscle mass (quadriceps thickness and muscle area) parameters have an inverse relationship with resistance and a direct relationship with estimated muscle mass parameters defined by impedanciometry; this condition demonstrates a relationship of leg muscle mass with variables that estimate whole body muscle mass. This relationship could be useful for measuring active mass and monitoring nutritional treatment in patients with ALS, as Tandan et al demonstrated with DXA values [26]. Muscle quality determined by ultrasonography (Y-X index and echogenicity) had a direct relationship with handgrip strength; this circumstance has been observed in other patients with disease-related malnutrition with an inverse relationship with echogenicity (r = −0.36) and X-Y index (r = −0.18).…”

Section: Discussionmentioning

confidence: 96%

Use of Muscle Ultrasonography in Morphofunctional Assessment of Amyotrophic Lateral Sclerosis (ALS)

López-Gómez,

Izaola-Jauregui,

Almansa-Ruiz

et al. 2024

Nutrients

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Amyotrophic lateral sclerosis (ALS) is a progressive disease with a high prevalence of malnutrition that can influence prognosis. The main objective of this study is to compare the validity of muscle ultrasonography in the diagnosis of malnutrition and the prognosis of patients with ALS. Methods: This is a prospective observational study that analyzes the nutritional status of patients at the beginning of nutritional monitoring. The morphofunctional assessment included the examination of anthropometric variables such as weight, height, body mass index (BMI), arm circumference, and calf circumference. Additionally, electrical bioimpedanciometry (BIA) was used to measure electrical parameters and estimate other relevant metrics. Muscle ultrasonography® (quadriceps rectus femoris (QRF)) assessed muscle mass parameters, including muscle area index (MARAI), anteroposterior diameter of the QRF (Y-axis) (cm), transverse diameter of the QRF (X-axis) (cm), and the sum of the quadriceps thickness (RF+VI) (cm), as well as muscle quality parameters such as echogenicity and the Y–X index. Results: A total of 37 patients diagnosed with amyotrophic lateral sclerosis (ALS) were included in this study. Of these patients, 51.4% were men. The mean age was 64.27 (12.59) years. A total of 54.1% of the patients had a bulbar onset of amyotrophic lateral sclerosis, and 45.9% had spinal onset. The percentage of subjects with malnutrition diagnosed by the Global Leadership Initiative on Malnutrition (GLIM) criteria was 45.9% of patients. There was a direct correlation between muscle mass parameters assessed by muscle ultrasonography (RF+VI) and active mass markers measured by bioimpedanciometry (body cellular mass index (BCMI) (r = 0.62; p < 0.01), fat-free mass index (FFMI) (r = 0.75; p < 0.01), and appendicular skeletal mass index (ASMI) (r = 0.69; p < 0.01)). There was a direct correlation between echogenicity and resistance (r = 0.44; p = 0.02), as well as between the fat-free mass index and the Y–X index (r = 0.36; p = 0.14). Additionally, there was a negative correlation between echogenicity and BCMI (r = −0.46; p < 0.01) and ASMI (r = 0.34; p = 0.06). Patients with low quadriceps thickness (male < 2.49 cm; female < 1.84 cm) showed an increased risk of hospital admission adjusted by age, sex, and presence of dysphagia (OR: 7.84 (CI 95%: 1.09–56.07); p-value = 0.04), and patients with low-quality mass (Y–X index < 0.35) had a higher risk of hospital admission adjusted by age, sex, and presence of dysphagia (OR: 19.83 (CI 95%: 1.77–222.46); p-value = 0.02). Conclusions: In patients with ALS, ultrasonography echogenicity was inversely related to BCMI, FFMI, and ASMI, and the Y–X index was directly related to FFMI. The lowest quartiles of quadriceps thickness and Y–X index are risk factors for hospital admission.

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Section: Discussionmentioning

confidence: 96%

Use of Muscle Ultrasonography in Morphofunctional Assessment of Amyotrophic Lateral Sclerosis (ALS)

López-Gómez,

Izaola-Jauregui,

Almansa-Ruiz

et al. 2024

Nutrients

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“…20 Despite the persistently elevated mortality rate associated with the procedure in this population, there continues to be a paucity of strong data to support useful and practical nutritional surrogates to monitor longitudinally in ALS patients. 31,32 More recent approaches to monitor for malnutrition have focused on serial measurements of body fat in order to monitor daily energy expenditure as well as hydration. Both factors, when at a deficit, have been associated with more rapid progression of disease in ALS patients.…”

Section: Discussionmentioning

confidence: 99%

Outcomes after intervention for enteral nutrition in patients with amyotrophic lateral sclerosis in multidisciplinary clinics

Fullam,

Hunt,

Han

et al. 2024

Muscle and Nerve

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Introduction/AimsPatients with amyotrophic lateral sclerosis (ALS) are susceptible to malnutrition, with appropriate management of nutritional interventions an active area of investigation. We sought to determine the impact of gastrostomy tube placement in ALS patients, exploring the correlation between forced vital capacity (FVC), malnutrition, and perioperative complications.MethodsA retrospective review was performed of clinically diagnosed ALS patients treated at two multidisciplinary clinics (University of Kansas, University of Nebraska) from January 2009 to September 2020 who were referred for gastrostomy. Data collected included demographics, disease characteristics, and key gastrostomy related dates/outcomes.ResultsTwo hundred thirty‐nine patients were included with a median age of 65 years and median of 589 days from symptom onset to gastrostomy (interquartile range, 404–943). The population was predominantly Non‐Hispanic White with bulbar‐onset ALS. 30‐day mortality was 4% and 30‐day morbidity was 13%. Weight loss, body mass index, and predicted FVC at placement showed no increased 30‐day morbidity or mortality association. Bulbar‐onset ALS patients exhibited higher overall mortality postplacement than limb onset (odds ratio: 1.85, 95% confidence interval: 1.03–3.33). There was a 5% incidence of symptoms suggestive of refeeding syndrome.DiscussionRates of major/minor complications and 30‐day mortality related to gastrostomy placement in our population were similar compared with prior studies in ALS. The lack of difference in outcomes based on FVC at procedure may suggest this is not predictive of outcome, or perhaps, high‐quality perioperative respiratory management. Alternative reasons may account for the increased morbidity and mortality of gastrostomy placement in the ALS population.

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“…DXA is useful in evaluating changes in diseases such as the muscular dystrophies and congenital myotonic dystrophy as the patients experience progressive loss of muscle fibers, with fat and connective tissue replacement 9–14 . A recent longitudinal study suggested that DXA can serve as a biomarker for body composition in evaluating progression of disease in amyotrophic lateral sclerosis (ALS) 8,15 . This study presents a thorough examination of body composition using DXA scans in patients affected by MSP1.…”

Section: Introductionmentioning

confidence: 99%

“…[9][10][11][12][13][14] A recent longitudinal study suggested that DXA can serve as a biomarker for body composition in evaluating progression of disease in amyotrophic lateral sclerosis (ALS). 8,15 This study presents a thorough examination of body composition using DXA scans in patients affected by MSP1. The study's rationale lies in exploring DXA's viability as a marker for lean body and fat mass among patients with MSP1 that might be of use in the clinical and research settings.…”

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confidence: 99%

Cross‐sectional study of patients with VCP multisystem proteinopathy 1 using dual‐energy x‐ray absorptiometry

Columbres,

Luu,

Nguyen

et al. 2024

Muscle and Nerve

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Introduction/AimsVCP multisystem proteinopathy 1 (MSP1), encompassing inclusion body myopathy (IBM), Paget's disease of bone (PDB) and frontotemporal dementia (FTD) (IBMPFD), features progressive muscle weakness, fatty infiltration, and disorganized bone structure in Pagetic bones. The aim of this study is to utilize dual‐energy x‐ray absorptiometry (DXA) parameters to examine it as a biomarker of muscle and bone disease in MSP1.MethodsDXA scans were obtained in 28 patients to assess body composition parameters (bone mineral density [BMD], T‐score, total fat, and lean mass) across different groups: total VCP disease (n = 19), including myopathy without Paget's (“myopathy”; n = 12) and myopathy with Paget's (“Paget”; n = 7), and unaffected first‐degree relatives serving as controls (n = 6).ResultsIn the VCP disease group, significant declines in left hip BMD and Z‐scores were noted versus the control group (p ≤ .03). The VCP disease group showed decreased whole body lean mass % (p = .04), and increased total body fat % (p = .04) compared to controls. Subgroup comparisons indicated osteopenia in 33.3% and osteoporosis in 8.3% of the myopathy group, with 14.3% exhibiting osteopenia in the Paget group. Moreover, the Paget group displayed higher lumbar L1‐L4 T‐score values than the myopathy group.DiscussionIn MSP1, DXA revealed reduced bone and lean mass, and increased fat mass. These DXA insights could aid in monitoring disease progression of muscle loss and secondary osteopenia/osteoporosis in MSP1, providing value both clinically and in clinical research.

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Body composition in amyotrophic lateral sclerosis subjects and its effect on disease progression and survival

Cited by 10 publications

References 48 publications

Use of Muscle Ultrasonography in Morphofunctional Assessment of Amyotrophic Lateral Sclerosis (ALS)

Use of Muscle Ultrasonography in Morphofunctional Assessment of Amyotrophic Lateral Sclerosis (ALS)

Outcomes after intervention for enteral nutrition in patients with amyotrophic lateral sclerosis in multidisciplinary clinics

Cross‐sectional study of patients with VCP multisystem proteinopathy 1 using dual‐energy x‐ray absorptiometry

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