Prognostic significance of body weight variation after diagnosis in ALS: a single-centre prospective cohort study

Shimizu, Toshio; Nakayama, Yuki; Matsuda, Chiharu; Haraguchi, Michiko; Bokuda, Kota; Ishikawa-Takata, Kazuko; Kawata, Akihiro; Isozaki, Eiji

doi:10.1007/s00415-019-09276-2

Cited by 37 publications

(35 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Although we could not control the patients’ energy intake before and after TIV use, there were no significant differences in the duration of enteral nutrition, energy intake, and BMI at the end of follow-up between the two subgroups (Table 1). As previously reported 21 , nutritional intervention in the early stage and resultant weight increase may improve survival before TIV use. However, if the hypermetabolism and weight loss at the early stage are associated with the hypothalamus lesion specific to ALS, one can easily imagine that early nutritional intervention will not easily modify the natural course of ALS with a long-term TIV use.…”

Section: Discussionsupporting

confidence: 58%

“…Second, we could not define the premorbid baseline BMI in each patient. A rapid BMI decline from onset to the time of diagnosis is strongly associated with survival in ALS as well as a post-diagnostic BMI decline 19–21 . Third, we could not evaluate the effect of nutritional intervention in the early stages of ALS.…”

Section: Discussionmentioning

confidence: 99%

“…Third, we could not evaluate the effect of nutritional intervention in the early stages of ALS. Nutritional intervention has a potential effect on weight increase and may improve survival 21 . However, a nutritional therapy regimen has not yet been established, and a completely controlled prospective study for nutritional intervention would be difficult to perform.…”

Section: Discussionmentioning

confidence: 99%

“…For assessment of the nutritional condition, we evaluated patients’ body weight (kg) at the time of diagnosis, at the time of TIV use, and at the end of follow-up. We calculated the BMI (kg/m 2 ) at each point, and annual rate of BMI decline (ΔBMI) from the time of diagnosis to the time of TIV use, using the following formula: (BMI at the time of diagnosis–BMI at the time of TIV use)/time interval from diagnosis to TIV use (year) 20,21 . We classified the patients into two subgroups (those with ΔBMI < 1.7 kg/m 2 /year and those with ≥1.7 kg/m 2 /year) since the mean value of ΔBMI of the total patient cohort was 1.7 kg/m 2 /year.…”

Section: Methodsmentioning

confidence: 99%

“…Widespread emerging fasciculation potential, hyperexcitability of the peripheral motor axons, and hyperexcitability of the motor and sensory cortices are predictors of short-term survival in ALS 12–15 . With regard to the nutritional aspect, low body mass index (BMI) at diagnosis 16–18 , rapid body weight decline 19–21 , and hypermetabolism 22,23 in the early stages typically indicate a short survival time or an earlier need for tracheostomy.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis

Nakayama

Shimizu

Matsuda

et al. 2019

Sci Rep

Self Cite

View full text Add to dashboard Cite

Weight loss is an independent predictor of survival in the early stages of amyotrophic lateral sclerosis (ALS). However, the effects of weight variations on the functional prognosis after tracheostomy and invasive ventilation (TIV) in ALS remain unknown. This prospective cohort study aimed to investigate the relationship between weight loss before TIV and disease progression after TIV in ALS patients. Sixty ALS patients with TIV were enrolled and classified into subgroups based on the rate of decline in body mass index, from onset to TIV utilization (ΔBMI). During follow-up, we assessed the patients for presence of communication impairments, ophthalmoplegia, total quadriplegia, mouth opening disability, and dysuria. We analyzed the relationship between ΔBMI and the communication stage or motor disabilities. The log-rank test showed that patients with a ΔBMI ≥ 1.7 kg/m 2 /year showed a shorter period of preserved communication ability ( p = 0.0001), shorter time to develop ophthalmoplegia ( p = 0.0001), total quadriplegia ( p < 0.0001), mouth opening disability ( p < 0.0001), and dysuria ( p = 0.0455). Cox multivariate analyses showed that a larger ΔBMI was an independent prognostic factor for the early development of ophthalmoplegia ( p = 0.0400) and total quadriplegia ( p = 0.0445). Weight loss in the early stages of ALS predicts disease progression in patients with advanced stages of ALS using TIV.

show abstract

Section: Discussionsupporting

confidence: 58%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis

Nakayama

Shimizu

Matsuda

et al. 2019

Sci Rep

Self Cite

View full text Add to dashboard Cite

show abstract

Update on recent advances in amyotrophic lateral sclerosis

Riva,

Domi,

Pozzi

et al. 2024

J Neurol

View full text Add to dashboard Cite

In the last few years, our understanding of disease molecular mechanisms underpinning ALS has advanced greatly, allowing the first steps in translating into clinical practice novel research findings, including gene therapy approaches. Similarly, the recent advent of assistive technologies has greatly improved the possibility of a more personalized approach to supportive and symptomatic care, in the context of an increasingly complex multidisciplinary line of actions, which remains the cornerstone of ALS management. Against this rapidly growing background, here we provide an comprehensive update on the most recent studies that have contributed towards our understanding of ALS pathogenesis, the latest results from clinical trials as well as the future directions for improving the clinical management of ALS patients.

show abstract

Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions

Bjelica,

Bartels,

Hesebeck-Brinckmann

et al. 2024

J Neurol

View full text Add to dashboard Cite

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the progressive degeneration of both upper and lower motor neurons. A defining histopathological feature in approximately 97% of all ALS cases is the accumulation of phosphorylated trans-activation response (TAR) DNA-binding protein 43 protein (pTDP-43) aggregates in the cytoplasm of neurons and glial cells within the central nervous system. Traditionally, it was believed that the accumulation of TDP-43 aggregates and subsequent neurodegeneration primarily occurs in motor neurons. However, contemporary evidence suggests that as the disease progresses, other systems and brain regions are also affected. Despite this, there has been a limited number of clinical studies assessing the non-motor symptoms in ALS patients. These studies often employ various outcome measures, resulting in a wide range of reported frequencies of non-motor symptoms in ALS patients. The importance of assessing the non-motor symptoms reflects in a fact that they have a significant impact on patients’ quality of life, yet they frequently go underdiagnosed and unreported during clinical evaluations. This review aims to provide an up-to-date overview of the current knowledge concerning non-motor symptoms in ALS. Furthermore, we address their diagnosis and treatment in everyday clinical practice.

show abstract

Prognostic significance of body weight variation after diagnosis in ALS: a single-centre prospective cohort study

Cited by 37 publications

References 38 publications

Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis

Body weight variation predicts disease progression after invasive ventilation in amyotrophic lateral sclerosis

Update on recent advances in amyotrophic lateral sclerosis

Non-motor symptoms in patients with amyotrophic lateral sclerosis: current state and future directions

Contact Info

Product

Resources

About