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

Abstract

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.

Amyotrophic lateral sclerosis (ALS), cancer, autoimmunity and metabolic disorders: An unsolved tantalizing challenge

“…A recent study reported that ALS patients with significant weight loss (>1.7 BMI units/year) at early stages of the disease had a more aggressive disease course (Nakayama et al, 2019). Complementarily, Moglia et al (2019) reported in an Italian cohort including 620 ALS patients that patient survival was independently related to the mean monthly percentage of weight loss at diagnosis. All this evidence has led to the consideration of BMI as an independent prognostic factor at the time of diagnosis (Moglia et al, 2019).…”

“…Therefore, the data did not support a causal role of genetically increased or decreased BMI on the risk of ALS (Zeng, Yu, & Xu, 2019). A recent study reported that ALS patients with significant weight loss (>1.7 BMI units/year) at early stages of the disease had a more aggressive disease course (Nakayama et al, 2019). Complementarily, Moglia et al (2019) reported in an Italian cohort including 620 ALS patients that patient survival was independently related to the mean monthly percentage of weight loss at diagnosis.…”

“…Complementarily, Moglia et al (2019) reported in an Italian cohort including 620 ALS patients that patient survival was independently related to the mean monthly percentage of weight loss at diagnosis. All this evidence has led to the consideration of BMI as an independent prognostic factor at the time of diagnosis (Moglia et al, 2019).…”

Gastrostomy and mechanical ventilation in amyotrophic lateral sclerosis: how best to support the decision-making process?

“…Importantly, concomitant chronic diseases have no influence on the outcome of IV [118]. In a recent study, a steeper BMI decrease prior to the start of IV was related to a significantly faster progression of motor disabilities, including ophthalmoplegia, total quadriplegia, and mouth-opening ability after IV placement [119].…”

Metabolic Abnormalities, Dietary Risk Factors and Nutritional Management in Amyotrophic Lateral Sclerosis

“…Evidence suggests that ALS patients may suffer from an abnormal energy metabolism, as well as an impaired glucose and lipid metabolism [ 19 , 20 , 21 ]. Furthermore, body weight and body mass index (BMI) have been hypothesized to play a role in disease occurrence and progression in ALS [ 22 ]. Diet has been shown to potentially play a role in less disruptive mental health disorders [ 23 ], but there is a growing interest in exploring the potential role of nutritional and metabolic factors in affecting the survival of ALS patients [ 24 ].…”