Metabolic syndrome in advanced amyotrophic lateral sclerosis patients with tracheostomy‐invasive ventilation

Abstract

Background Amyotrophic lateral sclerosis (ALS) patients have an energy imbalance. Little is known regarding the association between ALS and metabolic syndrome. Aim We aimed to investigate the clinical characteristics of patients with ALS and the association with metabolic syndrome. Methods We examined the prevalence of metabolic syndrome using the International Diabetes Federation (IDF) diagnostic criteria and analyzed its association with clinical characteristics, including subcutaneous and preperitoneal fat thickness, in 66 patients with ALS who underwent tracheostomy‐invasive ventilation. Results Metabolic syndrome was diagnosed in 29 patients (43.9% of all participants). Metabolic syndrome was more common in women and in those with a longer ALS disease duration, higher body mass index, and higher total serum cholesterol levels. Subcutaneous and preperitoneal fat thickness were significantly higher in patients diagnosed with metabolic syndrome. Conclusion Metabolic syndrome is prevalent among patients with advanced ALS. The hypometabolism in patients receiving mechanical ventilation may be the underlying cause.

Body Weight Gain Is Associated with the Disease Stage in Advanced Amyotrophic Lateral Sclerosis with Invasive Ventilation

“…We hypothesize that the central metabolic dysregulation in ALS induces "hypermetabolism" with weight loss in the early stage and "hypometabolism" with weight gain in the advanced stage of patients using TIV. Hypometabolism may lead to previously reported manifestations in advanced ALS, such as visceral fat accumulation [25], macroglossia [26], hyperosmolar hyperglycemic state [27], hypertensive attack [28], and hypothermia [29].…”

“…Its causes (pelvic floor muscle weakness or neurogenic bladder) were not completely identified; however, we virtually accepted any reason for the performance of urinary catheterization in individual patients [27]. A hyperglycemic state was defined as an episode of paroxysmal hyperglycemia with a blood glucose level >400 mg/dL [25].…”

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

“…We hypothesize that the hypothalamus lesion in ALS causes “hypermetabolism” with weight loss in the early stage and “hypometabolism” with weight gain in the advanced stage using TIV. The latter may lead to visceral fat accumulation 54 , macroglossia 55,56 , hyperosmolar hyperglycemic state 57 , hypertensive attack 36 , and hypothermia 24 .…”

“…First, the number of enrolled patients was small compared with those in previous clinical studies on ALS. However, as the study was only conducted in patients with long-term TIV use, the number of patients was not that small 4,54 . In Japan, 20–30% of patients with ALS use TIV 6,58 .…”