Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic options

Abstract

Background: Weight loss is a frequent feature in the motor neuron disease Amyotrophic lateral sclerosis (ALS). In this study we investigated possible causes of weight loss in ALS, its impact on mood/quality of life (QOL) and the benefit of high calorie nutritional/other dietary supplements and percutaneous endoscopic gastrostomy (PEG).

Methods: 121 ALS patients were interviewed and answered standardized questionnaires (Beck depression inventory - II, SF36 Health Survey questionnaire, revised ALS functional rating scale). Two years after the initial survey we performed a follow-up interview.

Results: In our ALS-cohort, 56.3% of the patients suffered from weight loss. Weight loss had a negative impact on QOL and was associated with a shorter survival. Patients who took high calorie nutritional supplements respectively had a PEG stated a great benefit regarding weight stabilization and/or QOL.38.2% of our patients had significant weight loss without suffering from dysphagia. To clarify the reasons for weight loss in these patients, we compared them with patients without weight loss. The two groups did not differ regarding severity of disease, depression, frontotemporal dementia or fasciculations, but patients with weight loss declared more often increased respiratory work.

Conclusions: Weight loss is a serious issue in ALS and cannot always be attributed to dysphagia. Symptomatic treatment of weight loss (high calorie nutritional supplements and/ or PEG) should be offered more frequently.

Figure 1

Mean scores of ALSFRS_R and SF36 questionnaire of patients without and with weight loss and impact of high calorie supplements/PEG. Mean scores of ALSFRS_R (A) and SF36 questionnaire (B) of patients without and with weight loss. The latter group showed significantly lower scores at the ALSFRS_R and the SF36 subscales “physical functioning” and “vitality”. 58.3% of patients consuming high calorie supplements and 76.9% of patients who had undergone PEG reported subsequent weight stabilization or even weight gain (C and D). * p < 0.05, ** p < 0.01.

Figure 2

Comparison of patients with weight loss with/without dysphagia and patients without weight loss. Patients with weight loss and dysphagia differed significantly from patients without weight loss in BDI and ALSFRS_R (total and bulbar). Patients with weight loss without dysphagia, on the other hand, did not have higher BDI/ lower ALSFRS_R scores than patients without weight loss (A). Weight loss in patients without dysphagia therefore does not seem to be directly related to a more advanced disease stage or increased depression. Patients with weight loss and dysphagia significantly more often declared increased respiratory work than patients without weight loss. Patients with weight loss without dysphagia showed a tendency towards increased respiratory work compared to patients without weight loss (p = 0.12). There were no differences regarding frequency of fasciculations between the groups (B). Follow-up by telephone two years after the initial survey highlighted the prognostic value of weight loss: Kaplan-Meier survival analysis for ALS patients with and without weight loss revealed significantly shorter survival of ALS patients with weight loss (log rank p = 0.001) (C).

Figure 3

Mean scores of ALSFRS-R, BDI and SF36 of patients with and without intake of dietary supplements. Mean scores of ALSFRS-R (A), BDI (B) and SF36 (C) of patients with and without intake of other (not high calorie) dietary supplements. Supplement intake was associated with significantly higher scores at the ALSFRS_R and the SF36 subscales “physical functioning”, “vitality” and “social functioning” and significantly lower scores in BDI. * p < 0.05.