Trends of quality of life changes in amyotrophic lateral sclerosis patients

• All domains of quality of life deteriorated consistently in ALS patients.
• Quality of life worsening correlated with physical and functional disability trends.
• Deterioration of quality of life was slower in severe and terminal stages.
• Quality of life worsened irrespective of age, sex and riluzole consumption.
• bulbar onset patients showed significantly higher rate of deterioration.

BackgroundAmyotrophic lateral sclerosis (ALS) is an incurable progressive neurodegenerative disease and thus the assessment of quality of life (QOL) changes and factors that may influence its course is valuable in the meantime.ObjectivesThe present study aimed to assess the deterioration rate of QOL and influencing factors in different subgroups of Iranian ALS patients.Methods132 patients were evaluated in this prospective multicenter observational study. QOL was measured using ALS Assessment Questionnaire (ALSAQ-40) during 1 year follow up and its progression rate was assessed in different subgroups of patients according to age, sex, stage of disease, riluzole consumption, onset type. Also physical disability and functional disability were measured using MMT and ALSFRS-R scores respectively and their progression rates were compared with ALSAQ-40 changes.ResultsSignificant deterioration of the scores of ALSAQ-40 during study was consistent in all of its domains (p = 0.000). There was a significant negative correlation between ALSFRS-R and MMT changes and ALSAQ-40 change (p = 0.000) and this was consistently observed in all domains of ALSAQ-40 (p = 0.00). ALSAQ-40 deterioration rate was shown to be significantly lower in severe/terminal stages compared to mild/moderate stages (p = 0.00). Significantly higher deterioration rate was observed in bulbar onset versus limb onset patients [F (1,130) = 4.52, p = 0.04] but no significant difference was observed among other subgroups according to age, sex and riluzole consumption.ConclusionAll domains of QOL significantly deteriorate during ALS course and there is a significant correlation between their changes and progression of physical and functional disabilities. Rate of degradation of QOL may be different at different stages of the disease. QOL worsens independent of factors such as sex, age and consumption of riluzole; but onset type (bulbar versus limb) is an imperative factor in quality of life changes during the disease course.