title: Trends of quality of life changes in amyotrophic lateral sclerosis patients.
abstract: BACKGROUND
  Amyotrophic 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.
  
  
  OBJECTIVE
  The present study aimed to assess the deterioration rate of QOL and influencing factors in different subgroups of Iranian ALS patients.
  
  
  METHODS
  132 patients were evaluated in this prospective multicenter observational study. QOL was measured using ALS Assessment Questionnaire (ALSAQ-40) during 1year 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.
  
  
  RESULTS
  Significant 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.
  
  
  CONCLUSIONS
  All 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.
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Hosein
authors.initials: H
authors.lastname: Shamshiri
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Farzad
authors.initials: F
authors.lastname: Fatehi
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Roya
authors.initials: R
authors.lastname: Abolfazli
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mohammad Hossein
authors.initials: MH
authors.lastname: Harirchian
authors.affiliation: Department of Neurology, Kerman University of Medical Sciences, Kerman, Iran.
authors.forename: Behnaz
authors.initials: B
authors.lastname: Sedighi
authors.affiliation: Department of Neurology, Iran University of Medical Sciences, Tehran, Iran.
authors.forename: Babak
authors.initials: B
authors.lastname: Zamani
authors.affiliation: Department of Neurology, Gilan University of Medical sciences, Rasht, Iran.
authors.forename: Ali
authors.initials: A
authors.lastname: Roudbari
authors.affiliation: Department of Neurology, Kermanshah University of Medical Sciences, Kermanshah, Iran.
authors.forename: Nazanin
authors.initials: N
authors.lastname: Razazian
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Fatemeh
authors.initials: F
authors.lastname: Khamseh
authors.affiliation: Iranian Center of Neurological Research, Department of Neurology, Tehran University of Medical Sciences, Tehran, Iran. Electronic address: nafisi@sina.tums.ac.ir.
authors.forename: Shahriar
authors.initials: S
authors.lastname: Nafissi
mesh_terms: D000367:Age Factors; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology* / Q000523:psychology; D004185:Disability Evaluation; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007492:Iran; D008297:Male; D008875:Middle Aged; D053580:Muscle Strength; D018696:Neuroprotective Agents / Q000627:therapeutic use; D011446:Prospective Studies; D011788:Quality of Life; D019782:Riluzole / Q000627:therapeutic use; D012720:Severity of Illness Index; D012737:Sex Factors
chemical_list: D018696:Neuroprotective Agents; D019782:Riluzole
keywords: Amyotrophic lateral sclerosis; Functional disability; Physical disability; Quality of life