title: Quality of life in hereditary neuromuscular diseases.
abstract: OBJECTIVE
  The aim of the study is to evaluate different aspects in the quality of life (QoL) in a group of patients affected by hereditary neuromuscular disease.
  
  
  METHODS
  Forty-five consecutive outpatients (mean age 49.46 +/- 17.07 years, range 19-80 years) with hereditary neuromuscular disease underwent the Brooke scale and functional independent measure to assess the functional status, the Sickness Impact Profile (SIP) and Psychological General Well-Being Index (PGWBI), as a measure of the QoL.
  
  
  CONCLUSIONS
  All patients had a poor QoL assessment with SIP. The higher disability level was not related to a worse QoL perception with the exception of the physical area. Females and patients above 49 years showed the worst QoL profile. The discomfort linked to the emotional and affective sphere, assessed with PGWBI, appeared low in the study sample. Therefore psychosocial aspects and economical and environmental factors may influence the QoL.
authors.affiliation: Fondazione Don Carlo Gnocchi-ONLUS, Centro Santa Maria agli Ulivi, Pozzolatico Florence, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Piccininni
authors.forename: C
authors.initials: C
authors.lastname: Falsini
authors.forename: A
authors.initials: A
authors.lastname: Pizzi
mesh_terms: D000328:Adult; D000368:Aged; D006233:Persons with Disabilities / Q000523:psychology*; D005260:Female; D006304:Health Status; D020271:Heredodegenerative Disorders, Nervous System / Q000473:pathology / Q000523:psychology*; D006801:Humans; D008297:Male; D008603:Mental Health; D008875:Middle Aged; D009468:Neuromuscular Diseases / Q000473:pathology / Q000523:psychology*; D010045:Outpatients; D011446:Prospective Studies; D011788:Quality of Life; D012737:Sex Factors; D012944:Social Support