Quality of life in hereditary neuromuscular diseases.

Abstract

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. 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. 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.