health related quality of life in myotonic dystrophy type 1 and its relationship with cognitive and emotional functioning

Objective: To evaluate the health-related quality of life in myotonic dystrophy type 1 and its relationships with clinical, genetic, neuropsychological and emotional factors. Design: Case-control study of a continuous series of patients with myotonic dystrophy type 1. Patients and methods: Twenty patients, and 20 age-, sex- and education-matched healthy controls underwent the MOS 36Item Short-Form Health Survey (SF-36), an extensive neuropsychological battery and emotional functioning tests. Results: Patients' SF-36 mean scores were lower than those of controls in all dimensions. The neuropsychological study showed a significant impairment in visuospatial and verba