Evaluation of activities of daily living in patients with slowly progressive neuromuscular diseases.

Slowly progressive neuromuscular diseases include but are not limited to: facioscapulohumeral muscular dystrophy (FSHD) and limb-girdle muscular dystrophy (LGMD), hereditary motor and sensory neuropathy (HMSN) and spinal muscular atrophy type III (SMA3). The purpose of this study is to present an evaluation of basic and complex activities of daily living in patients suffering from these diseases. The study was conducted on a group of 58 Polish patients: 25 patients with HMSN, 19 with LGMD and FSHD and 14 with SMA3. The research instrument consisted of two parts: a specially designed questionnaire and Nottingham Extended ADL Index. The survey was voluntary, anonymous and self-administered. In our study the highest scores on the NEADL scale were achieved by HMSN patients, and the lowest by patients with SMA3. The research revealed statistically significant differences between all the groups in the total number of points achieved on NEADL scale. The study revealed that for most respondents the most difficult tasks were those in the area of 'mobility'. It is consistent with reports in the literature, which confirm that out of the slowly progressive neuromuscular diseases included in this research, SMA3 is a disease leading to the biggest limitations in performing the activities of everyday life.