G.P.249

Myotonic dystrophy (DM) is a multisystem disorder characterized by skeletal muscle and multiple organ impairment. The aim of the study is to compare our local data from the registry with that known in other countries. The data from the local part of Czech national registry of myotonic dystrophy including focused questionnaires were evaluated. The population consists of 107 patients: 28 DM type 1 mean age 42.6 (range, 23–63), 79 DM type 2 mean age 54.1 (range, 22–76); 67 females and 40 males. One of the most common complaints of patients was muscle pain. Long-term prevalence of muscle pain occurred in 63.6% of patients (71.4% of DM type 1 and 60.8% of DM type 2). Half of the patients (52.4%) suffered from excessive daytime sleepiness in varying degree of intensity. Cognitive dysfunction had 8.6% of our patients, but mostly in DM type 1. Diabetes mellitus or impaired glucose tolerance were found in 21.5% of patients and thyroid dysfunction in 17.4%. Increased sweating indicated 44.8% of patients. Dysphagia was found in 27.8% and 55.8% of patients had fecal incontinence (15.1% was never able to prevent the incontinence of loose stools and 29.1% was not able to prevent the incontinence of solid stool). More than half of the patients (51.2%) reported stress incontinence (during exercise, coughing or sneezing) and 41.9% have other various forms of the urinary incontinence. Our results are consistent with previously published data. We found surprisingly high frequency of bowel incontinence which was not so far comprehensively studied.