Systemic Organ Involvement in Myotonic Dystrophy Except For Neuromuscular Symptoms ( A prospective Analysis of 32 Patients)

Abstract

Although principal manifestations of Myotonic Muscular Dystrophy (MMD) are in skeletal muscle, the disease affects many different organs some of which can be life threatening e.g. aspiration pneumonia and cardiac conduction abnormalities. Brain, smooth muscle, cardiac conduction system and endocrine system are the most affected systems. In this study, 32 MMD patients were evaluated in order to determine the frequency of the involvement of systems except for skeletal muscle and nerve. The patients were interviewed with regard to their symptoms related to each systems with a standardized questionnaire and also were undergone some laboratory studies. Central nervous system (CNS) was the most affected system among our MMD patients (%63) followed by gastrointestinal system (GIS) (%44), cardiac system (CS) (%38), endocrine system (%38) and respiratory system (RS) (%13). A significant relation was found between the degree of disability and the involvement of CNS, es and respiratory system. GIS, CNS, and cardiac system were significantly much more affected in childhood onset MMD patients than in adult onset ones. Being aware of the type of problems related to system involvement and supporting patients with symptomatic management process will greatly help to improve quality of life and to reduce the morbidity and mortality in MMD.