Abstract:
Neuromuscular diseases are a large group of diseases that are characterized by defective
functions of peripheral nervous system, neuromuscular junction and/or muscle. Due to the similar
structure of skeletal and cardiac muscles it is possible to associate neuromuscular diseases with
cardiac disorders. The aim of the work was to identify the incidence rate of cardiovascular
abnormalities in children with neuromuscular diseases. During the period from January to December
2008 at the Clinic of Neurology and Psychiatry for children and Youth the Faculty of Medicine in
Belgrade, 44 patients with neuromuscular diseases had cardiovascular examination (physical,
electrocardiographic and echocardiographic). All of the examined patients fell ill before turning 18
years of age. The patients’ ages (M: 25, F: 19) ranged from 3 to 38 years (X = 16 ± 8.35) at the time
of cardiovascular examination. High incidence rate of mitral valve dysplasia, without haemodynamic
changes, has been diagnosed (38.6%). Patients with distrophinopathy are often referred to
cardiovascular examination. Five (26.3%) of the patients with distrophinopathy have dilated
cardiomyopathy, and two patients with distrophinopathy have congenital heart disease and diseases of
the valve. One of the examined patients had congenital heart disease as well as nondistrophinopathic
dystrophy (LGM.D.), and spinal muscular atrophy (SMA) while patients with peripheral neuropathy
hadn’t been diagnosed with pathological cardiovascular findings. Two out of five examined patients
with disease of neuromuscular junction had results of the cardiovascular examination that matches the
ones found in heart valve diseases. Dilatated cardiomyopathy, isolated or associated with other
cardiology abnormalities can only be diagnosed in distrophinopathy. In other forms of dystrophy, as
well as other neuromuscle diseases occurrence of described diseases of valve and congenital heart
diseases has been diagnosed.
