Myotonic dystrophy is the most common muscular dystrophy of adulthood. Neurological manifestation of the disease is typical, however diagnosis could be challenge in patients presentig with predominant cardiac or systemic symptoms. 46 year old male was suffering from presyncope attacks for 16 years, and first examined by a cardiologist. Because of the recent complaints including walking difficulty, he was referred to neurology department. Following a detailed neurological examination and electrodiagnostic workup, he was finally diagnosed myotonic dystrophy type 1. The case highlights the necessitiy of neurological consultation in patients who present with conduction defects, arythmias or cardiomyopathies of unknown origin, accompanied by overt or subtle neurological symptoms. In such patients, neuromuscular disorders should be considered in the differential diagnosis; and in order to provide thorough diagnostic evaluation, multidisciplinary approach is essential.
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