Publication details

Fibrilace komor - první klinický projev myotonické dystrofie

Title in English Ventricular fibrillation as an initial clinical manifestation of myotonic dystrophy
Authors

RYZÍ Marie VÍT Pavel BUREŠOVÁ Miroslava RYZÍ Michal

Year of publication 2011
Type Article in Periodical
Magazine / Source Pediatrie pro praxi
MU Faculty or unit

Faculty of Medicine

Citation
Field Paediatry
Keywords myotonic dystrophy type I; ventricular fibrillation; implantable cardioverter-defibrillator
Description Ventricular fibrillation as an initial clinical manifestation of myotonic dystrophy Myotonic dystrophy type 1 (MD1) is an autosomal dominant disease affecting both striated and smooth muscle. Clinical symptomatology of the disease includes not only muscular manifestations but also other symptoms resulting, in particular, from the involvement of the heart, eyes, central nervous system or some endocrine glands. A case report is presented of a boy who was diagnosed with asymptomatic MD1 at the age of ten years old. In the subsequent period, he had not been under neurological observation and had never undergone cardiological evaluation. At the age of 17, he had collapsed during sports and was found to have severe ventricular fibrillation. Subsequently, the boy underwent a comprehensive evaluation and had an implantable cardioverter-defibrillator inserted as a prevention of further possible dysrhythmia. The paper aims to point out that muscular diseases may involve some other, not uncommonly neglected accompanying symptoms.

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