Fibrilace komor - první klinický projev myotonické dystrofie
| Title in English | Ventricular fibrillation as an initial clinical manifestation of myotonic dystrophy |
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| Authors | |
| Year of publication | 2011 |
| Type | Article in Periodical |
| Magazine / Source | Pediatrie pro praxi |
| MU Faculty or unit | |
| 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. |