Neurol. praxi. 2024;25(5):353-356 | DOI: 10.36290/neu.2024.033

Severity of cardiac involvement in Emery­‑Dreifuss muscular dystrophy

doc. MUDr. Edvard Ehler, CSc.1, prof. MUDr. Ivana Štětkářová, CSc., MHA2, doc. MUDr. Ing. Karol Čurila, Ph.D.3
1 Neurologická klinika FZS Univerzity Pardubice a PKN, Pardubice
2 Neurologická klinika 3. LF UK a FNKV, Praha
3 Kardiologická klinika 3. LF UK a FNKV, Praha

Emery-Dreifuss muscular dystrophy (EDMD) belongs to a rare genetic diseases with disturbances of the genes encoding nuclear membrane proteins. Neurological symptoms are less pronounced and not too limiting for EDMD patients; however, dominant cardiac symptoms with conduction impairment (AV block, atrial arrhythmias) and/or cardiomyopathy are the most serious manifestations of EDMD. We present 10 patients with EDMD, pointing out the variability and severity of cardiac problems, including long-term medical care within the approach of the multidisciplinary medical team.

Keywords: muscle dystrophy, muscle contractures, cardiac arrhythmias, cardiomyopathy.

Received: May 8, 2024; Revised: May 8, 2024; Accepted: May 8, 2024; Prepublished online: May 8, 2024; Published: November 19, 2024  Show citation

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Ehler E, Štětkářová I, Čurila K. Severity of cardiac involvement in Emery­‑Dreifuss muscular dystrophy. Neurol. praxi. 2024;25(5):353-356. doi: 10.36290/neu.2024.033.
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