28
Emery-Dreifuss and Limb-girdle Type 1B
Muscular Dystrophy
Table 5. Clinical scenarios for the management of arrhythmias
in EDMD and LGMD1B
Clinical scenario
EDMD
1. A 25-year-old man presents with left upper extremity weakness and difficulty
speaking. The neurological findings resolve spontaneously in the emergency
department over a period of 1 hour. The patient has a history of elbow and ankle
contractures since childhood and a presumptive diagnosis of EDMD. There are no
other family members with a similar phenotype. ECG reveals sinus bradycardia,
low-amplitude P waves, a PR interval of 260 ms, and nonspecific intraventricular
conduction delay with a QRS duration of 110 ms. Transthoracic echocardiogram
reveals biatrial dilation, left ventricle chamber size at the upper limits of normal,
moderate mitral and tricuspid regurgitation, and an LVEF of 55%.
