What is the role of EEG in the workup of limb-girdle muscular dystrophy (LGMD)?

Updated: Aug 15, 2019
  • Author: Monica Saini, MD, MBBS, MRCP(UK); Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Cardiac involvement is common in the autosomal dominant syndromes of LGMD1A and 1B (50%–65%). Cardiomyopathy and cardiac arrhythmias in LGMD1B may cause clinically significant morbidity. In patients with LGMD1E (dilated cardiomyopathy with conduction defect and muscular dystrophy), cardiomyopathy and arrhythmias are nearly always present.

In the autosomal recessive LGMD syndromes, cardiomyopathy is uncommon except in LGMD2G and 2I, where as many as 30%–50% of patients can have mild-to-moderate cardiomyopathy. In the sarcoglycanopathies (most often LGMD2E and 2F), cardiomyopathy is occasionally problematic.

In myofibrillar myopathies, cardiac disease is common, occurring in more than 50% of cases. Presentation can be with cardiomyopathy or cardiac conduction disturbances.

Annual screening with ECG (and possibly echocardiography if the patient is symptomatic) is important for quick diagnosis and follow-up in cases of LGMD and myofibrillar myopathy with cardiac disease.


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