What is the role of EMG and NCS in the workup of Emery-Dreifuss muscular dystrophy (EDMD)?

Updated: May 23, 2019
  • Author: Eli S Neiman, DO, FACN; Chief Editor: Amy Kao, MD  more...
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EMG and NCSs should be obtained to confirm the myopathic nature of the disease and to exclude other neuromuscular syndromes.

In EDMD, EMG shows small amplitude narrow duration motor unit potentials (MUPs) with early recruitment (as is typical for myopathies).

Fibrillations and positive sharp waves are rare. NCSs are normal.

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