A 50-year-old man with facioscapulohumeral muscular dystrophy 1 (FSHD1), scapuloperoneal weakness, and bilateral scapular winging presents a downward movement of his umbilicus while attempting to get up from the recumbent position (video 1). Beevor sign, an upward umbilicus deviation when the supine patient is flexing the neck or tries to sit up without the use of the hands, indicates lower abdominal muscle weakness. It is highly sensitive and specific, but not diagnostic, for FSHD1, because it is described in other myopathies.1,2 Beevor sign may be present in this “inverted” form, indicating predominant weakness of upper rectus abdominis muscle.
Video 1
Inverted Beevor sign in a 50-year-old man with genetically confirmed facioscapulohumeral muscular dystrophy. Note the downward deviation of the umbilicus when the patient tries to sit up from the supine position.Download Supplementary Video 1 via http://dx.doi.org/10.1212/010646_Video_1
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Teaching slides links.lww.com/WNL/B188
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