Teaching video neuro images. the beevor sign in late-onset pompe disease

The Beevor sign, an upward deflection of the umbilicus on flexion of the neck, is a characteristic finding in facioscapulohumeral muscular dystrophy.1 Many other neuromuscular disorders involving axial muscles can present a Beevor sign.2 We report a 45-year-old man with late-onset Pompe disease showing a major Beevor sign (figure 1 and video on the Neurology® Web site at Neurology.org). He had progressive limb-girdle weakness that started in his 20s and severe axial weakness. Whole-body muscle MRI showed a complete fatty replacement and atrophy of the lower part of rectus abdominis and a milder involvement of the upper part