Beevor's sign in facioscapulohumeral muscular dystrophy: an old sign with new implications

Beevor's sign, an upward deflection of the umbilicus on flexion of the neck, is the result of paralysis of the inferior portion of the rectus abdominis muscle, so that the upper fibers predominate, pulling the umbilicus upwards. The condition may be caused by spinal cord injury at or below the level of Th10. It has also been observed in patients with facioscapulohumeral muscular dystrophy (FSHD). Positive Beevor's sign has been described as a sign of more than 90% sensitivity and specificity with regard to diagnosis of FSHD. We investigated 28 patients with FSHD, proven by genetic analysis, and 65 non-FSHD patients with other neuromuscular diseases. In 13 patients classical FSHD phenotype was observed, in 15 patients phenotype was atypical. Beevor's sign was positive in 15 out of 28 FSHD patients as well as in two of the 65 non-FSHD patients. In patients with typical FSHD phenotype, Beevor's sign was positive in 11/13. Only 4/15 patients with atypical FSHD phenotype showed Beevor's sign. Beevor's sign is less frequent in patients with atypical phenotype. Although Beevor's sign is significantly more frequent in FSHD patients than in patients with other neuromuscular diseases, Beevor's sign is not as sensitive as previously reported. However, especially in atypical cases, Beevor's sign might help in the diagnosis of FSHD.