Beevor's sign in facioscapulohumeral dystrophy

Introduction: Charles E. Beevor first documented the finding of an upward deflection of the umbilicus on flexion of the neck in spinal cord injury at or below the level of T9. Beevor's sign has also been observed in patients with facioscapulohumeral dystrophy. In two series, 27 of 30 patients¹ and 19 of 20 patients² with FSHD had positive Beevor's sign but 2 in 28 non-FSHD myopathy patients² and none in 40 controls with other neuromuscular diseases¹.

Methods: We investigated 21 patients with FSHD: 9 patients with known gene rearrangement at 4q35, 12 new patients with unclassified myopathy at time of investigation, in whom diagnosis of FSHD was subsequently proven by gene analysis, and 65 non-FSHD patients with other neuromuscular disease. Beevor's sign was considered positive when the patient was examined in supine position and there was an upward movement of the umbilicus upon neck flexion.

Results: Beevor's sign was positive in 10 out of 21 FSHD patients as well as in 2 of the 65 non-FSHD patients. 8 of the FSHD patients with Beevor's sign showed typical phenotype of FSHD. In two patients with facial sparing myopathy Beevor's sign was suspicious for the diagnosis of FSHD. The diagnosis was finally established by the gene rearrangement at 4q35. One of the non-FSHD patients with Beevor's sign had Pompe's disease and one had Myotonic dystrophy Type 1 (DM1).

Discussion: 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 reported before. Nevertheless, Beevor's sign may lead to the diagnosis of FSHD in particular cases, especially in atypical cases, e.g. facial sparing FSHD.

¹ Awerbuch GI, et al. Beevor's sign and facioscapulohumeral dystrophy. Arch Neurol 1990;47:1208–9.

² Shahrizaila N and Wills AJ. Significance of Beevor's sign in facioscapulohumeral dystrophy and other neuromuscular diseases. J Neurol Neurosurg Psychiatry 2005;76:869–870.