Myopathies involving axial musculature can also produce a Beevor’s sign. It was first reported with FSH dystrophy (2-5). While the presence of Beevor’s sign can help separate FSH dystrophy from other facioscapulohumeral syndromes, it has also been reported in adult onset acid maltase deficiency and in both sporadic and hereditary inclusion body myositis.(6-8)
Video courtesy Dr. Francis Walker
1. Campbell WW. Clinical signs in neurology: a compendium. Philadelphia: Wolters Kluwer Health, 2016.
2. Awerbuch GI, Nigro MA, Wishnow R. Beevor's sign and facioscapulohumeral dystrophy. Arch Neurol. 1990;47:1208-9.
3. Mathys J, De Marchis GM. Teaching video neuroimages: Beevor sign: when the umbilicus is pointing to neurologic disease. Neurology. 2013;80:e20.
4. Eger K, Jordan B, Habermann S, Zierz S. Beevor's sign in facioscapulohumeral muscular dystrophy: an old sign with new implications. J Neurol. 2010;257:436-8.
5. Shahrizaila N, Wills AJ. Significance of Beevor's sign in facioscapulohumeral dystrophy and other neuromuscular diseases. J Neurol Neurosurg Psychiatry. 2005;76:869-70.
6. Garibaldi M, Diaz-Manera J, Gallardo E, Antonini G. Teaching Video NeuroImages: The Beevor sign in late-onset Pompe disease. Neurology. 2016;86:e250-1.
7. Preethish-Kumar V, Pogoryelova O, Polavarapu K, et al. Beevor's sign: a potential clinical marker for GNE myopathy. Eur J Neurol. 2016;23:e46-8.
8. Sugie K, Kumazawa A, Ueno S. Sporadic Inclusion Body Myositis Presenting with Beevor's Sign. Intern Med. 2015;54:2793-4.