Significance of Beevor’s sign in facioscapulohumeral dystrophy and other neuromuscular diseases

An atypical presentation of facioscapulohumeral dystrophy (FSH) is described, where the presence of a positive Beevor’s sign led to genetic testing and subsequent probable diagnostic confirmation. This prompted evaluation of a further 68 patients for the presence of Beevor’s sign. Among these, 19/20...

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Veröffentlicht in:	Journal of neurology, neurosurgery and psychiatry neurosurgery and psychiatry, 2005-06, Vol.76 (6), p.869-870
Hauptverfasser:	Shahrizaila, N, Wills, A J
Format:	Artikel
Sprache:	eng
Schlagworte:	Age Aged Beevor’s sign Biological and medical sciences Biopsy Chromosomes Chromosomes, Human, Pair 4 - genetics Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases Disease Diseases of striated muscles. Neuromuscular diseases Elbow - physiopathology facioscapulohumeral dystrophy Family medical history Female FSH Hip - physiopathology Humans Knee - physiopathology Male Medical sciences Middle Aged Muscle Cramp - complications Muscle Cramp - genetics Muscle Cramp - pathology Muscle Weakness - complications Muscle Weakness - diagnosis Muscle Weakness - physiopathology Muscle, Skeletal - pathology Muscular dystrophy Muscular Dystrophy, Facioscapulohumeral - complications Muscular Dystrophy, Facioscapulohumeral - genetics Muscular Dystrophy, Facioscapulohumeral - pathology Neck - physiopathology Necrosis Neurology neuromuscular disease Neuromuscular diseases Short Report Shoulder - physiopathology Spinal cord Umbilicus - physiopathology
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Beschreibung
Zusammenfassung:	An atypical presentation of facioscapulohumeral dystrophy (FSH) is described, where the presence of a positive Beevor’s sign led to genetic testing and subsequent probable diagnostic confirmation. This prompted evaluation of a further 68 patients for the presence of Beevor’s sign. Among these, 19/20 patients with FSH had a positive Beevor’s sign, compared with 2/28 with other muscle diseases, and 0/20 in a neurological control group. Beevor’s sign should be considered as an additional criterion for the diagnosis of FSH.
ISSN:	0022-3050 1468-330X
DOI:	10.1136/jnnp.2004.052019