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 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.
Shahrizaila
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Department of Neurology, Queen's Medical Centre, Nottingham NG7 2UH, United Kingdom. tshahrizaila@hotmail.com
Wills
A J
AJ
D000368:Aged; D001706:Biopsy; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics*; D004550:Elbow / Q000503:physiopathology; D005260:Female; D006615:Hip / Q000503:physiopathology; D006801:Humans; D007717:Knee / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009120:Muscle Cramp / Q000150:complications / Q000235:genetics / Q000473:pathology; D018908:Muscle Weakness / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D018482:Muscle, Skeletal / Q000473:pathology*; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000150:complications / Q000235:genetics / Q000473:pathology; D009333:Neck / Q000503:physiopathology; D009336:Necrosis; D012782:Shoulder / Q000503:physiopathology; D014472:Umbilicus / Q000503:physiopathology