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NEUROIMAGE
Year : 2007  |  Volume : 55  |  Issue : 4  |  Page : 436-437

An extra hill in a poly-hill sign in a patient with facioscapulohumeral dystrophy


Department of Neurology, Institute of Human Behaviour & Allied Sciences, Dilshad Garden, New Delhi, India

Date of Acceptance24-Apr-2007

Correspondence Address:
Sunil Pradhan
Department of Neurology, Institute of Human Behaviour & Allied Sciences, Dilshad Garden, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.37109

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How to cite this article:
Pradhan S. An extra hill in a poly-hill sign in a patient with facioscapulohumeral dystrophy. Neurol India 2007;55:436-7

How to cite this URL:
Pradhan S. An extra hill in a poly-hill sign in a patient with facioscapulohumeral dystrophy. Neurol India [serial online] 2007 [cited 2018 Oct 23];55:436-7. Available from: http://www.neurologyindia.com/text.asp?2007/55/4/436/37109


An 18-year-old boy presented in the outpatient clinic with difficulty in raising hands above shoulders and inability to sip drinks through a straw. Examination revealed marked weakness of the muscles around shoulder girdle, particularly trapezius, serratus anterior, biceps and triceps muscles. Mild weakness was noted in facial, proximal lower limb and anterior tibial muscles. The scapulae were winged and placed wide apart. His serum creatine kinase level was marginally raised at 510IU/L. Electromyography showed myopathic pattern. A clinical diagnosis of facioscapulohumeral dystrophy (FSHD) was made. As described earlier, poly-hill sign was elicited by asking patient to raise his arms with elbows flexed to nearly 90 and shoulders abducted to his maximum ability (around 70). [1] The sign [Figure - 1] which showed six "hills" on either side consisted of (1) enlarged infraspinatus muscle overlying the winged inferior angle of scapula, (2) upwardly projected superior angle of scapula tenting the wasted trapezius muscle, (3) prominence of laterally projected acromio-clavicular joint due to wasting of trapezius muscle attached to it medially and supero-medial part of deltoid muscle attached to it laterally, (4) prominence of infero-lateral part of the deltoid muscle due to wasting of its own supero-medial part on one side and biceps brachii muscle on the other side, (5) unusual bulge due to some preserved bulk in the middle of the wasted biceps brachii muscle and (6) prominent and well preserved extensor digitorum communis / brachioradialis muscles. The poly-hill sign is based on the fact that various muscular dystrophies have selective weakness, wasting or enlargement of either a group of muscles or a part of a muscle. [2],[3] Patients with FSHD generally have markedly wasted biceps brachii muscle resulting in the description of arm's appearance as "Popeye's arm". [4] Classical "poly-hill sign" therefore has only five hills [Figure - 2]; bulge in the middle of the biceps brachii muscle as seen in this patient, is an unusual feature. The poly-hill sign is most useful in the diagnosis of FSHD in patients with minimal or insignificant facial muscle involvement

 
  References Top

1.Pradhan S. Poly-hill sign in facioscapulohumeral dystrophy. Muscle Nerve 2002;25:754-5.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Brook MH. Disorders of skeletal muscles. In : Bradley WG, Daroff RB, Fenichel GM, Marsden CD, editors. Neurology in clinical practice. Butterworth-Heinemann: Boston; 1991. p. 1843-86.  Back to cited text no. 2    
3.Pradhan S. New clinical sign in Duchenne muscular dystrophy. Pediatr Neurol 1994;11:298-300.  Back to cited text no. 3  [PUBMED]  
4.Cumming WJK. Facioscapulohumeral syndrome. In : Lane RJM, editors. Handbook of muscle disease. Marcel Dekker: New York; 1996. p. 275-86.  Back to cited text no. 4    


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