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Bethlem sign

MedGen UID:
866695
•Concept ID:
C4021042
•
Finding
Synonym: Bethlem phenomenon
 
HPO: HP:0030233

Definition

Limitation of wrist and finger extension on asking patient to form a prayer sign. This is a result of progressive wrist and finger flexion contractures. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBethlem sign

Recent clinical studies

Etiology

Autosomal recessive Bethlem myopathy: A clinical, genetic and functional study.
Caria F, Cescon M, Gualandi F, Pichiecchio A, Rossi R, Rimessi P, Cotti Piccinelli S, Gallo Cassarino S, Gregorio I, Galvagni A, Ferlini A, Padovani A, Bonaldo P, Filosto M
Neuromuscul Disord 2019 Sep;29(9):657-663. Epub 2019 Jul 30 doi: 10.1016/j.nmd.2019.07.007. PMID: 31471117

Diagnosis

Characteristic muscle signatures assessed by quantitative MRI in patients with Bethlem myopathy.
Salim R, Dahlqvist JR, Khawajazada T, Kass K, Revsbech KL, de Stricker Borch J, Munawar Sheikh A, Vissing J
J Neurol 2020 Aug;267(8):2432-2442. Epub 2020 May 3 doi: 10.1007/s00415-020-09860-x. PMID: 32363432
"Target" and "Sandwich" Signs in Thigh Muscles have High Diagnostic Values for Collagen VI-related Myopathies.
Fu J, Zheng YM, Jin SQ, Yi JF, Liu XJ, Lyn H, Wang ZX, Zhang W, Xiao JX, Yuan Y
Chin Med J (Engl) 2016 Aug 5;129(15):1811-6. doi: 10.4103/0366-6999.186638. PMID: 27453230Free PMC Article
Importance and challenge of making an early diagnosis in LMNA-related muscular dystrophy.
Menezes MP, Waddell LB, Evesson FJ, Cooper S, Webster R, Jones K, Mowat D, Kiernan MC, Johnston HM, Corbett A, Harbord M, North KN, Clarke NF
Neurology 2012 Apr 17;78(16):1258-63. Epub 2012 Apr 4 doi: 10.1212/WNL.0b013e318250d839. PMID: 22491857

Clinical prediction guides

Systematic review of the efficacy of non-pharmacological interventions in people with Lewy body dementia.
Morrin H, Fang T, Servant D, Aarsland D, Rajkumar AP
Int Psychogeriatr 2018 Mar;30(3):395-407. Epub 2017 Oct 9 doi: 10.1017/S1041610217002010. PMID: 28988547
"Target" and "Sandwich" Signs in Thigh Muscles have High Diagnostic Values for Collagen VI-related Myopathies.
Fu J, Zheng YM, Jin SQ, Yi JF, Liu XJ, Lyn H, Wang ZX, Zhang W, Xiao JX, Yuan Y
Chin Med J (Engl) 2016 Aug 5;129(15):1811-6. doi: 10.4103/0366-6999.186638. PMID: 27453230Free PMC Article

Recent systematic reviews

Systematic review of the efficacy of non-pharmacological interventions in people with Lewy body dementia.
Morrin H, Fang T, Servant D, Aarsland D, Rajkumar AP
Int Psychogeriatr 2018 Mar;30(3):395-407. Epub 2017 Oct 9 doi: 10.1017/S1041610217002010. PMID: 28988547

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