Schrander-Stumpel C T, Höweler C J, Reekers A D, De Smet N M, Hall J G, Fryns J P
Department of Clinical Genetics, Academic Hospital, Maastricht, The Netherlands.
J Med Genet. 1993 Jan;30(1):78-80. doi: 10.1136/jmg.30.1.78.
Arthrogryposis multiplex congenita is a heterogeneous condition and many different types are clinically recognisable. Recently, a new type of autosomal dominant arthrogryposis was described in a father and son. We report on a male patient with similar clinical features, confirming this distinct type of arthrogryposis. The condition is characterised by congenital contractures of the hands and feet with diminished or absent phalangeal creases, ophthalmoplegia, a rigid trunk, deep set eyes, and (in the oldest patient) an abnormal electroretinogram. Differential diagnosis from amyoplasia, the different types of distal arthrogryposis, and symphalangism is discussed.
先天性多发性关节挛缩症是一种异质性疾病,临床上可识别出许多不同类型。最近,在一位父亲和儿子身上发现了一种新型的常染色体显性关节挛缩症。我们报告了一名具有相似临床特征的男性患者,证实了这种独特类型的关节挛缩症。该疾病的特征是手足先天性挛缩、指(趾)褶痕减少或缺失、眼肌麻痹、躯干僵硬、眼深陷,以及(年龄最大的患者)视网膜电图异常。文中讨论了与先天性肌无力、不同类型的远端关节挛缩症和关节融合症的鉴别诊断。