Document Detail


A family with autosomal dominant distal arthrogryposis multiplex congenita and brown syndrome.
MedLine Citation:
PMID:  10617921     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Arthrogryposis multiplex congenita is a heterogeneous condition found in a number of different disorders and characterized by congenital joint contractures. We describe typical signs of congenital Brown syndrome (inability to elevate the affected eye actively or passively in full adduction) in three relatives with distal arthrogryposis multiplex congenita. We found a thickening of the superior oblique muscles in these patients with pain and increased intraocular pressure in upgaze. The pathogenesis of clinical and morphological findings is discussed. The association of Brown syndrome with distal arthrogryposis multiplex congenita has not been previously reported and provides us with an important point of reference in the understanding of both syndromes.
Authors:
L T Lobefalo; A T Mancini; M T Petitti; A E Verrotti; G E Della Loggia; A E Di Muzio; F E Chiarelli; P E Gallenga
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ophthalmic genetics     Volume:  20     ISSN:  1381-6810     ISO Abbreviation:  Ophthalmic Genet.     Publication Date:  1999 Dec 
Date Detail:
Created Date:  2000-02-25     Completed Date:  2000-02-25     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9436057     Medline TA:  Ophthalmic Genet     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  233-41     Citation Subset:  IM    
Affiliation:
Institute of Ophthalmology, University of Chieti, Chieti, Italy. l.lobefalo@eye.unich.it
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Arthrogryposis / complications*,  genetics,  pathology
Eye / pathology,  ultrasonography
Family Health
Female
Genes, Dominant*
Humans
Male
Ocular Motility Disorders / complications*,  genetics,  pathology
Oculomotor Muscles / ultrasonography
Pedigree
Comments/Corrections
Comment In:
Ophthalmic Genet. 2001 Jun;22(2):125-30   [PMID:  11455953 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  A possible hot spot in exon 21 of the retinoblastoma gene predisposing to a low penetrant retinoblas...
Next Document:  Exclusion of AR-CHED from the chromosome 20 region containing the PPMD and AD-CHED loci.