A large duplication involving the IHH locus mimics acrocallosal syndrome

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Acrocallosal syndrome.

Presented here is a case of a 8 year old boy with typical clinical manifestations of Acrocallosal syndrome. The characteristic features of this syndrome are craniofacial abnormalities, distinctive digital malformation, mental retardation. The clinical and major nosologic aspects of this condition are discussed.

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The acrocallosal syndrome and Greig syndrome are not allelic disorders.

Acrocallosal syndrome is an autosomal recessive form of polysyndactyly associated with mental retardation and agenesis of the corpus callosum. There have been suggestions that it is allelic to the Greig cephalopolysyndactyly syndrome. Linkage analysis, using flanking markers, shows this suggestion is unlikely to be correct.

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ژورنال

عنوان ژورنال: European Journal of Human Genetics

سال: 2012

ISSN: 1018-4813,1476-5438

DOI: 10.1038/ejhg.2011.250