Further evidence of genetic homogeneity in Sjogren-Larsson syndrome

Sjogren-Larsson syndrome is an autosomal recessive disorder characterized b y congenital ichthyosis, spastic di- or tetraplegia and mental retardation. In 1994 Sjogren-Larsson syndrome was mapped to chromosome 17, close to the genetic marker D17S805 in a study of 24 Swedish families. We have analysed 12 microsatellite markers in 10 additional non-Swedish families with Sjogr en-Larsson syndrome originating from Germany, Lebanon, Spain and Canada. Th e results are consistent with earlier data and give further evidence of Sjo gren-Larsson syndrome being a homogeneous disorder. Swedish soldiers were b ivouacking in Germany during the 30-year war in the 17th century and it has been suggested that they could have introduced the Sjogren-Larsson syndrom e gene to the German population. Haplotypes from 7 German families with Sjo gren-Larsson syndrome were compared with earlier analysed Swedish haplotype s. No evidence of all German patients carrying the same mutation or the maj or "Swedish Sjogren-Larsson syndrome gene" was found.