Home > Encyclopedia of Neurological Disorders > Sjogren-Larsson Syndrome

Definition

Sjogren-Larsson syndrome is an inherited condition resulting in thickened, dry, rough skin (ichthyosis), mental retardation, and stiff, rigid muscles (spasticity). Although not all the manifestations of the disease may be immediately evident at birth, the disease is not considered to be progressive.

Description

Originally identified in Swedish patients, Sjogren-Larsson is a rare genetic disorder. The condition is more common in places where intermarriage within families is traditional, such as among the Haliwa Native Americans of Halifax and Warren counties in North Carolina, and in Vasterbotten and Norrbotten Counties in Sweden.

Demographics

The frequency of Sjogren-Larsson syndrome in the United States is unknown....