Computed tomography in Sjögren-Larsson syndrome.

A family with 12 children, 6 of whom had Sjögren-Larsson syndrome, was studied clinically. On CT, all six siblings with the disease had patchy regions of white matter hypodensity, most marked in the frontal lobes, which were confluent in the more severe cases. The ventricles were not enlarged and no other abnormality was demonstrated. The CTs of the 6 unaffected siblings and the mother, who was also unaffected by the disease, were normal.