Four siblings with Hallervorden-Spatz disease

We reported four cases of Hallervorden-Spatz disease. All four siblings (th ree males and one female) in the family are affected. The first symptoms of the disease were spastic paraparesis and optic atrophy followed by trunkal dystonia and lower motor neurone involvement. The average age of the onset was: 4.25 years. The diagnosis was made at the ages of 17, 14, 11 and 10 y ears. The diagnosis was confirmed clinically, electrophysiologically and by MRI. On MRI scans all patients demonstrated hypointense areas in globus pa llidus. There is neither specific treatment nor prenatal diagnosis. (C) 200 1 Elsevier Science B.V. All rights reserved.