Abstract

Two siblings, from consanguineous parents, developed in their twenties a Parkinsonian syndrome. In the elder, the disease evolved for 13 years and the necropsic study was diagnostic of Hallervorden-Spatz disease. The younger sibling is severely affected after 12 years of the disorder. Several CT and one MR studies done in this patient during the last 4 years have been normal. Ultrastructural studies of the bone marrow histiocytes and blood lymphocytes disclosed peculiar inclusions. Bromocriptine in low doses proved to be a beneficial therapy for this patient.