RESPIRATORY-CHAIN AND MITOCHONDRIAL DEOXYRIBONUCLEIC-ACID IN BLOOD-CELLS FROM PATIENTS WITH FOCAL AND GENERALIZED DYSTONIA

An increasing number of neurodegenerative diseases seem to be associat ed with or even due to disturbances of cerebral energy metabolism. One generally accepted example is complex I deficiency in substantia nigr a from patients with Parkinson's disease. Reports on a complex I defec t in platelets from patients with dystonia led us to check for disturb ances of the respiratory chain or of the mitochondrial genome in isola ted mitochondria from patients with focal or generalized dystonia. We could not confirm the idea of mitochondrial disturbance in platelets f rom patients with dystonia because we did not find abnormal enzyme act ivities or any deletions of the mitochondrial genome. Thus, we do not think that blood cells such as platelets can serve as markers for neur odegenerative disorders such as dystonia.