Abstract

Background: Mitochondrial disorders may affect basal ganglia function. In addition, decreased activity of complex I of the mitochondrial electron transport chain has been linked to the pathogenesis of dopaminergic cell loss in Parkinson’s disease.

Objective :To investigate the dopaminergic system in patients with known mitochondrial disorders and complex I deficiency.

Methods: Dopamine transporter density was studied in 10 female patients with mitochondrial complex I deficiency by ¹²³I-FP-CIT (N-β-fluoropropyl-2β-carbomethyl-3β-(4-iodophenyl)-nortropane) SPECT.

Results: No differences in ¹²³I-FP-CIT striatal binding ratios were observed and no correlation of the degree of complex I deficiency and striatal binding ratios could be detected.

Conclusions: These data argue against the possibility that mitochondrial complex I deficiency by itself is sufficient to elicit dopaminergic cell loss.