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Dopamine transporter SPECT in patients with mitochondrial disorders

¹ Department of Neurology, University of Bonn, Bonn, Germany
² Department of Nuclear Medicine, University of Bonn
³ Department of Epileptology, University of Bonn
⁴ Department of Nuclear Medicine, University of Munich, Munich, Germany

Correspondence to:
Correspondence to:
Dr Ullrich Wüllner
Department of Neurology, University of Bonn, Sigmund-Freud-Strasse 25, Bonn 53105, Germany; wuellner{at}uni-bonn.de

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-ß-fluoropropyl-2ß-carbomethyl-3ß-(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.

Abbreviations: CPEO, chronic progressive external ophthalmoplegia; DAT, dopamine transporter; SPECT, single photon emission computed tomography

Keywords: dopamine; 123 I-FP-CIT SPECT; mitochondrial disorders