We have studied dopamine D-2 receptor binding by [C-11]raclopride posi
tron emission tomography in 14 patients with dopa-responsive dystonia
(DRD). Data were compared with 16 levodopa-treated patients with Parki
nson's disease (PD) and 26 healthy controls. The results revealed an e
levated [C-11]raclopride binding index in the putamen and caudate nucl
eus of DRD patients compared with controls as well as a significant el
evation in the caudate nucleus compared with PD patients. The increase
of [C-11]raclopride binding may be interpreted either as reduced trac
er displacement by endogenous dopamine, or as an alteration of the rec
eptor features due to chronic dopamine deficiency. The difference in [
C-11]raclopride binding in DRD and PD patients in the caudate nucleus
suggests that this structure may be of pathophysiological relevance in
the presentation of the clinical features of both diseases.