Dopamine Dl and D2 receptors were studied in brain tissue sections from a typical patient with progressive supranuclear palsy and in 7 age-matched brains. The sensity of Dl receptors in the caudate-putamen and fontal cortex of the patient was within control limits, By contrast, the density of nigral Dl receptors and strial D2 receptors was dramatically reduced in the patient as compared to the control brains. This work shows again that the loss of striatal D2 receptors is the most plausible explanation for the poor response to dopaminergic drugs in patients with progressive supranuclear palsy. While the loss of nigral Dl receptors can be explained by the loss of nigral neurons, it seems that neurons bearing striatal Dl receptors are spared in progressive supranuclear palsy. The clinical effects of selective D1 agonists are worth testing in this devastating disorder.