The radiotracer [I-123]beta-CIT is a sensitive marker of dopamine upta
ke sites that can be used to visualize dopaminergic nerve endings in v
ivo in the human brain. We report on [I-123]beta-CIT single-photon emi
ssion computed tomography (SPECT) findings in a patient with DOPA-resp
onsive dystonia (DRD). [I-123]beta-CIT SPECT showed a striatal radiotr
acer uptake in the upper range of normal, indicating intact dopamine t
ransporters and structural integrity of nigrostriatal neurons. This di
fferentiates DRD from clinically similar cases with juvenile-onset par
kinsonism with dystonia that have a considerably poorer prognosis. [I-
123]beta-CIT SPECT may provide a method equally as useful as fluorodop
a positron emission tomography in DRD.