The differential diagnosis of parkinsonian syndromes continues to chal
lenge clinicians. The clinical diagnosis of idiopathic Parkinson's dis
ease is correct in only about three quarters of cases when reevaluated
neuropathologically, emphasizing the need for more discriminative dia
gnostic criteria. The clinical spectrum of brain stem Lewy body idiopa
thic Parkinson's disease itself may be heterogeneous, including dement
ing and nondementing, familial and sporadic, and levodopa-responsive a
nd -nonresponsive subgroups. Recent clinicopathologic evidence suggest
s that other parkinsonian syndromes such as progressive supranuclear p
alsy may also be neuropathologically heterogeneous. Pharmacologic crit
eria of dopaminergic responsiveness have no absolute power of differen
tiating between idiopathic Parkinson's disease and other parkinsonian
disorders, although an absent response argues against idiopathic Parki
nson's disease. The best diagnostic imaging criteria still come from p
ositron emission tomography studies of the functional integrity of the
nigrostriatal dopaminergic system, but more widely applicable techniq
ues are needed. Promising perspectives for this have come form studies
of D2-receptor binding with iodobenzamide single photon emission comp
uted tomography in parkinsonian syndromes.