The internal globus pallidus is affected in progressive supranuclear palsy and Parkinson's disease.

Our structural studies of the substantia nigra in parkinsonian patients identified previously unsuspected changes in the pars reticulata, suggesting significant dysfunction in this basal ganglia output. There have been few similar structural studies of the other major basal ganglia output, the internal segment of the globus pallidus. This is despite significant evidence that this basal ganglia region is crucially important for generating parkinsonian symptoms. In fact current surgical interventions target this region in Parkinson's disease. The cellular anatomy of the internal globus pallidus was compared among five controls, six patients with Parkinson's disease, and five patients with progressive supranuclear palsy. Neurons and pathological structures were quantified using the unbiased fractionator method. Only cases with progressive supranuclear palsy had detectable pathology within the internal globus pallidus in the form of tau-positive neuronal and glial tangles and substantial neurodegeneration. Cases with Parkinson's disease had a significant reduction in the proportion of neurons containing parvalbumin but were without significant neurodegeneration, consistent with dysfunction of both basal ganglia output nuclei in advanced parkinsonism. Surgical ablation of the internal globus pallidus for Parkinson's disease appears at odds with the significant neurodegeneration in the similarly akinetic and rigid patients with progressive supranuclear palsy. The results are discussed in association with current hypotheses of basal ganglia function and recent experimentation in patients undergoing pallidotomy for hyperkinetic disorders.