Sites affected may include one or more of the following:
(1) putamen
(2) caudate nucleus
(3) hypothalamus
(4) globus pallidus
(5) locus ceruleus
(6) inferior olives
(7) substantia nigra
(8) pontine nuclei
(9) arcuate nuclei (medulla oblongata)
(10) autonomic nuclei in the brainstem
(11) intermediolateral cell columns in the spinal cord
(12) Onufs nucleus in the spinal cord
(13) cerebellum
The variability in the sites affected underlies the variability in the clinical features.
Involvement in these areas can be identified on MRI.
Histologic findings:
(1) neuronal loss (atrophy)
(2) astrocytosis
(3) glial cytoplasmic inclusions (GCI) in oligodendrocytes
The GCIs stain positively for:
(1) tau protein (variable)
(2) alpha-synuclein
(3) ubiquitin
