Parkinsonism, dementia and vertical gaze palsy in a Guamanian with atypical neuroglial degeneration

A 58-year-old Chamorro female patient, who died in 1993, was examined clini copathologically. At the age of 51, she suffered from hemiparkinsonism, the n bradykinesia, rigidity without tremor, and dementia. Extrapyramidal sympt oms developed, and at the age of 57, vertical gaze palsy was noted. The cli nical diagnosis was parkinsonism-dementia complex (PDC) with vertical gaze palsy. The brain showed atrophy in the frontal and temporal lobes, and the atrophy was accentuated in the dentate gyrus, Ammon's horn and parahippocam pal gyrus. The basal ganglia, thalamus and midbrain were moderately atrophi c. The substantia nigra and locus ceruleus were completely depigmented. Num erous neurofibrillary tangles (NFTs) were seen in the subiculum and amygdal oid nucleus. Many NFTs were evident in the parahippocampal gyrus, lateral o ccipitotemporal gyrus, insula, Sommer sector, basal nucleus of Meynert, lat eral nucleus of the thalamus, subthalamic nucleus and brain stem, and sever al were observed in the globus pallidus and hypothalamus, The Sommer sector , substantia nigra, locus ceruleus and basal nucleus of Meynert showed seve re loss of neurons, and a moderate loss of neurons was exhibited by the glo bus pallidus. These findings were apparently consistent with those associat ed with PDC. However, in this patient, severe neuronal loss was seen in the subthalamic nucleus and lateral nucleus of the thalamus, and grumose degen eration, which has not previously been reported in PDC, was seen in the den tate nucleus, In addition, many tufted astrocytes, which have been reported to occur in progressive supranuclear palsy (PSP) and postencephalitic park insonism, but scarcely observed in PDC, were present. Furthermore, astrocyt ic plaques, which have been considered as a specific finding of corticobasa l degeneration (CBD), were observed in the cerebral cortex. On the other ha nd, granular hazy astrocytic inclusions, previously reported to occur in PD C, were not seen. Chromatolytic neurons were not observed. The question thu s arises as to whether it is appropriate to consider this patient as having suffered from a combination of PDC, PSP and CBD. From the view points of a bsence of granular hazy astrocytic inclusions and chromatolytic neurons, an d of tufted astrocytes in the neostriatum, it is conceivable that this pati ent is a case of a new disease entity.