No abstract

The most striking observation was marked atrophy with neural loss and gliosis in the caudate nucleus and putamen and, to a lesser extent, in the nucleus accumbens and globus pallidus (GP; patchy distribution, no clear rostrocaudal gradient). The neuropil showed rarefaction, vacuolation, scattered brown pigment, and marked reactive astrocytosis. Dopaminergic fibers in the striatum were well preserved as found using tyrosine hydroxylase immunohistochemistry. Tau immunohistochemistry showed thorn-shaped astrocytes in subpial and perivascular spaces at the base of the brain, consistent with aging-related tau astrogliopathy (ARTAG) 4 and sparse mediobasal NFTs. Hypothalamic perivascular taupositive neuritic dystrophy was consistent with sex-related tauopathy. ARTAG and sex-related tauopathy have no known clinical correlates.Autopsy findings were compatible with MLS. Mild Alzheimer's-type changes (Braak NFT III, Thal amyloid I), mild ARTAG, and hypothalamic perivascular dystrophy were considered coincidental.Only three other autopsies are reported with MLS. [5][6][7] Selective striatal atrophy, a mainly unaffected SN, an unaffected thalamus, and absence of Lewy bodies were described. Our results are similar. However, we performed more extensive immunohistochemistry to investigate the presence of other proteinopathies.Neuropathological hallmarks of MLS are atrophy of the caudate nucleus and putamen and, to a lesser extent, of the GP with dilated lateral ventricles, normal SN, normal dopaminergic neurons, and no distinctive neuronal or glial inclusions (α-synuclein, TDP-43, p62; tau: only age-related changes).

Background Most studies of progressive supranuclear palsy (PSP) have been conducted in White populations. Objective The objective of this study was to identify whether differences exist for patients with PSP among Whites, East Asians (EAs), and Native Hawaiians/Pacific Islanders (NHPIs) in Hawaii. Methods We conducted a single‐center, retrospective study of patients meeting Movement Disorder Society probable PSP criteria (2006–2021). Data variables included age of onset and diagnosis, comorbidities, and survival rate. Variables were compared across groups using Fisher's exact test, Kruskal–Wallis rank sum test, and log‐rank tests. Results A total of 94 (59 EAs, 9 NHPIs, 16 Whites, and 10 Others) patients were identified. Mean age ± standard deviation (in years) of symptom onset/diagnosis were both youngest in NHPIs (64.0 ± 7.2/66.3 ± 8.0) followed by Whites (70.8 ± 7.6/73.9 ± 7.8), then EAs (75.9 ± 8.2/79.2 ± 8.3) (P < 0.001). Median survival from diagnosis was significantly lower (P < 0.05) in NHPIs (2 years) compared with EAs (4 years) and Whites (6 years). Conclusions There may be racial disparities for PSP, and studies are needed to identify genetic, environmental, and socioeconomic contributions. © 2023 International Parkinson and Movement Disorder Society.