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. 2023 Aug 30;146(4):631–645. doi: 10.1007/s00401-023-02625-6

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© The Author(s) 2023

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 2 — PSP neuropathology in anti-IgLON5 disease (patient 1). Neuropathology shows neuronal loss and the presence of neurofibrillary tangles in the tegmentum of the midbrain, pons, and medulla oblongata (a–c; AT8), subthalamic nucleus (d; H&E), substantia nigra pars compacta (e; H&E), locus coeruleus (f; AT8; upper rectangle in b enlarged in f), and magnocellular nuclei of the medulla oblongata (g; AT8, upper rectangle in c enlarged in g) that were strongly immunoreactive for hTau, particularly for 4-repeat tau isoforms (h, substantia nigra; RD4) and negative for 3-repeat tau isoforms (i, substantia nigra; RD3). In addition, neurofibrillary tangles and neuropil threads were visible in the pontine base (j; AT8; lower rectangle in b enlarged in j), synaptic glomeruli of the cerebellar cortex (k; AT8; arrows), and olivary nucleus (l; AT8; lower rectangle in c enlarged in l). Glial pathology in basal ganglia consists of tufted astrocytes (m; AT8), some granular fuzzy astrocytes (n; GFAP), and oligodendroglial coiled bodies (o; AT8). Scale bars: d, e, k, m, n, o: 50 μm; f–j, l: 100 μm