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