Thorn-shaped astrocytes in the depth of cortical sulci in Western Pacific ALS/Parkinsonism-Dementia complex
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Thorn‑shaped astrocytes in the depth of cortical sulci in Western Pacific ALS/Parkinsonism‑Dementia complex Gabor G. Kovacs1,2,3,4 · John L. Robinson1 · Daniel P. Perl5 · Virginia M.‑Y. Lee1 · John Q. Trojanowski1 Received: 9 April 2020 / Revised: 2 July 2020 / Accepted: 3 July 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is a progressive neurodegenerative disorder characterized by diverse neuronal and glial tau pathologies [6]. With the aim to evaluate aging-related tau astrogliopathy (ARTAG) we examined the collection at the CNDR, University of Pennsylvania, consisting of blocks of the frontal parietal, temporal, and occipital cortices in 33, the hippocampus in 13, and the spinal cord samples in 30 additional cases. Formalin fixed, paraffin-embedded tissue blocks were evaluated using anti-tau antibodies PHF-1 (Ser396/Ser404, 1:2000; Gift of Peter Davies) and AT8 (phospho-epitope Ser202/Thr205; Pierce Biotechnology, Rockford, IL, USA). In addition to neuronal and oligodendroglial tau pathology, we observed astrocytic tau pathology reminiscent of granular/fuzzy astrocytes (GFAs) as in gray matter ARTAG [3] (Fig. 1a, b). Furthermore, thorn-shaped astrocytes (TSAs) in subpial location (Fig. 1c) and in the
* Gabor G. Kovacs [email protected] * John Q. Trojanowski [email protected] 1
Center for Neurodegenerative Disease Research (CNDR), Institute On Aging and Department of Pathology and Laboratory Medicine, University of Pennsylvania, 3600 Spruce Street, 3 Maloney Building, Philadelphia, PA 19104‑4283, USA
2
Tanz Centre for Research in Neurodegenerative Disease (CRND), University of Toronto, Krembil Discovery Tower, 60 Leonard Ave, Toronto, ON M5T 0S8, Canada
3
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada
4
Laboratory Medicine Program, University Health Network, Toronto, ON, Canada
5
Department of Pathology, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD 20814, USA
white (Fig. 1d) with gray matter GFAs (Fig. 1e) were also observed. Twenty-one out of 33 cases (63%; 2 controls, 14 PDC and 5 without clinical classification; median age 69 years, range 55–93 years) showed subpial TSAs diffusely along the cortical surface in one or more cortical regions. Fourteen of the 21 cases (66%) showed white matter TSAs in the same area. Subpial TSAs were variably seen in the frontal (n = 14 out of 23 where region was available for examination; 60.8%), temporal (12/26; 46%), parietal (7/17; 41.1%), and occipital cortices (1/11; 9%). One case (73-year-old female patient with PDC) showed diffuse subpial TSAs in all four cortical regions (out of 8 where all regions were available). Three PDC showed involvement of the frontal, temporal, and parietal cortices, and 2 each (PDC) of frontal and temporal and parietal and temporal cortices together. Accumulation of TSAs in the depth of the sulci (Fig. 1e, f)
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