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Subpial Thorn-shaped Astrocytes Are Prevalent In Guam ALS/PDC

Published online by Cambridge University Press:  29 July 2021

G.G. Kovacs ,
J.L. Robinson ,
D.P. Perl ,
V.M.Y. Lee  and
J.Q. Trojanowski
G.G. Kovacs
Affiliation:
Laboratory Medicine Program, University Health Network, Toronto, Ontario, Canada
J.L. Robinson
Affiliation:
Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia, Pennsylvania, USA
D.P. Perl
Affiliation:
Uniformed Services University of the Health Sciences, Bethesda, MD, USA
V.M.Y. Lee
Affiliation:
Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia, Pennsylvania, USA
J.Q. Trojanowski
Affiliation:
Center for Neurodegenerative Disease Research, University of Pennsylvania, Philadelphia, Pennsylvania, USA
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Abstract

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Guam amyotrophic lateral sclerosis/parkinsonism-dementia complex is a progressive neurodegenerative disorder characterized by neuronal and glial tau pathologies. With the aim to evaluate aging-related tau astrogliopathy (ARTAG) we examined the collection at the University of Pennsylvania, consisting of blocks of the frontal parietal, temporal, and occipital cortices. Formalin fixed, paraffin-embedded tissue blocks were evaluated using anti-tau antibodies PHF-1 and AT8. In addition to neuronal and oligodendroglial tau pathology, granular/fuzzy astrocytes in the gray matter and thorn-shaped astrocytes (TSAs) in subpial location were also observed. Twenty-one out of 33 cases (63%) showed subpial TSAs diffusely along the cortical surface in one or more cortical regions. Accumulation of TSAs in the depth of the sulci were seen in 41% in the temporal, 7% in the frontal and 14% in parietal cortex. This was not associated with perivascular neuronal tau pathology in the depth of the sulci. Accumulation of TSAs in the depth of cortical sulci in this cohort is approximately 20 times more frequent than reported in a European aging cohort. The presence of subpial TSAs in the depth of cortical sulci in CTE and Guam PDC, and less frequently in aging brains, might suggest common mechanisms.

Learning Objectives

  • Describe the spectrum of neuropathology in Guam ALS/PDC

  • Describe the frequency of tau positive cortical subpial thorn-shaped astrocytes

Type
Abstracts
Information
Canadian Journal of Neurological Sciences , Volume 48 , Supplement s2: ABSTRACT: 60th Annual Canadian Association of Neuropathologists Meeting , July 2021 , pp. S9 - S10
Copyright
© The Canadian Journal of Neurological Sciences Inc. 2021