Atypical globular glial tauopathy with a combination of types I and II pathology

• Published in: Neuropathology 2019-04, Vol.39 (2), p.127-134
• Main Authors: Kon, Tomoya, Mori, Fumiaki, Arai, Akira, Miki, Yasuo, Tanji, Kunikazu, Kurotaki, Hidekachi, Tomiyama, Masahiko, Wakabayashi, Koichi
• Format: Article
• Language: English
• Subjects: Aged, 80 and over; Astrocytes - pathology; Atrophy; Autopsy; Brain - pathology; Degeneration; Dementia disorders; Female; Frontotemporal Dementia - etiology; Frontotemporal Dementia - pathology; frontotemporal lobar degeneration; globular glial inclusion; globular glial tauopathy; Humans; Inclusion Bodies - pathology; Morphology; Neurodegenerative diseases; Neurofibrillary tangles; Neurofibrillary Tangles - pathology; Neuroglia - pathology; Oligodendroglia - pathology; Pathology; Phenotypes; Pyramidal tracts; Semantic dementia; Substantia alba; Tau protein; tau Proteins - metabolism; Tauopathies - complications; Tauopathies - pathology; Temporal lobe; ultrastructure
• ISSN: 0919-6544; 1440-1789
• DOI: 10.1111/neup.12536

Globular glial tauopathy (GGT) is a group of 4‐repeat tauopathies characterized by widespread globular glial inclusions (GGIs). GGT is now classified into three subtypes based on the distribution and morphology of the GGIs. We report an autopsy case of GGT in an 85‐year‐old woman who presented with semantic dementia, a rare phenotype in GGT. Postmortem examination revealed marked atrophy of the frontotemporal and motor cortices and corticospinal tract degeneration with widespread occurrence of globular neurofibrillary tangles and GGIs. The distribution of the pathology was similar to that seen in GGT type III. However, the morphology of astrocytic inclusions in the present case differed from that in type III. Moreover, the tau burden in the primary motor area was more severe in the gray than in the white matter, and globular oligodendroglial inclusions were more numerous than astrocytic inclusions, corresponding to GGT type II. By contrast, the tau pathology in the temporal lobe was chiefly globular oligodendroglial inclusions in the white matter, corresponding to GGT type I. Thus, the present case exhibited a combination of GGT types I and II pathology. Our findings appear to extend the pathological heterogeneity of GGT.