TY - JOUR
T1 - Morphological characterisation of glial and neuronal tau pathology in globular glial tauopathy (Types II and III)
AU - Tanaka, H.
AU - Toyoshima, Y.
AU - Kawakatsu, S.
AU - Kobayashi, R.
AU - Yokota, O.
AU - Terada, S.
AU - Kuroda, S.
AU - Miura, T.
AU - Higuchi, Y.
AU - Otsu, H.
AU - Sanpei, K.
AU - Otani, K.
AU - Ikeuchi, T.
AU - Onodera, O.
AU - Kakita, A.
AU - Takahashi, H.
N1 - Funding Information:
We thank C. Tanda, J. Takasaki, A. Minami, and S. Nigorikawa for their technical assistance, and M. Yoshida and Y. Furukane for secretarial assistance. This work was supported by Grants‐in‐Aid, 26430052 (to Y. T.), and 26250017 and 17H03554 (to H. T.), for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology, Japan.
Publisher Copyright:
© 2019 British Neuropathological Society
PY - 2020/6/1
Y1 - 2020/6/1
N2 - Aims: Globular glial tauopathy (GGT) is a new category within the 4-repeat tauopathies that is characterised neuropathologically by tau-positive globular glial inclusions (GGIs), namely, globular oligodendrocytic and astrocytic inclusions (GOIs and GAIs). Occurrence of tau-positive neuronal cytoplasmic inclusions (NCIs) is also a feature. GGT is classified into three pathological subtypes (Types I, II and III). We studied the tau pathology in 6 cases of GGT (Type II, n = 3; Type III, n = 3), with special reference to GAIs and NCIs. Methods: Neuropathological examinations were conducted, along with immunohistochemistry, morphometry and three-dimensional imaging, and biochemical and genetic analysis of tau. Results: The cortical GAIs in Type II and those in Type III were distinguishable from each other. In the motor cortex, GAIs were much more numerous in Type III than in Type II. Prominent occurrence of perikaryal globular structures was a feature of GAIs in Type III. By contrast, prominent occurrence of radiating process-like structures was a feature of GAIs in Type II. Overall, the GAIs were significantly smaller in Type III than in Type II. NCIs were divisible into three subgroups in terms of shape: diffuse granular, thick cord-like, and round/horseshoe-shaped structures. In all cases, NCIs were a feature of the upper and lower motor neurons. Interestingly, the round/horseshoe-shaped NCIs were observed only in Type III cases. Conclusions: These findings, which characterised GAIs and NCIs, indicated that Type II and Type III constitute two distinct pathological subtypes, and also further strengthen the concept of GGT as a distinct entity.
AB - Aims: Globular glial tauopathy (GGT) is a new category within the 4-repeat tauopathies that is characterised neuropathologically by tau-positive globular glial inclusions (GGIs), namely, globular oligodendrocytic and astrocytic inclusions (GOIs and GAIs). Occurrence of tau-positive neuronal cytoplasmic inclusions (NCIs) is also a feature. GGT is classified into three pathological subtypes (Types I, II and III). We studied the tau pathology in 6 cases of GGT (Type II, n = 3; Type III, n = 3), with special reference to GAIs and NCIs. Methods: Neuropathological examinations were conducted, along with immunohistochemistry, morphometry and three-dimensional imaging, and biochemical and genetic analysis of tau. Results: The cortical GAIs in Type II and those in Type III were distinguishable from each other. In the motor cortex, GAIs were much more numerous in Type III than in Type II. Prominent occurrence of perikaryal globular structures was a feature of GAIs in Type III. By contrast, prominent occurrence of radiating process-like structures was a feature of GAIs in Type II. Overall, the GAIs were significantly smaller in Type III than in Type II. NCIs were divisible into three subgroups in terms of shape: diffuse granular, thick cord-like, and round/horseshoe-shaped structures. In all cases, NCIs were a feature of the upper and lower motor neurons. Interestingly, the round/horseshoe-shaped NCIs were observed only in Type III cases. Conclusions: These findings, which characterised GAIs and NCIs, indicated that Type II and Type III constitute two distinct pathological subtypes, and also further strengthen the concept of GGT as a distinct entity.
KW - frontotemporal dementia
KW - frontotemporal lobar degeneration
KW - globular astrocytic inclusion
KW - globular glial inclusion
KW - globular glial tauopathy
KW - motor neuron disease
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U2 - 10.1111/nan.12581
DO - 10.1111/nan.12581
M3 - Article
C2 - 31600825
AN - SCOPUS:85074763501
VL - 46
SP - 344
EP - 358
JO - Neuropathology and Applied Neurobiology
JF - Neuropathology and Applied Neurobiology
SN - 0305-1846
IS - 4
ER -