TY - JOUR
T1 - Glial involvement in diffuse Lewy body disease
AU - Terada, Seishi
AU - Ishizu, Hideki
AU - Yokota, Osamu
AU - Tsuchiya, Kuniaki
AU - Nakashima, Hanae
AU - Ishihara, Takeshi
AU - Fujita, Daisuke
AU - Uéda, Kenji
AU - Ikeda, Kenji
AU - Kuroda, Shigetoshi
N1 - Funding Information:
Acknowledgements The authors thank Dr. S. Engelender, Dr. Y. Tanaka, and Dr. C. A. Ross (Johns Hopkins University School of Medicine, Baltimore) for supplying polyclonal anti-synphilin 1 antibody. The authors also thank Ms. M. Onbe for her skillful technical assistance. This work was supported by a Research Grant from Zikei Institute of Psychiatry, Japan.
PY - 2003/2/1
Y1 - 2003/2/1
N2 - Diffuse Lewy body disease (DLBD) is characterized by the presence of Lewy bodies (LB) in the neurons and neurites of cortical, subcortical, and brain stem structures. Recently, α-synuclein (αS) has been found to be a central constituent of LB. In DLBD, abnormal accumulation of αS has been reported in both neurons and glia, but studies on glial lesions in DLBD have been limited. We examined in detail the constituents and distribution of glial lesions in eight patients with DLBD and report the pathogenesis of the glial lesions. αS-positive neuronal cytoplasmic inclusions (NI), neuropil threads (NT), and coiled bodies (CB) showed similar immunostaining profiles. Without pretreatment, NI, NT, and CB were detected by all antibodies against αS. The immunostaining profile of star-like astrocytes (SLA) was quite different from those of NI, NT, and CB. A few SLA were stained by an antibody against the non-Aβ component portion of αS without pretreatment, but formic acid pretreatment dramatically enhanced SLA immunoreactivity. SLA and CB were found in all eight brains with DLBD. SLA were scarce in the brain stem, but there were hundreds of SLA per visual field at ×100 magnification in the temporal cortex of most cases, while CB were found diffusely in both the cerebral cortex and brain stem, similar to NI. This suggests that the pathogenesis of SLA is different from those of NI and CB.
AB - Diffuse Lewy body disease (DLBD) is characterized by the presence of Lewy bodies (LB) in the neurons and neurites of cortical, subcortical, and brain stem structures. Recently, α-synuclein (αS) has been found to be a central constituent of LB. In DLBD, abnormal accumulation of αS has been reported in both neurons and glia, but studies on glial lesions in DLBD have been limited. We examined in detail the constituents and distribution of glial lesions in eight patients with DLBD and report the pathogenesis of the glial lesions. αS-positive neuronal cytoplasmic inclusions (NI), neuropil threads (NT), and coiled bodies (CB) showed similar immunostaining profiles. Without pretreatment, NI, NT, and CB were detected by all antibodies against αS. The immunostaining profile of star-like astrocytes (SLA) was quite different from those of NI, NT, and CB. A few SLA were stained by an antibody against the non-Aβ component portion of αS without pretreatment, but formic acid pretreatment dramatically enhanced SLA immunoreactivity. SLA and CB were found in all eight brains with DLBD. SLA were scarce in the brain stem, but there were hundreds of SLA per visual field at ×100 magnification in the temporal cortex of most cases, while CB were found diffusely in both the cerebral cortex and brain stem, similar to NI. This suggests that the pathogenesis of SLA is different from those of NI and CB.
KW - Coiled bodies
KW - Diffuse Lewy body disease
KW - Glia
KW - Star-like astrocyte
KW - α-Synuclein
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U2 - 10.1007/s00401-002-0622-9
DO - 10.1007/s00401-002-0622-9
M3 - Article
C2 - 12536227
AN - SCOPUS:0037313281
SN - 0001-6322
VL - 105
SP - 163
EP - 169
JO - Acta neuropathologica
JF - Acta neuropathologica
IS - 2
ER -