Glial involvement in diffuse Lewy body disease.
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, alpha-synuclein (alphaS) has been found to be a central constituent of LB. In DLBD, abnormal accumulation of alphaS 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. alphaS-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 alphaS. 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-Abeta component portion of alphaS 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 x100 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.
Terada
Seishi
S
Department of Neuropsychiatry, Okayama Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikata-cho, Japan. terada_1@cc.okayama-u.ac.jp
Ishizu
Hideki
H
Yokota
Osamu
O
Tsuchiya
Kuniaki
K
Nakashima
Hanae
H
Ishihara
Takeshi
T
Fujita
Daisuke
D
Uéda
Kenji
K
Ikeda
Kenji
K
Kuroda
Shigetoshi
S
D000368:Aged; D000369:Aged, 80 and over; D001253:Astrocytes / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D001921:Brain / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D020541:Coiled Bodies / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D016631:Lewy Bodies / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D020961:Lewy Body Disease / Q000378:metabolism / Q000473:pathology*; D008297:Male; D018613:Microscopy, Confocal; D008855:Microscopy, Electron, Scanning; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009457:Neuroglia / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D009474:Neurons / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D019600:Neuropil Threads / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D051843:Synucleins; D051844:alpha-Synuclein
D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein