Amyotrophic lateral sclerosis with dementia: an autopsy case showing many Bunina bodies, tau-positive neuronal and astrocytic plaque-like pathologies, and pallido-nigral degeneration

2006 ◽  
Vol 112 (5) ◽  
pp. 633-645 ◽  
Author(s):  
Osamu Yokota ◽  
Kuniaki Tsuchiya ◽  
Tatsurou Oda ◽  
Takeshi Ishihara ◽  
Rohan de Silva ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Autopsy Case ◽  
Bunina Bodies ◽  
Astrocytic Plaque ◽  
Nigral Degeneration ◽  
Lateral Sclerosis

scholarly journals An Autopsy Case of Amyotrophic Lateral Sclerosis with Waldenström Macroglobulinemia and Anti-MAG Gammopathy

2011 ◽  
Vol 3 (3) ◽  
pp. 294-300 ◽  
Author(s):  
Snejana Jurici ◽  
Annie Laquerrière ◽  
Anne-Laure Bedat-Millet ◽  
Fabrice Jardin ◽  
Lucile Musset ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Autopsy Case ◽  
Waldenström Macroglobulinemia ◽  
Waldenstrom Macroglobulinemia ◽  
Lateral Sclerosis

scholarly journals An autopsy case of amyotrophic lateral sclerosis with ampulla cardiomyopathy

2008 ◽  
Vol 48 (4) ◽  
pp. 249-254 ◽  
Author(s):  
Yumi Matsuyama ◽  
Naokazu Sasagasako ◽  
Akihiro Koike ◽  
Masashiro Matsuura ◽  
Takaomi Koga ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Autopsy Case ◽  
Ampulla Cardiomyopathy ◽  
Lateral Sclerosis

Concomitant alpha-synuclein pathology in an autopsy case of amyotrophic lateral sclerosis presenting with orthostatic hypotension and cardiac arrests

2013 ◽  
Vol 34 (2) ◽  
pp. 164-169 ◽  
Author(s):  
Takehiro Yamada ◽  
Kyoko Itoh ◽  
Koushun Matsuo ◽  
Yoshihiro Yamamoto ◽  
Youhei Hosokawa ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Orthostatic Hypotension ◽  
Alpha Synuclein ◽  
Autopsy Case ◽  
Lateral Sclerosis

scholarly journals Autophagy Is a Common Degradation Pathway for Bunina Bodies and TDP-43 Inclusions in Amyotrophic Lateral Sclerosis

2019 ◽  
Vol 78 (10) ◽  
pp. 910-921 ◽  
Author(s):  
Fumiaki Mori ◽  
Yasuo Miki ◽  
Tomoya Kon ◽  
Kunikazu Tanji ◽  
Koichi Wakabayashi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Cathepsin B ◽  
Cathepsin D ◽  
Early Stage ◽  
Degradation Pathway ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Bunina Bodies ◽  
Related Proteins ◽  
Lateral Sclerosis

Abstract Bunina bodies (BBs) coexisting with TDP-43-immunoreactive (TDP-43-IR) skein-like inclusions (SIs) and round inclusions (RIs) in lower motor neurons are a frequent feature of sporadic amyotrophic lateral sclerosis (sALS). Since previous studies have shown that BBs and TDP-43-IR inclusions are often detected in association with autophagy-related structures (autophagosomes and autolysosomes), we examined the anterior horn cells (AHCs) of the spinal cord from 15 patients with sALS and 6 control subjects, using antibodies against autophagy-related proteins (LC3, cathepsin B, and cathepsin D). Among AHCs with SIs, 43.9% contained BBs, whereas 51.7% of AHCs with RIs did so. The cytoplasm of AHCs showed diffuse immunoreactivity for LC3, cathepsin B and cathepsin D in both sALS and controls. Ultrastructurally, SIs and mature BBs contained autophagosomes and autolysosomes. Mature BBs were localized in the vicinity of SIs. RIs also contained autophagosomes, autolysosomes, and early-stage BBs. These findings suggest that autophagy is a common degradation pathway for BBs and TDP-43-IR inclusions, which may explain their frequent coexistence.

An autopsy case of familial amyotrophic lateral sclerosis with aTARDBPQ343R mutation

2015 ◽  
Vol 35 (5) ◽  
pp. 462-468 ◽  
Author(s):  
Koichi Okamoto ◽  
Yukio Fujita ◽  
Eri Hoshino ◽  
Yuhji Tamura ◽  
Toshio Fukuda ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Autopsy Case ◽  
Lateral Sclerosis

The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis

2002 ◽  
Vol 103 (6) ◽  
pp. 583-589 ◽  
Author(s):  
M. van Welsem ◽  
Hogenhuis J. ◽  
Meininger V. ◽  
Metsaars W. ◽  
Hauw J.-J. ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuronal Loss ◽  
Bunina Bodies ◽  
The Relationship ◽  
Lateral Sclerosis

scholarly journals An Autopsy Case Involving a 12-year History of Amyotrophic Lateral Sclerosis with CIDP-like Polyneuropathy

2014 ◽  
Vol 53 (12) ◽  
pp. 1371-1375 ◽  
Author(s):  
Tetsuya Akaishi ◽  
Maki Tateyama ◽  
Kazuhiro Kato ◽  
Emiko Miura ◽  
Rumiko Izumi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Autopsy Case ◽  
History Of ◽  
Lateral Sclerosis
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