Neuropathology of Speech Network Distinguishes Bulbar From Nonbulbar Amyotrophic Lateral Sclerosis.
Amyotrophic lateral sclerosis (ALS)
Bulbar
Frontotemporal lobar degeneration (FTLD)
Immunohistochemistry
Neuropathology
Speech network
Journal
Journal of neuropathology and experimental neurology
ISSN: 1554-6578
Titre abrégé: J Neuropathol Exp Neurol
Pays: England
ID NLM: 2985192R
Informations de publication
Date de publication:
01 03 2020
01 03 2020
Historique:
received:
26
10
2019
accepted:
26
11
2019
pubmed:
18
1
2020
medline:
22
7
2020
entrez:
18
1
2020
Statut:
ppublish
Résumé
Bulbar amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative subtype affecting speech and swallowing motor functions as well as associated with the burden of cognitive deficits. The neuroanatomical underpinnings of bulbar ALS are not well understood. The aim of this study was to compare neuropathology of the speech network (SpN) between 3 cases of bulbar-onset ALS (bALS), 3 cases of spinal-onset ALS (sALS) with antemortem bulbar ALS (sALSwB) against 3 sALS without antemortem bulbar ALS (sALSnoB) and 3 controls. Regional distribution and severity of neuronal loss, TDP-43 (transactive response DNA-binding protein of 43 kDa), and tau proteinopathy were examined. All 3 bALS cases showed marked neuronal loss and severe proteinopathy across most SpN regions; sALSwB cases showed no neuronal loss but mild and variable TDP-43 pathology in focal regions; sALSnoB cases demonstrated an absence of pathology. Two bALS cases had coexisting tauopathy in SpN regions, which was not noted in any sALS cases. The findings suggested that bALS may have a distinct neuropathological signature characterized by marked neuronal loss and polypathology in the SpN. Milder TDP-43 pathology in the SpN for sALSwB cases suggested a link between severity of bulbar ALS and SpN damage. Findings support a clinicopathologic link between bulbar symptoms and pathology in the SpN.
Identifiants
pubmed: 31951003
pii: 5708971
doi: 10.1093/jnen/nlz130
pmc: PMC7036661
doi:
Substances chimiques
DNA-Binding Proteins
0
MAPT protein, human
0
TARDBP protein, human
0
tau Proteins
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
284-295Subventions
Organisme : NIDCD NIH HHS
ID : R01 DC009890
Pays : United States
Organisme : NIDCD NIH HHS
ID : R01 DC013547
Pays : United States
Informations de copyright
© 2019 American Association of Neuropathologists, Inc. All rights reserved.
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