Polyglucosan body disease in an aged chimpanzee (Pan troglodytes).
Lafora bodies
adult polyglucosan body disease (APBD)
aging
corpora amylacea
neurodegenerative disease
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Dec 2023
Dec 2023
Historique:
revised:
31
03
2023
received:
13
02
2023
accepted:
04
04
2023
pmc-release:
21
10
2024
medline:
5
12
2023
pubmed:
22
4
2023
entrez:
22
04
2023
Statut:
ppublish
Résumé
A 57-year-old female chimpanzee presented with a brief history of increasing lethargy and rapidly progressive lower-limb weakness that culminated in loss of use. Postmortem examination revealed no significant gross lesions in the nervous system or other organ systems. Histological analysis revealed round, basophilic to amphophilic polyglucosan bodies (PGBs) in the white and gray matter of the cervical, thoracic, lumbar, and coccygeal regions of spinal cord. Only rare PGBs were observed in forebrain samples. The lesions in the spinal cord were polymorphic, and they were positively stained with hematoxylin, periodic acid Schiff, Alcian blue, toluidine blue, Bielschowsky silver, and Grocott-Gomori methenamine-silver methods, and they were negative for von Kossa and Congo Red stains. Immunohistochemical evaluation revealed reactivity with antibodies to ubiquitin, but they were negative for glial fibrillary acidic protein, neuron-specific enolase, neurofilaments, tau protein, and Aβ protein. Electron microscopy revealed non-membrane-bound deposits composed of densely packed filaments within axons and in the extracellular space. Intra-axonal PGBs were associated with disruption of the axonal fine structure and disintegration of the surrounding myelin sheath. These findings are the first description of PGBs linked to neurological dysfunction in a chimpanzee. Clinicopathologically, the disorder resembled adult PGB disease in humans.
Identifiants
pubmed: 37086019
doi: 10.1111/neup.12906
pmc: PMC10642523
mid: NIHMS1940167
doi:
Substances chimiques
polyglucosan
9012-72-0
Silver
3M4G523W1G
Glucans
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
463-471Subventions
Organisme : NINDS NIH HHS
ID : R24 NS092988
Pays : United States
Organisme : NIA NIH HHS
ID : R01 AG067419
Pays : United States
Organisme : NIA NIH HHS
ID : P01 AG026423
Pays : United States
Organisme : NIH HHS
ID : P51 OD011132
Pays : United States
Informations de copyright
© 2023 Japanese Society of Neuropathology.
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