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Case Reports
. 2023 Dec;43(6):463-471.
doi: 10.1111/neup.12906. Epub 2023 Apr 21.

Polyglucosan body disease in an aged chimpanzee (Pan troglodytes)

Sanjeev Gumber  1   2 , Fawn Connor-Stroud  3 , Dustin Howard  4 , Xiaodong Zhang  5 , Brenda J Bradley  4 , Chet C Sherwood  4 , Lary C Walker  6   7
Affiliations
Case Reports

Polyglucosan body disease in an aged chimpanzee (Pan troglodytes)

Sanjeev Gumber et al. Neuropathology. 2023 Dec.

Abstract

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.

Keywords: Lafora bodies; adult polyglucosan body disease (APBD); aging; corpora amylacea; neurodegenerative disease.

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Conflict of interest statement

DISCLOSURE

The authors declare no conflicts of interest for this report.

Figures

Figure 1.
Figure 1.
Profuse, structurally heterogeneous PGBs (purple spheroids) in the caudal spinal cord. The detectable lesions range in size from ~1 – 25μm in diameter. The image in B is the enlarged region in A denoted by the rectangle turned 90 degrees counterclockwise. PAS stain (Nissl counterstain). Scale bars = 200μm in A, 50μm in B, 30μm in C and D.
Figure 2.
Figure 2.
PGBs in the spinal cord detected by hematoxylin and eosin (panels A and B); ubiquitin immunostain (panels C and D); Bielschowsky silver stain (panel E); and Alcian Blue (panel F). Nissl counterstain in C, D and F. The images in B and D are the respective boxed regions in A and C turned 90 degrees counterclockwise. Scale bars = 200μm in A and C; 50μm in B, D, E and F.
Figure 3.
Figure 3.
PGBs in nerves of the cauda equina. A and B: Light-microscopic images of tissue stained with PAS (Nissl counterstain); scale bars = 30μm. C and D: Electron microscopic images of small, intra-axonal bodies; note the displacement of normal axonal contents and the disintegration of surrounding myelin sheaths. Scale bars = 1μm.
Figure 4.
Figure 4.
Ultrastructure of small PGBs in the spinal cord. Panel B is an enlargement of the boxed area in A. Scale bars = 500nm in A,B,C,E,F and 1000nm in D.
See this image and copyright information in PMC

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