Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling
Lafora disease is an autosomal recessive glycogen-storage disorder resulting from an accumulation of toxic polyglucosan bodies (PGBs) in the central nervous system, which causes behavioral and neurologic symptoms in humans and other animals. In this case study, brains collected from two young adult...
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ftdoajarticles:oai:doaj.org/article:baa55cd065124ca486c61ededa9d9e5e 2023-05-15T13:13:02+02:00 Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling Madhu Ravi Atilano Lacson Margo Pybus Mark C. Ball 2022-06-01T00:00:00Z https://doi.org/10.3390/ani12131633 https://doaj.org/article/baa55cd065124ca486c61ededa9d9e5e EN eng MDPI AG https://www.mdpi.com/2076-2615/12/13/1633 https://doaj.org/toc/2076-2615 doi:10.3390/ani12131633 2076-2615 https://doaj.org/article/baa55cd065124ca486c61ededa9d9e5e Animals, Vol 12, Iss 1633, p 1633 (2022) moose circling blindness Lafora disease polyglucosan bodies Lewy body Veterinary medicine SF600-1100 Zoology QL1-991 article 2022 ftdoajarticles https://doi.org/10.3390/ani12131633 2022-12-31T02:03:48Z Lafora disease is an autosomal recessive glycogen-storage disorder resulting from an accumulation of toxic polyglucosan bodies (PGBs) in the central nervous system, which causes behavioral and neurologic symptoms in humans and other animals. In this case study, brains collected from two young adult free-ranging moose ( Alces alces ) cows that were seemingly blind and found walking in circles were examined by light and electron microscopy. Microscopic analysis of the hippocampus of the brain revealed inclusion bodies resembling PGBs in the neuronal perikaryon, neuronal processes, and neuropil. These round inclusions measuring up to 30 microns in diameter were predominantly confined to the hippocampus region of the brain in both animals. The inclusions tested α-synuclein-negative by immunohistochemistry, α-synuclein-positive with PAS, GMS, and Bielschowsky’s staining; and diastase-resistant with central basophilic cores and faintly radiating peripheral lines. Ultrastructural examination of the affected areas of the hippocampus showed non-membrane-bound aggregates of asymmetrically branching filaments that bifurcated regularly, consistent with PGBs in both animals. Additionally, α-synuclein immunopositivity was noted in the different regions of the hippocampus with accumulations of small granules ultrastructurally distinct from PGBs and morphologically compatible with alpha-synucleinopathy (Lewy body). The apparent blindness found in these moose could be related to an injury associated with secondary bacterial invasion; however, an accumulation of neurotoxicants (PGBs and α-synuclein) in retinal ganglions cells could also be the cause. This is the first report demonstrating Lafora disease with concurrent alpha-synucleinopathy (Lewy body neuropathy) in a non-domesticated animal. Article in Journal/Newspaper Alces alces Directory of Open Access Journals: DOAJ Articles Animals 12 13 1633 |
institution |
Open Polar |
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Directory of Open Access Journals: DOAJ Articles |
op_collection_id |
ftdoajarticles |
language |
English |
topic |
moose circling blindness Lafora disease polyglucosan bodies Lewy body Veterinary medicine SF600-1100 Zoology QL1-991 |
spellingShingle |
moose circling blindness Lafora disease polyglucosan bodies Lewy body Veterinary medicine SF600-1100 Zoology QL1-991 Madhu Ravi Atilano Lacson Margo Pybus Mark C. Ball Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
topic_facet |
moose circling blindness Lafora disease polyglucosan bodies Lewy body Veterinary medicine SF600-1100 Zoology QL1-991 |
description |
Lafora disease is an autosomal recessive glycogen-storage disorder resulting from an accumulation of toxic polyglucosan bodies (PGBs) in the central nervous system, which causes behavioral and neurologic symptoms in humans and other animals. In this case study, brains collected from two young adult free-ranging moose ( Alces alces ) cows that were seemingly blind and found walking in circles were examined by light and electron microscopy. Microscopic analysis of the hippocampus of the brain revealed inclusion bodies resembling PGBs in the neuronal perikaryon, neuronal processes, and neuropil. These round inclusions measuring up to 30 microns in diameter were predominantly confined to the hippocampus region of the brain in both animals. The inclusions tested α-synuclein-negative by immunohistochemistry, α-synuclein-positive with PAS, GMS, and Bielschowsky’s staining; and diastase-resistant with central basophilic cores and faintly radiating peripheral lines. Ultrastructural examination of the affected areas of the hippocampus showed non-membrane-bound aggregates of asymmetrically branching filaments that bifurcated regularly, consistent with PGBs in both animals. Additionally, α-synuclein immunopositivity was noted in the different regions of the hippocampus with accumulations of small granules ultrastructurally distinct from PGBs and morphologically compatible with alpha-synucleinopathy (Lewy body). The apparent blindness found in these moose could be related to an injury associated with secondary bacterial invasion; however, an accumulation of neurotoxicants (PGBs and α-synuclein) in retinal ganglions cells could also be the cause. This is the first report demonstrating Lafora disease with concurrent alpha-synucleinopathy (Lewy body neuropathy) in a non-domesticated animal. |
format |
Article in Journal/Newspaper |
author |
Madhu Ravi Atilano Lacson Margo Pybus Mark C. Ball |
author_facet |
Madhu Ravi Atilano Lacson Margo Pybus Mark C. Ball |
author_sort |
Madhu Ravi |
title |
Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
title_short |
Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
title_full |
Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
title_fullStr |
Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
title_full_unstemmed |
Lafora Disease and Alpha-Synucleinopathy in Two Adult Free-Ranging Moose ( Alces alces ) Presenting with Signs of Blindness and Circling |
title_sort |
lafora disease and alpha-synucleinopathy in two adult free-ranging moose ( alces alces ) presenting with signs of blindness and circling |
publisher |
MDPI AG |
publishDate |
2022 |
url |
https://doi.org/10.3390/ani12131633 https://doaj.org/article/baa55cd065124ca486c61ededa9d9e5e |
genre |
Alces alces |
genre_facet |
Alces alces |
op_source |
Animals, Vol 12, Iss 1633, p 1633 (2022) |
op_relation |
https://www.mdpi.com/2076-2615/12/13/1633 https://doaj.org/toc/2076-2615 doi:10.3390/ani12131633 2076-2615 https://doaj.org/article/baa55cd065124ca486c61ededa9d9e5e |
op_doi |
https://doi.org/10.3390/ani12131633 |
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Animals |
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12 |
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13 |
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1633 |
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1766255625736552448 |