Abstract
We report an encephalomyelopathy in three 18-month-old Merino sheep with features of adult-onset Alexander's disease (AD), a human primary astrocytic disorder. The signature histologic finding was the presence of numerous hypereosinophilic, intra-astrocytic inclusions (Rosenthal fibers), mainly in perivascular, subpial, and subependymal sites, especially in the caudal brain stem and spinal cord. Although AD usually results from mutations in the glial fibrillary acidic protein (GFAP) gene, no such mutation was detected in these sheep. However, the annual clinical presentation of this disorder in a few sheep in the affected flock is suggestive of a familial pattern of occurrence.
MeSH terms
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Alexander Disease / genetics
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Alexander Disease / pathology
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Alexander Disease / veterinary*
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Animals
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Astrocytes / pathology*
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Astrocytes / ultrastructure
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Brain / pathology
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Central Nervous System Diseases / genetics
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Central Nervous System Diseases / pathology
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Central Nervous System Diseases / veterinary
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Diagnosis, Differential
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Female
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Glial Fibrillary Acidic Protein / genetics
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Glial Fibrillary Acidic Protein / metabolism*
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Humans
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Mutation
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Pregnancy
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Sheep
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Sheep Diseases / diagnosis
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Sheep Diseases / genetics
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Sheep Diseases / pathology*
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South Australia
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Spinal Cord / pathology
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alpha-Crystallin B Chain / metabolism*
Substances
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Glial Fibrillary Acidic Protein
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alpha-Crystallin B Chain