Spongiform central nervous system myelinopathy in african dwarf goats

• Published in: Journal of comparative pathology Vol. 113; no. 4; pp. 357 - 372
• Main Authors: Obermaier, G., Kretzschmar, H.A., Hafner, A., Heubeck, D., Dahme, E.
• Format: Journal Article
• Language: English
• Published: London Elsevier Ltd 01.11.1995; Elsevier
• Subjects: Animals; Base Sequence; Biological and medical sciences; Brain - pathology; Brain - ultrastructure; Cerebellum - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Goat Diseases - pathology; Goats; Heterozygote; Male; Medical sciences; Molecular Sequence Data; Neurology; Oligodendroglia - ultrastructure; Polymerase Chain Reaction; Prion Diseases - genetics; Prion Diseases - pathology; Prion Diseases - veterinary; Prions - genetics; Sequence Analysis, DNA; Sheep; Species Specificity; Spinal Cord - pathology; Vacuoles - pathology; Vacuoles - ultrastructure; Africa; Etiopathogenesis; Veterinary medicine; Myelopathy; Vertebrata; Mammalia; Histopathology; Microscopy; Etiology; Animal; Central nervous system disease; Degenerative disease; Goat; Artiodactyla; Ungulata
• ISSN: 0021-9975; 1532-3129
• DOI: 10.1016/S0021-9975(05)80121-5

A novel spongiform myelinopathy of the central nervous system (CNS) of eleven African dwarf goats was examined by light and electron microscopy. Histological lesions consisted of extensive vacuolation predominantly of the white matter of the diencephalon, midbrain and cerebellar peduncles, as well as of spinal white matter. Ultrastructurally, vacuoles were shown to be intramyelinic, resulting from the splitting of the outer myelin lamellae at the intraperiod line. A few oligodendrocytes showed vacuolar degeneration of cell bodies and processes. Inflammatory reactions were absent. The observed lesions point to an unknown primary damage of oligodendroglia and central myelin. A hereditary background of the disorder is suspected as all investigated dwarf goats were half-brothers or-sisters and partly descended from the mating of adult females with their own sire.