Summary
Chronic wasting disease (CWD), a progressive and uniformly fatal neurological disorder, is characterized neuropathologically by intraneuronal vacuolation, spongiform change of the neuropil and astrocytic hyperplasia and hypertrophy. Ultrastructural neuropathological findings consist of (1) extensive vacuolation in neuronal processes, within myelin sheaths, formed by splitting at the major dense lines or within axons; (2) dystrophic neurites (dendrites, axonal preterminals and myelinated axons containing degenerating mitochondria and pleomorphic, electron-dense inclusion bodies); (3) prominent astrocytic gliosis; (4) amyloid plaques; and (5) giant neuronal autophagic vacuoles. Other findings include activated macrophages and occasional spheroidal structures containing densely packed fibrillar material of unknown origin, abundant structures suggestive of degenerating microtubules entrapped in filamentous masses, vacuoles and myelin figures. Similar findings have been previously observed in scrapie-infected hamsters and Creutzfeldt-Jakob disease (CJD)-infected mice, bovine spongiform encephalopathy, and CJD indicating that CWD in captive mule deer belongs to the subacute spongiform encephalopathies (transmissible brain amyloidoses).
Similar content being viewed by others
References
Bahmanyar S, Williams ES, Johnson FB, Young S, Gajdusek DC (1985) Amyloid plaques in spongiform encephalopathy of mule deer. J Comp Pathol 95:1–5
Beck E, Daniel PM (1979) Kuru and Creutzfeldt-Jakob disease: neuropathological lesions and their significance. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 253–270
Beck E, Daniel PM (1987) Neuropathology of transmissible spongiform encephalopathies. In: Prusiner SB, McKinley MP (eds) Prions. Academic Press, New York, pp 331–385
Boellard JW, Schlote W, Tateishi J (1989) Neuronal autophagy in experimental Creutzfeldt-Jakob disease. Acta Neuropathol 78:410–418
Chou SM, Payne WN, Gibbs CJ Jr, Gajdusek DC (1980) Transmission and scanning electron microscopy of spongiform change in Creutzfeldt-Jakob disease. Brain 103:885–994
DeArmond SJ, Kretzschmar HA, McKinley MP, Prusiner SB (1987) Molecular pathology of prion diseases. In: Prusiner SB, McKinley MP (eds) Prions. Academic Press, New York, pp 278–310
Fraser H (1979) Nèuropathology of scrapie: the precision of the lesions and their diversity. In: Prusiner SB, Hadlow WJ (eds) Slow transmissible diseases of the nervous system, vol 1. Academic Press, New York, pp 387–406
Gibson PH, Liberski PP (1987) An electron and light microscopic study of the numbers of dystrophic neurites and vacuoles in the hippocampus of mice infected intracerebrally with scrapie. Acta Neuropathol (Berl) 73:379–382
Guiroy DC, Williams ES, Yanagihara R, Gajdusek DC (1991) Topographic distribution of scrapie amyloid-immunoreactive plaques in chronic wasting disease in captive mule deer (Odocoileus hemionus · hemionus). Acta Neuropathol 81:475–478
Guiroy DC, Williams ES, Yanagihara R, Gajdusek DC (1991) Immunolocalization of scrapie amyloid (PrP27-30) in chronic wasting disease of Rocky Mountain elk and hybrids of captive mule deer and white-tailed deer. Neurosci Lett 126:195–198
Guiroy DC, Yanagihara R, Gajdusek DC (1991) Localization of amyloidogenic proteins and sulfated glycosaminoglycans in nontransmissible and transmissible cerebral amyloidoses. Acta Neuropathol 82:87–92
Guiroy DC, Marsh R, Yanagihara R, Gajdusek DC (1991) Immunolocalization of scrapie amyloid in non-congophilic and non-birefringent deposits in golden Syrian hamsters with transmissible mink encephalopathy. Neurology 41:217
Guiroy DC, Williams ES, Yanagihara R, Gajdusek DC (1992) Neurodegenerative and immunohistochemical changes of chronic wasting disease in captive mule. J Cell Biochem [Suppl] 16:211
Jellinger K (1973) Neuroaxonal dystrophy: its natural history and related disorders. In: Zimmerman HM (ed) Progress in neuropathology, vol 2. Grune and Stratton, New York, pp 129–180
Kaneko Y, Kitamoto T, Tateishi J, Yamaguchi K (1989) Ferritin immunohistochemistry as a marker for microglia. Acta Neuropathol 79:129–136
Kim JH, Manuelidis EE (1986) Serial ultrastructural study of experimental Creutzfeldt-Jakob disease in guinea pigs. Acta Neuropathol (Berl) 69:81–90
Klatzo I, Gajdusek DC, Zigas V (1959) Pathology of kuru. Lab Invest 8:799–847
Lampert PW, Hooks J, Gibbs CJ Jr, Gajdusek DC (1969) Altered plasma membranes in experimental scrapie. Acta Neuropathol (Berl) 19:81–93
Liberski PP (1987) Electron microscopic observations on dystrophic neurites in hamster brains with the 263K strain of scrapie. J Comp Pathol 97:35–39
Liberski PP (1990) Ultrastructural neuropathologic features of bovine spongiform encephalopathy. J Am Vet Med Assoc 196:1683
Liberski PP (1992) Neuronal degeneration in unconventional slow virus disorders. In: Liberski PP (ed) Light and electron microscopic neuropathology of slow virus disorders. CRC Press, Boca Raton (in press)
Liberski PP, Yanagihara R, Gibbs CJ, Jr, Gajdusek DC (1989) Scrapie as a model for neuroaxonal dystrophy. Exp Neurol 106:133–141
Liberski PP, Yanagihara R, Gibbs CJ, Jr, Gajdusek DC (1990) Re-evaluation of experimentel Creutzfeldt-Jakob disease. serial ultrastructural studies of the Fujisaki strain of Creutzfeldt-Jakob virus in mice. Brain 113:121–137
Masters CL, Richardson EP Jr (1978) Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): the nature and progression of spongiform change. Brain 101:133–144
Masters CL, Kakulas BA, Alpers MP, Gajdusek DC, Gibbs CJ Jr (1976) Preclinical lesions and their progression in the experimental spongiform encephalopathies (kuru and Creutzfeldt-Jakob disease) in primates. J Neuropathol Exp Neurol 35:593–604
Palmer AC (1960) Vacuolated neurons in sheep affected with scrapie. Nature 179:480–481
Snow AD, Kisilevsky R, Willmer J, Prusiner SB, DeArmond SJ (1989) Sulfated glycosaminoglycans in amyloid plaques of prion diseases. Acta Neuropathol 77:337–342
Wilesmith JW, Wells GAH, Cranwell MP, Ryan JBM (1988) Bovine spongiform encephalopathy: epidemiological studies. Vet Rec 123:638–644
Williams ES, Young S (1980) Chronic wasting disease of captive mule deer: a spongiform encephalopathy. J Wildl Dis 16:89–98
Williams ES, Young S (1982) Spongiform encephalopathy of Rocky Mountain elk. J Wildl Dis 18:465–471
Zlotnik I (1957) Significance of vacuolated neurons in the medulla of sheep affected with scrapie. Nature 180:393–394
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Guiroy, D.C., Williams, E.S., Liberski, P.P. et al. Ultrastructural neuropathology of chronic wasting disease in captive mule deer. Acta Neuropathol 85, 437–444 (1993). https://doi.org/10.1007/BF00334456
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00334456