Skip to main content

Myeloencephalopathy resembling Alexander's disease in a Scottish terrier dog

Summary

Diffuse degenerative myeloencephalopathy resembling Alexander's disease in a tetraparetic 9-month-old Scottish terrier dog is described. There were widespread deposits of Rosenthal fibers throughout brain and spinal cord in subpial, subependymal, perivascular and white matter regions. A diffuse proliferation of pleomorphic astrocytes in the white matter and subependymal areas was also seen. Demyelination was not a salient feature of the disease.

This is a preview of subscription content, access via your institution.

References

  1. 1.

    Alexander WS (1949) Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant. Brain 72:373–381

    Google Scholar 

  2. 2.

    Cole G, De Villiers F, Proctor NSF, Freiman I, Bill P (1979) Alexander's disease: case report including histopathological and electron microscopic features. J Neurol Neurosurg Psychiatry 42:619–624

    Google Scholar 

  3. 3.

    Escourolle R, Baecque C de, Gray F, Baumann N, Hauw J-J (1979) Etude en microscopic electronique et neurochimique d'un cas de maladie d'Alexander. Acta Neuropathol (Berl) 45:133–140

    Google Scholar 

  4. 4.

    Fankhauser R, Fatzer R, Bestetti G, Deruaz JP, Perentes E (1980) Encephalopathy with Rosenthal fibre formation in a sheep. Acta Neuropathol (Berl) 50:57–60

    Google Scholar 

  5. 5.

    Friede R (1964) Alexander's disease. Arch Neurol 11:414–422

    Google Scholar 

  6. 6.

    Garret R, Ames RP (1974) Alexander disease. Case report with electron microscopical studies and review of the literature. Arch Pathol 98:379–385

    Google Scholar 

  7. 7.

    Gerlach VH, Feist H, Holzhausen H-J, Koch E (1982) Alexandersche Krankheit. Zentralbl Allg Pathol 126:105–112

    Google Scholar 

  8. 8.

    Habib M, Hassoun J, Ali-Cherif A, Alonzo B, Toga M, Khalil R (1984) Maladie d'Alexander de l'adulte. Rev Neurol (Paris) 140:179–189

    Google Scholar 

  9. 9.

    Herndon RM, Rubinstein LJ, Freeman JM, Mathieson G (1970) Light and electron microscopic observations on Rosenthal fibers in Alexander's disease and in multiple sclerosis. J Neuropathol Exp Neurol 29:524–551

    Google Scholar 

  10. 10.

    McGrath JT (1980) Fibrinoid leukodystrophy (Alexander's disease). In: Andrews EJ, Ward BC, Altman NH (eds) Spontaneous animal models of human disease, vol 2. Academic Press, New York, pp 147–148

    Google Scholar 

  11. 11.

    Rizzuto N, Ferrari G, Piscioli A (1980) Diffuse Rosenthal fiber formation in adults. A case report. Acta Neuropathol (Berl) 50:237–240

    Google Scholar 

  12. 12.

    Rosenthal W (1898) Über eine eigentümliche, mit Syringomyelin komplizierte Geschwulst des Rückenmarks. Beitr Pathol Anat Allg Pathol 23:111–143

    Google Scholar 

  13. 13.

    Russo LS, Aron A, Anderson PJ (1976) Alexander's disease: a report and reappraisal. Neurology 26:607–614

    Google Scholar 

  14. 14.

    Schlote W (1966) Rosenthalsche “Fasern” und Spongioblasten in Zentralnervensystem. II. Elektronenmikroskopische Untersuchungen. Bedeutung der Rosenthalschen “Fasern”. Beitr Pathol Anat Allg Pathol 133:461–480

    Google Scholar 

  15. 15.

    Schochet SS Jr, Lampert PW, Earle KM (1968) Alexander's disease: a case report with electron microscopic observations. Neurology 18:543–549

    Google Scholar 

  16. 16.

    Spalke G, Mennel HD (1982) Alexander's disease in an adult: clinicopathologic study of a case and review of the literature. Clin Neuropathol 1:106–112

    Google Scholar 

  17. 17.

    Towfighi J, Young R, Sassani J, Ramer J, Horoupian DS (1983) Alexander's disease: further light-, and electron-microscopic observations. Acta Neuropathol (Berl) 61:36–42

    Google Scholar 

  18. 18.

    Townsend JJ, Wilson JF, Harris T, Coulter D, Fife R (1985) Alexander's disease. Acta Neuropathol (Berl) 67:163–166

    Google Scholar 

  19. 19.

    Vogel FS, Hallervorden J (1962) Leukodystrophy with diffuse Rosenthal fiber formation. Acta Neuropathol (Berl) 2:126–143

    Google Scholar 

  20. 20.

    Walls TJ, Jones RA, Cartlidge NEF, Saunders M (1984) Alexander's disease with Rosenthal fibre formation in an adult. J Neurol Neurosurg Psychiatry 47:399–403

    Google Scholar 

Download references

Author information

Affiliations

Authors

Corresponding author

Correspondence to N. R. Cox.

Additional information

Supported by the Scott-Ritchey Program and the Department of Pathology and Parasitology, Auburn University. Publication Number 1797

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Cox, N.R., Kwapien, R.P., Sorjonen, D.C. et al. Myeloencephalopathy resembling Alexander's disease in a Scottish terrier dog. Acta Neuropathol 71, 163–166 (1986). https://doi.org/10.1007/BF00687980

Download citation

Key words

  • Alexander's disease
  • Myeloencephalopathy
  • Rosenthal fibers
  • Dog