Skip to main content

Advertisement

Log in

Alexander’s disease in a bernese mountain dog

  • Cash Report
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

Abstract

We present a case of Alexander’s disease (AD) in a Bernese mountain dog. The male dog had a clinical history of tremors of the hind legs and posterior weakness, which deteriorated rapidly to posterior paresis and tetraparesis. After a disease duration of 4 weeks the dog was euthanatized at 13 weeks of age. Macroscopically the brain showed moderate enlargement of the lateral ventricles. Histologically there was marked proliferation of astrocytes with abnormally large cell bodies in the white matter of the brain and the white and gray matter of the spinal cord. In these regions numerous round, club-shaped, or elongated deposits consistent with Rosenthal fibers (RFs) were found. They were most prominent in perivascular, subependymal, and subpial areas where they were perpendicularly arranged. Additionally there was considerable loss of myelin. Immunohistologically the RFs were positive for glial fibrillary acidic protein and αB-crystallin. Unter the electron microscope the RFs were found to be located in the cell bodies and processes of astrocytes and appeared as osmiophilic irregularly formed bodies of uneven size with distinct borders that were tightly associated with glial filaments. The histological, immunohistochemical, and ultrastructural findings of this canine case of AD are identical with those in human cases.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Arend AO, Leary PM, Rutherford GS (1991) Alexander’s disease: a case report with brain biopsy, ultrasound, CT scan and MR1 findings. Clin Neuropathol 10:122–126

    PubMed  CAS  Google Scholar 

  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

    Article  PubMed  CAS  Google Scholar 

  3. Cox NR, Kwapien RP, Sorjonen DC, Braund KG (1986) Myeloencephalopathy resembling Alexander’s disease in a Scottish terrier dog. Acta Neuropathol (Berl) 71:163–166

    Article  CAS  Google Scholar 

  4. Escourolle R, DeBaecque C, Gray F, Baumann N, Hauw JJ (1979) Etude en microscopie electronique et neurochimique d’un cas de maladie d’Alexander. Acta Neuropathol (Berl) 45: 133–140

    Article  CAS  Google Scholar 

  5. Farrel K, Chuang S, Becker LE (1984) Computed tomography in Alexander’s disease. Ann Neurol 15:605–607

    Article  Google Scholar 

  6. 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

    Article  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  8. Goebel HH, Bode G, Caesar R, Kohlschütter A (1981) Bulbar palsy with Rosenthal fiber formation in the medulla of a 15-year-old girl. Localized form of Alexander’s disease? Neuropediatrics 12:382–391

    Article  PubMed  CAS  Google Scholar 

  9. Goldman JE, Corbin E (1991) Isolation of a major protein component of Rosenthal fibers. Am J Pathol 139:569–578

    Google Scholar 

  10. Goldman JE, Corbin E (1991) Rosenthal fibers contain ubiquitinated αB-crystallin. Am J Pathol 139:933–938

    PubMed  CAS  Google Scholar 

  11. Head MW, Corbin E, Goldman JE (1993) Overexpression and abnormal modification of the stress proteins αB-crystallin and hsp 27 in Alexander disease. Am J Pathol 143: 1743–1753

    PubMed  CAS  Google Scholar 

  12. Iwaki A, Iwaki T, Goldman JE, Ogomori K, Tateishi J, Sakaki Y (1992) Accumulation of αB-crystallin in brains of patients with Alexander’s disease is not due to an abnormality of the 5′-flanking and coding sequence of the genomic DNA. Neurosci Lett 140:89–92

    Article  PubMed  CAS  Google Scholar 

  13. Kato M, Herz F, Kato S, Hirano A (1992) Expression of stressresponse (heat-shock) protein 27 in human brain tumours: an immunohistochemical study. Acta Neuropathol 83:420–422

    Article  PubMed  CAS  Google Scholar 

  14. Kato S, Hirano A, Umahara T, Llena JF, Herz F, Ohama E (1992) Ultrastructural and immunohistochemical studies on ballooned cortical neurons in Creutzfeld-Jacob disease: expression of αB-crystallin, ubiquitin and stress-response protein 27. Acta Neuropathol 84:443–448

    PubMed  CAS  Google Scholar 

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

    Google Scholar 

  16. Neal JW, Cave EM, Shingrao SK, Cole G, Wallace SJ (1992) Alexander’s disease in infancy and childhood: a report of two cases. Acta Neuropathol 84:322–327

    Article  PubMed  CAS  Google Scholar 

  17. Ochi N, Kobayashi K, Maehara M, Nakayama A, Negoro T, Shinohara H, Watanabe K, Nagatsu T, Kato K (1991) Increment of αB-crystallin mRNA in the brain of patients with infantile type Alexander’s disease. Biochem Biophys Res Commun 179:1030–1035

    Article  PubMed  CAS  Google Scholar 

  18. Ogosawara N (1965) Multiple Sklerose mit Rosenthalschen Fasern. Acta Neuropathol (Berl) 5:61–68

    Article  Google Scholar 

  19. Pridmore CL, Baraitser M, Harding B, Boyd SG, Kendall B, Brett EM (1993) Alexander’s disease: clues to diagnosis. J Child Neurol 8:134–144

    Article  PubMed  CAS  Google Scholar 

  20. Richardson JA, Tang K, Burns DK (1991) Myeloencephalopathy with Rosenthal fiber formation in a Miniature Poodle. Vet Pathol 28:536–538

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  23. Sherwin RM, Berthrong M (1970) Alexander’s disease with sudanophilic leukodystrophy. Arch Pathol 89:321–328

    PubMed  CAS  Google Scholar 

  24. Sorjonen DC, Cox NR, Kwapien RP (1987) Myeloencephalopathy with eosinophilic refractile bodies (RFs) in a Scottish Terrier. J Am Vet Med Assoc 190: 1004–1006

    PubMed  CAS  Google Scholar 

  25. Tomokane N, Iwaki T, Tateishi J, Iwaki A, Goldman JE (1991) Rosenthal Fibers share epitopes with αB-crystallin, glial fibrillary acidic protein, and ubiquitin, but not with vimentin. Am J Pathol 138:875–885

    PubMed  CAS  Google Scholar 

  26. 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

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Weissenböck, H., Obermaier, G. & Dahme, E. Alexander’s disease in a bernese mountain dog. Acta Neuropathol 91, 200–204 (1996). https://doi.org/10.1007/s004010050414

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s004010050414

Key words

Navigation