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Related Experiment Videos

Alexander's disease in a Bernese mountain dog

H Weissenböck1, G Obermaier, E Dahme

  • 1Institute of Pathology and Forensic Veterinary Medicine, University of Veterinary Medicine, Vienna, Austria.

Acta Neuropathologica
|January 1, 1996
PubMed
Summary

Alexander's disease (AD) in a Bernese mountain dog presented with neurological deficits. Histopathology revealed Rosenthal fibers and myelin loss, identical to human AD cases.

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Area of Science:

  • Veterinary Neurology
  • Neurodegenerative Diseases
  • Canine Pathology

Background:

  • Alexander's disease (AD) is a rare, fatal, leukoencephalopathy characterized by the accumulation of Rosenthal fibers.
  • While primarily documented in humans, understanding AD in other species can offer comparative insights.

Purpose of the Study:

  • To document and characterize a case of Alexander's disease in a Bernese mountain dog.
  • To compare the pathological findings in this canine case with those reported in human AD.

Main Methods:

  • Clinical examination and neurological assessment of the affected dog.
  • Macroscopic and microscopic examination of the brain and spinal cord.
  • Immunohistochemical staining for glial fibrillary acidic protein and alpha B-crystallin.
  • Ultrastructural analysis using electron microscopy.

Main Results:

  • The dog exhibited progressive hind limb weakness, paresis, and tetraparesis.
  • Macroscopic findings included enlarged lateral ventricles.
  • Histopathology revealed astrogliosis with Rosenthal fiber deposition in the white matter of the brain and spinal cord, alongside myelin loss.
  • Immunohistochemistry confirmed Rosenthal fibers positive for GFAP and alpha B-crystallin.
  • Electron microscopy showed Rosenthal fibers within astrocytes, consistent with glial filaments.

Conclusions:

  • The histological, immunohistochemical, and ultrastructural findings in this canine case are identical to those observed in human Alexander's disease.
  • This case highlights the potential for Alexander's disease to occur in large breed dogs, presenting similar pathological hallmarks as in humans.

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