Fibrinoid leukodystrophy

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White matter EM study, showing enlarged astrocyte with a cytoplasm densely packed with RF filaments[1]

Fibronioid leukodystrophy is a rare genetic disease of dogs characterised by localized disease of the white matter of the cerebellum and brain stem.

Clinical symptoms include weight loss, mental depression, anorexia, generalized weakness and vomiting. Neurological signs are inconsistent but may include progressive tetraparesis and tremors, particularly of the hind limbs.

A common complication is megaesophagus, which has been diagnosed in the Burnese Mountain Dog[2] and French Bulldog[3].

Diagnosis usually requires pre- or postmortem brain biopsy and use of immunohistochemistry for detection of glial fibrillary acidic protein (Rosenthal fibers throughout the white matter) is required for a definitive diagnosis. Dystrophic astrocytes, enlarged with abundant RF particles, is characteristic of this disease.

A differential diagnosis include canine distemper virus.

Treatment responses to prednisolone are poor and the disease invariably progresses, requiring euthanasia.

References

  1. Alemañ N et al (2006) Rosenthal fiber encephalopathy in a dog resembling Alexander disease in humans. Vet Pathol 43(6):1025-1028
  2. Weissenböck, H et al (1996) Alexander's disease in a Bernese mountain dog. Acta Neuropathol 91(2):200-204
  3. Ito T et al (2010) Fibrinoid leukodystrophy (Alexander's disease-like disorder) in a young adult French bulldog. J Vet Med Sci 72(10):1387-1390