Canis ISSN: 2398-2942

Brain: cerebellum - congenital disorders

Contributor(s): Laurent Garosi, Rodney Bagley

Introduction

  • Several syndromes are recognized:
  • Seen from 6 weeks old.
  • Signs: non-progressive cerebellar signs.
  • Diagnosis: rule out other causes of similar signs, eg inflammatory and degenerative diseases.
  • Treatment: none.
  • Prognosis: guarded.

Pathogenesis

Etiology

  • Cerebellar abiotrophies: also called cerebellar cortical degeneration - it is a process by which cells develop normally but later degenerate because of an intrinsic cellular defect necessary for continued life of the neuron. Many of these diseases may result from underlying abnormalities of cellular metabolism or function. Known to be inherited in Old English Sheepdog, Gordon setter, Finnish Hound, Italian Spinone, Beagle and Brittany Spaniel.
  • Dandy Walker syndrome: is a congenital problem assumed to be associated with abnormal embryogenesis (primarily parenchymal midline developmental field defect of unknown origin). Represent partial or complete absence of the vermis of the cerebellum. Associated with these vermal defects is cyst-like dilation of the fourth ventricle. In addition many of these patients have dilated third and lateral ventricles (hydrocephalus), stenosis of the aqueduct, and absence of the corpus callosum.
  • Hypomyelination/dysmyelination: disease is inherited in an X-linked manner is Springer Spaniels.
  • Lysosomal storage diseases Storage disease progressive loss of neurone function → cerebellum degeneration.
  • Hypomyelination has been associated within uterocanine herpes virus infections Canine herpesvirus disease.

Pathophysiology

  • Failure of normal cerebellar development.
  • Degeneration of cerebellar neurones after the differentiation process is complete → inadequate function to control movement.
  • Dandy Walker syndrome: there is a malformation resulting in a cyst-like abnormality in the cerebellum. The lateral and third ventricles are commonly dilated concurrently.
  • Hypomyelination/dysmyelination: disorders of myelin synthesis and maintenance. In most cases the underlying derangement directly or indirectly affects the oligodendrocyte numbers or function.
  • Neuroaxonal dystrophy: underlying pathogenesis is unknown. The cell bodies in the grey matter are affected (axonal spheroids) throughout the nervous system except the cerebral cortex. The most severe lesions are in the spinocerebellar tracts and the Purkinje cells.

Timecourse

  • From 6 weeks of age (when puppies become more active).

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Van der Merwe L L, Lane E (2001) Diagnosis of cerebellar cortical degeneration in a Scottish terrier using magnetic resonance imaging. JSAP 42, 409-412 PubMed.
  • Tago Y et al (1993) Granule cell type cerebellar hypoplasia in a beagle dog. Lab Anim 27 (2), 151-155 PubMed.
  • Schmid V, Lang J & Wolf M (1992) Dandy-Walker-like syndrome in four dogs - Cisternography as a diagnostic aid. JAAHA 28 (4), 355-360 VetMedResource.
  • Kornegay J N (1986) Cerebellar vermian hypoplasia in dogs. Vet Pathol 23 (4), 374-379 PubMed.
  • Harari J et al (1983) Cerebellar agenesis in two canine littermates. JAVMA 182 (6), 622-623 PubMed.
  • Steinberg, H S, Troncoso, J C, Cork, L C & Price, D L (1981) Clinical features of inherited cerebellar degeneration in Gordon Setters. JAVMA 179 (9), 886-890 PubMed.
  • deLahunta A (1980) Comparative cerebellar disease in domestic animals. Comp Cont Educ 2, 8-19.
  • Holliday T A (1980) Clinical signs of acute and chronic experimental lesions of the cerebellum. Vet Sci Comm 3 (4), 259-78 VetMedResource.
  • Pearson H (1979) Changing attitudes to congenital and inherited diseases. Vet Rec 105 (14), 318-23 PubMed.

Other sources of information

  • Bagley R S, Platt S R (2013) Tremors, involuntary movements and paroxysmal disorders. In: Platt S E & Olby N (eds). BSAVA Manual of Canine and Feline Neurology. BSAVA, pp 233-251.
  • De Lahunta A, Glass E (2009) Veterinary Neuroanatomy and Clinical Neurology. 3rd edn. St. Louis, Saunders Elsevier, p 360-370.
  • Braund K G (1987) Degenerative and developmental diseases. In:Veterinary Neurology. Eds. J E Oliver Jr, B F Hoerlein & I B Mayhew. W B Saunders, Philadelphia. pp 186.
  • Chrisman C L (1986) Neuroaxonal dystrophy and Leukoencephalomyelopathy of Rottweiler dogs. In:Current Veterinary Therapy. 9th edn. Ed R W Kirk. W B Saunders, Philadelphia. pp 805-806.
  • deLahunta A (1983) In: Veterinary Neuroanatomy and Clinical Neurology. 2nd edn. W B Saunders, Philadelphia. pp 189.


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