ISSN 2398-2969      

Brain: cerebellar disease

icanis
Contributor(s):

Kyle Braund

Laurent Garosi


Introduction

  • Diseases of the cerebellum lead to loss of the 'fine-tuning' of movements of the body and head.
  • Cause: many causes including vascular, inflammatory/infectious, neoplastic, congenital malformations, and degenerative disorders, eg lysosomal storage diseases Storage disease.
  • Signs: presents as truncal ataxia, hypermetria with preservation of strength, intention tremor of head, broad base stance, possibly decerebellate rigidity.
  • Involvement of adjacent structures may complicate the clinical picture; may have vestibular signs if flocculo-nodular lobe affected.
  • Diagnosis: signs.
  • Prognosis: good to poor depending on etiology.

Pathogenesis

Etiology

Degenerative

  • Abiotrophies (most are autosomal recessive diseases).
  • Neuroaxonal dystrophies (probably autosomal recessive diseases).
  • Demyelinating diseases.
  • Storage diseases Storage disease (most are autosomal recessive diseases).
  • Spongiform encephalopathies (probable autosomal recessive diseases).

Neoplastic

Nutritional

  • Thiamine deficiency.

Inflammatory

Toxic

Vascular

  • Infarction.
  • Septic emboli.
  • Hemorrhage.

Traumatic

Anomalous

Predisposing factors

General

Specific

  • Presently unknown.

Pathophysiology

  • Most degenerative disorders of the cerebellum are genetically transmitted (or suspected of being hereditary) and are characterized by premature aging with degeneration and death of various neuronal cell populations.
  • This mechanism of premature degeneration of cerebellar cortical neurons is termed 'abiotrophy'.
  • Abiotrophy is a process by which cells develop normally but later degenerate because of an intrinsic cellular defect necessary for continued life of the neuron.
  • These disorders tend to be breed specific.
  • The cerebellum is a reinforcing and coordinating organ that plays an important role in harmonising muscle contraction without actually initiating motor activity.
  • Due to its close association with the brainstem vestibular nuclei, the cerebellum also plays a role in the maintenance of equilibrium.
  • Cerebellar disease results in an inability to regulate the rate, range and force of a movement, ie dysmetria.
  • Limb movements tend to be clumsy, faltering and jerky.
  • Initiation of movement is delayed and often accompanied by tremors, ie 'intention' tremors. Infrequently observed signs include opisthotonos (lesions occurring in flocculonodular lobe or fastigial nuclear area of cerebellum).

Timecourse

  • Clinical signs frequently occur in young animals, usually within a few weeks or months after birth. The clinical course is usually progressive (weeks or months).

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 MRI. JSAP 42 (8), 403-408 PubMed.
  • Lipsitz D et al (1999) Magnetic resonance imaging of a choroid plexus carcinoma and meningeal carcinomatosis in a dog. Vet Radiol Ultrasound 40 (3), 246-250 PubMed.
  • Franklin R J M et al (1997) An inherited neurological disorder of the St. Bernard dog characterised by unusual cerebellar cortical dysplasia. Vet Rec 140 (25), 656-657 PubMed.
  • Carmichael K P et al (1996) Clinical, hematologic, and biochemical features of a syndrome in Bernese Mountain Dogs characterised by hepatocerebellar degeneration. JAVMA 208 (8), 1277-1279 VetMedResource.
  • Vite C H et al (1996) Atypical disease progression and MR imaging of a Kerry Blue Terrier with cerebellar cortical and extrapyramidal nuclear abiotrophy. Prog Vet Neurol (1), 12-15 VetMedResource.
  • Jackson W et al (1995) Neospora caninum in an adult dog with progressive cerebellar signs. Prog Vet Neurol (4), 124-127 VetMedResource.

Other sources of information

  • Bagley R S, Platt S R (2013) Tremors, involuntary movements and paroxysmal disorders. In: Platt S R & Olby N (eds). BSAVA Manual of Canine and Feline Neurology. BSAVA, p 233-251.
  • De Lahunta A, Glass E (2009) Veterinary Neuroanatomy and Clinical Neurology. 3rd edn. St. Louis, Saunders, Elsevier, p 360-370.

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