CNS Degenerative Diseases - Pathology

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Equine Laryngeal Hemiplegia

Lysosomal Storage Disease

(LSD)

  • Occurs in many breeds of dogs and cats.
  • Genetic defects give deficiencies of specific enzymes of degradation.
    • Disease only occurs in animals homozygous at the gene pair controlling enzyme activity.
      • The genes are co-dominant.
    • Enzyme activity is extremely low or entirely absent.
  • Large amounts of waste products accumulate within cells.
    • Nervous system cells are predisposed.
    • Cells become engorged.
    • Normal function is prevented.

Clinical Signs

  • Nervous system cells are predisposed to the disease, and so progressive neurological signs predominate.
    • Develop between 2 weeks and 12 months of age.
    • The disease is ultimately fatal.
  • For example, Siamese cats show:
    • Ganglisidosis
    • Mucopolysaccharidosis

Diagnosis

  • Clinical signs are considered.
  • Urine enzyme assays may be helpful.
  • Post-mortem diagnosis is possible.
  • More recently, gene tests have been available.


Cognitive Dysfunction Syndrome

(CDS)

  • CDS causes signs of senility.
  • Increasing numbers of dogs and cats are suffering from CDS.
    • Prevalence is similar to that seen in humans: an 85 year-old human is roughly equivalent to a 15 year-old cat, and a 13-14 year-old dog.
      • Aging in dogs id breed specific.

Pathology

  • A number of changes are seen in the brains of geriatric animals which may cause the signs of CDS.
    • Vascular insufficiency leads to:
      • Hypoxia
      • Increased free redical production
      • Deposition of amyloid plaques
      • Modification of other brain proteins

Treatment

  • There are no published studies related to treatment of CDS in animals.
  • Potential therapies include:
    • Dietary modification
      • Diets are enriched with antioxidants, vitamin E, beta-carotene and essential fatty acids.
    • Environmental stimulation
    • Drugs
      • Selegiline
      • Propentofylline
      • Nicergoline