Purkinje Cell Degeneration

Cerebellar Purkinje cell degeneration is a form of abiotrophy, a spontaneous, premature neuronal death. In most types of abiotrophy, an inherited inborn error of metabolism of unknown origin is considered to be responsible. Most abiotrophies involve the cerebellar Purkinje cells, which appear to be exquisitely susceptible to derangement of their metabolism. Abiotrophies may affect the cerebellum alone or may affect the cerebellum and other areas of the brain. Cerebellar Purkinje cell degeneration is a progressive genetic disease resulting in cerebellum loss of function which includes coordination of voluntary, postural and reflex movements. The cerebellum also assists with the maintenance of equilibrium and movement coordination.

A syndrome of cerebellar Purkinje cell degeneration and coat color dilution was diagnosed in a family of Rhodesian Ridgebacks and Staffordshire Terriers. A slowly progressing late-onset form of cerebellar degeneration develops in Old English Sheepdogs. Epilepsy was reported in several litters of Lagotto Romagnolo dogs which was associated with loss of Purkinje cells. Many affected dogs remain with mild incoordination their entire lives, and live out a normal life span. Other animals progress to the point where they can not walk without assistance.



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Common signs of cerebellar Purkinje cell degeneration include growth retardation and movement incoordination. Animals may stand with a broad-based stance and swaying of the body may be present. Tremors, spastic, high stepping "over-measurement" gait in all four limbs may also be seen. Nystagmus, progressive gait abnormalities, and diluted coat color occur in some breeds, but littermates with non-diluted coat color are neurologically normal. The signs of incoordination in affected Scottish Terriers have been recognized by owners or breeders as early as 10 weeks of age, and as late as 4 years of age. Recognition of clinical signs has more to do with the severity of the disorder than the actual onset of cerebellar degeneration.

References

  1. Chieffo C, Stalis IH, Van Winkle TJ, Haskins ME, Patterson DF. Cerebellar Purkinje's cell degeneration and coat color dilution in a family of Rhodesian Ridgeback dogs.
  2. H. Steven Steinberg, VMD, DACVIM Thomas Van Winkle, VMD, DACVP Jerold S. Bell, DVM Alexander de Lahunta, DVM, PhD, DACVIM. Cerebellar degeneration in Old English Sheepdogs.
  3. T.S. Jokinen, L. Metsa¨honkala, L. Bergamasco, R. Viitmaa, P. Syrja¨ , H. Lohi, M. Snellman, J. Jeserevics, and S. Cizinauskas. Benign Familial Juvenile Epilepsy in Lagotto Romagnolo Dogs
  4. Open Letter from Dr. Bell, DVM. Scottish Terrier Club of America.
  5. Richard A. LeCouteur, BVSc, PhD, Diplomate ACVIM (Neurology), DECVN. Cerebellar Diseases of Dogs and Cats.



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