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X-linked Tremor, Congenital Tremor

Congenital problems associated with tremoring include hypomyelination, dysmyelination, and lysosomal storage disease.

Congenital tremors due to defects of the protein myelin covering parts of nerve cells (axons) which are responsible for transmitting impulses throughout the body. Axons are either naked or surrounded by a disproportionately thin layer of myelin.This group of disorders have been reported in many breeds of dogs. The condition is known to affect the Chow Chow,, Weimaraner, Bernese Mountain dog, Samoyed, Spinger spaniel, and Dalmatian. The defect is confined to the central nervous system and peripheral nerves are unaffected.

Clinical signs appear within the first several weeks of age, however, cases of delayed onset also have been reported. In the recessive hypomyelination disorder of Spinger spaniel dogs ("shaking pups"), a severe generalized tremor is first seen in the second week of life in affected male dogs. These dogs are much reduced in weight an size, unable to stand or walk, and do not improve over time. Female carriers of the defective gene may have a milder form of the disease which resolves by 4-6 week of life. Dogs with this disorder may develop seizures as they mature. Affected dogs will often have resting tremor which worsens with exercise and excitement.

The Samoyed breed is affected with a hypomyelination disorder very similar to that of Spinger spaniel dogs. Other breeds with this disorder seem to have less severe clinical signs than a href="../samoyed/samoyed.aspx">Samoyed and Spinger spaniel dogs. In these breeds, the disease is suspected to be inherited as an autosomal recessive trait.

There is no treatment for this disease and in male Springer spaniels, there is no improvement over time. Chow Chows and Weimaraners have an unpredicatble prognosis and some may recover completely.

Adapted from:
1. A Practical Guide to Canine and Feline Neurology By Curtis W. Dewey, Anton G. Hoffman, Carol Rudowsky
2. Animal Models of Neurological Disease: Neurodegenerative Diseases. Alan A. Boulton, Glen B. Baker, Roger F. Butterworth
3. Shaking pups: a disorder of central myelination in the Spaniel dog. Part 1. Griffiths IR, Duncan ID, McCulloch M, Harvey MJ. Clinical, genetic and light-microscopical observations. In: J Neurol Sci. 1981 Jun;50(3):423-33
4. Shaker Syndrome in a Cat. Chris Gordon. (Advisor: Dr. Scott Schatzberg; ecommons.library.cornell.edu)

 

Go Pets America recommends seeking the advice of your local veterinarian for the most appropriate vaccination program and for the diagnosis and treatment of your pet's health problems. For vaccination requirements please contact your state and local licensing authorities.

 


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