What is x-linked muscular dystrophy?
This inherited disease is caused by the lack of a single specific protein (dystrophin) which is normally found in muscle fibres. This disease is similar to Duchenne muscular dystrophy in humans. The first sign is usually difficulty in swallowing, which is seen as early as 6-8 weeks of age. The clinical signs will progress from poor suckling to generalized muscle weakness and exercise intolerance to muscle withering (atrophy), curvature of the spine, muscle contractures, and heart problems.
How is x-linked muscular dystrophy inherited?
The trait is sex-linked. This means that it is carried by females (clinically unaffected) whose male offspring will have a 50 per cent chance of developing the disease.
What breeds are affected by x-linked muscular dystrophy?
This disorder is seen in the Belgian shepherd, golden retriever, Irish terrier, miniature schnauzer, samoyed, and rottweiler.
For many breeds and many disorders, the studies to determine the mode of inheritance or the frequency in the breed have not been carried out, or are inconclusive. We have listed breeds for which there is a consensus among those investigating in this field and among veterinary practitioners, that the condition is significant in this breed.
What does x-linked muscular dystrophy mean to your dog & you?
Unfortunately this is a severe, debilitating, progressive disease. It may progress rapidly or more slowly, and your dog will gradually become weaker and more debilitated. There is no specific treatment or cure.
How is x-linked muscular dystrophy diagnosed?
Your veterinarian will suspect this disease if your young pup exhibits a combination of the following clinical signs: generalized muscle weakness and atrophy, exercise intolerance, abnormal gait, excessive drooling, abnormal spinal curvature. This diagnosis can be confirmed with blood tests (to look for elevated muscle enzymes), muscle biopsy, electromyography, and a neurological exam.
How is x-linked muscular dystrophy treated?
There is no cure or specific treatment for this disease. Some of the clinical signs may be treated with drugs, and physiotherapy may also be helpful.
Affected dogs should not be bred. The mother of any affected dog is a carrier of the condition and should not be used for breeding purposes.
FOR MORE INFORMATION ABOUT THIS DISORDER, PLEASE SEE YOUR VETERINARIAN.
Kornegay JN. 1995. Disorders of the skeletal muscles. In EJ Ettinger and EC Feldman (eds) Textbook of Veterinary Internal Medicine, pp.727-736. WB Saunders Co., Toronto.
Ackerman L. 1999. The Genetic Connection: A Guide to Health Problems in Purebred Dogs pp.121-122. AAHA Press,Lakewood, Colorado.
Copyright © 1998 Canine Inherited Disorders Database. All rights reserved.
Revised: April 23, 2002.This database is a joint initiative of the Sir James Dunn Animal Welfare Centre at the Atlantic Veterinary College, University of Prince Edward Island, and the Canadian Veterinary Medical Association.