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 Health Trust HTF Health Series Information Brochures HTF
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Last update - 3/15/05 Content Provider: Jim Orsborn Copyright © 2004 - 2005 STCA |
 Cerebellar Abiotrophy in the Scottish Terrier Why is my Scottie so wobbly?
What is Cerebellar Abiotrophy? Cerebellar Abiotrophy (CA) is a neurological disease recently described in the Scottish Terrier. The term defines progressive, premature cell death in the cerebellum, an area of the brain that controls smooth, coordinated motor skills such as walking. If the cerebellum is damaged or begins to degenerate, fine tuned, precise movement is affected and smooth, voluntary movements become coarse, jerky and uncoordinated. Simply put, a dog affected with CA becomes ataxic, or wobbly. What are the symptoms of CA? Dogs affected with CA will slowly lose their well-defined motor skills and may exhibit subtle incoordination or clumsiness. An owner might observe an unsteady gait, a loose and wobbly rear end or noticeable rolling of the hips. Affected dogs can trip occasionally, bang their chin, fall down, or have trouble negotiating stairs. Eventually, they may begin using the nose to aid in balance and develop a wide-based stance in the rear legs. Irregularities in the front legs can also be present. The symptoms may become severe in one affected dog or remain relatively mild in another. There might be a faster rate of degeneration in one dog or very slow progression of the disease in another, with gradual changes taking place over several years. Can CA be difficult to diagnose? Yes. At this time there is no test to definitively diagnose CA in the living animal. A clinical diagnosis is made based on symptoms, but a final diagnosis can only be made with a post mortem exam. Symptoms of CA can be confused with those of Scottie Cramp, another genetic disorder known to exist in the breed. However, Cramp is an occasional condition, brought on by stress, excitement and/or exercise. Most of the time, a Scottie with Cramp will appear perfectly normal. By contrast, the incoordination associated with CA is always present once its been detected. Patellar luxation (slipping knee caps) can also occur in Scotties, but this is a separate, unrelated condition. Back to TopAre CA symptoms present at birth? No. Affected dogs seem normal at birth. Occasionally symptoms are seen as early as 6 months of age, but many times they aren't noticed until the dog is one year or older. Is CA a fatal disease? No. Affected dogs can live out a full lifespan. The disease affects only balance and motor skills; the mind remains normal throughout the disease process. What is the quality of life one can expect in a dog affected with CA? Affected dogs can have a good quality of life. The disease is very slow to progress and the dog is able to play, enjoy life and participate in the lifestyle his/she is accustomed to for some time. It's been said that the disease is harder on the owner than it is on the dog. One has to keep in mind that CA is not painful. However, some Scotties with advanced CA may require assistance while walking, eating and drinking. Is CA a hereditary disease? Yes. CA is caused by a simple autosomal recessive gene. Affected dogs have 2 copies of the defective gene, one inherited from each parent. Therefore, both parents of an identified affected dog are known carriers of CA. Full siblings may be affected, they may be carriers or they may be clear. Males and females can be affected. Is there any treatment available for CA? No. Unfortunately, there is none available at this time. Some owners have tried vitamins, dietary supplements and acupuncture, hoping to delay the progression of the disease. There is no medical data supporting benefit from any of these treatments, but that is an issue to be discussed with a qualified veterinarian. Back to TopWhat is being done about CA in the Scottie? The STCA HTF is fully involved and is looking into CA in the Scottish Terrier. In 1995, the STCA HTF Breed Survey indicated that the incidence of ataxia in Scotties was a re- ported 13%. It is not possible to know how many of these cases were indeed CA, but the incidence of ataxia was relatively high. In 2001, the STCA HTF retained the services of Dr. Jerold Bell DVM, Assistant Professor of Genetics at Tufts University, to look into this matter. Dr. Bell is currently accepting information about any Scottie with possible neurological disease. He will assist owners by determining a diagnosis, which may or may not turn out to be CA. Once he collects enough data, Dr. Bell's goal is to determine how widespread CA is in the breed and to make recommendations on how to help lower the incidence of this disease. Eventually Dr. Bell, other doctors and research scientists hope to prevent CA from occurring in the future. One goal is to conduct DNA studies, which may one day provide a test for breeders to identify carriers in their lines. Dr Bell needs your help. Dr. Jerold Bell asks that those who have a dog they suspect may have CA contact him. He will review a video and a pedigree (if available) of your Scottie to determine if he or she could be affected and offer a diagnosis. There is no charge for this service. Dr. Bell offers complete confidentiality. Dr. Alexander de Lahunta, a renowned neurologist, is assisting Dr. Bell in this research. He has offered to do necropsies (post mortem exams) on deceased Scotties affected with CA to confirm the diagnosis at no cost. He will also accept and review a video of a Scottie suspected of having CA. Back to TopContacts If you suspect you have a Scottie with CA and are uncertain what the symptoms might look like, a video is available showing multiple Scotties at different stages of the disease. Please make your request for a copy of this video to: Debbie Smith For Diagnostic Information, contact: Dr. Alexander de Lahunta, DVM
Copyright 2004 |
