Cerebellar Degeneration

[gundogs xoxo]

I have a Golden Retriever puppy from my last litter (now 10 weeks old) that has just been diagnosed with Cerebellar Degeneration.

Her only symptom is a head nod like Perkinsons in humans. It started when she was around 6 weeks old.

Has any one had a puppy with this problem or heard off it? I am unsure of the long term outcome for this puppy and would be interested in any one elses experiences??

[Miranda]

I think it will depend on whether the degeneration is progressive, is it cerebellar dysfunction or cerebellar abiotrophy? I think CA is progressive, but I'm not sure about the other one, I think it may be non-progressive. Your vet would be the best person to ask.

[lappiemum]

From the National Institute of Neourological Disorders and Stroke (NINDS) - US information source http://www.ninds.nih.gov/disorders/cerebel...egeneration.htm

What is Cerebellar Degeneration?

Cerebellar degeneration is a disease process in which neurons in the cerebellum - the area of the brain that controls muscle coordination and balance - deteriorate and die. Diseases that cause cerebellar degeneration can also involve areas of the brain that connect the cerebellum to the spinal cord, such as the medulla oblongata, the cerebral cortex, and the brain stem. Cerebellar degeneration is most often the result of inherited genetic mutations that alter the normal production of specific proteins that are necessary for the survival of neurons.

Associated diseases: Diseases that are specific to the brain, as well as diseases that occur in other parts of the body, can cause neurons to die in the cerebellum. Neurological diseases that feature cerebellar degeneration include:

• acute and hemorrhagic stroke, when there is lack of blood flow or oxygen to the cerebellum
  
• cerebellar cortical atrophy, multisystem atrophy and olivopontocerebellar degeneration, progressive degenerative disorders in which cerebellar degeneration is a key feature
  
• Friedreich’s ataxia, and other spinocerebellar ataxias, which are caused by inherited genetic mutations that progressively kill neurons in the cerebellum, brain stem, and spinal cord
  
• transmissible spongiform encephalopathies (such as "Mad Cow Disease" and Creutzfeldt-Jakob disease) in which abnormal proteins cause inflammation in the brain, particularly in the cerebellum
  
• multiple sclerosis, in which damage to the insulating membrane (myelin) that wraps around and protects nerve cells can involve the cerebellum
  

Other diseases that can cause cerebellar degeneration include:

• endocrine diseases that involve the thyroid or the pituitary gland
  
• chronic alcohol abuse that leads to temporary or permanent cerebellar damage
  
• paraneoplastic disorders in which tumors in other parts of the body produce substances that cause immune system cells to attack neurons in the cerebellum
  

Symptoms: The most characteristic symptom of cerebellar degeneration is a wide-legged, unsteady, lurching walk, usually accompanied by a back and forth tremor in the trunk of the body. Other symptoms include slow, unsteady and jerky movement of the arms or legs, slowed and slurred speech, and nystagmus -- rapid, small movements of the eyes.

What research is being done?

The NINDS funds research to find the genes involved in diseases that cause cerebellar degeneration. Discovering these genes, identifying their mutations, and understanding how the abnormal proteins they produce cause cerebellar degeneration, will eventually help scientists find ways to prevent, treat, and even cure the diseases that involve cerebellar degeneration.

NIH Patient Recruitment for Cerebellar Degeneration Clinical Trials

At NIH Clinical Center

Throughout the U.S. and Worldwide

Organizations

National Ataxia Foundation (NAF)

2600 Fernbrook Lane North

Suite 119

Minneapolis, MN 55447-4752

[email protected]

http://www.ataxia.org

Tel: 763-553-0020

Fax: 763-553-0167

Friedreich's Ataxia Research Alliance (FARA)

P.O. Box 1537

Springfield, VA 22151

[email protected]

http://www.CureFA.org

Tel: (703) 426-1576

Fax: (703) 425-0643

National Multiple Sclerosis Society

733 Third Avenue

3rd Floor

New York, NY 10017-3288

[email protected]

http://www.nationalmssociety.org

Tel: 212-986-3240 800-344-4867 (FIGHTMS)

Fax: 212-986-7981

[lappiemum]

A dog related site on the subject is http://www.upei.ca/~cidd/Diseases/nervous%...0abiotrophy.htm

It lists breeds that are affected by cerebellar abiotrophy, also talks about postnatal cerebellar abiotrophy.

It does have labs down as one of the breeds, but hopefully its not CA as there isn't much you can do for the pup affected. If its CA there may also be consequences for that line, as the paper states that for most CA they suspect an autosomal recessive mode of inheritance.

Edited February 9, 2010 by lappiemum

[Nekhbet]

I've hit a few journals. Some say it is x-linked and hence only male pups will show it (apprently in english pointer) and in labs it is autosomal recessive (both parents are carriers) and hits them from about 6 weeks onwards.

All say that euthanasia is the only option as the dogs waste away or become so confused and uncoordinated.

[gundogs xoxo]

Thanks everyone. I will probably not know for a little whether it is progressive or not, there is not testing to confirm. I have read that it can be caused by the mother coming into contact while pregnant with the Herpies viris. I am just so hoping she gets better not worse!!

[Nekhbet]

I think when it comes to degenerative conditions like this you can only hope for stability more then improvement. It seems to be following the pattern of a genetic degerative condition, is there more pups affected or just this one?

[gundogs xoxo]

It is only this puppy. It is very mild and you really have to sit and watch her to notice it. Her head looks like it is nodding side to side when she sits still, while walking playing she is right.