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Syringohydromyelia in Dogs: Implications for the Cavalier King Charles Spaniel

What is Syringohydromyelia?

Syringohydromyelia (syringomyelia, hydromyelia, syrinx, SM) is a congenital or acquired intraspinal disease. It is a disorder where a cyst or herniation, known as a syrinx, forms within the spinal cord and cause the flow of cerebrospinal fluid (CSF) to become obstructed and accumulate, causing symptoms such as scratching, pain and limb dysfunction. In some cases it is seen in combination with hydrocephalus.

This condition is thought to be similar to the Arnold Chiari Syndrome in humans which may also be accompanied by secondary syringomyelia or hydromyelia. It is considered multi species and has been reported in humans, dogs, cats, horses among others. Nor is it limited to one breed having been reported in breeds such as the Labrador Retriever, Weimaraner, Fox Terrier, Lhasa Apso, Pomeranian, Cavalier King Charles Spaniel.

What are the symptoms?

The most common symptoms of Syringohydromyelia are a hypersensitivity in the neck area resulting in an uncontrolled urge to scratch at the neck and shoulders, quite often to the point that the dog will fall over. There is quite often discomfort and pain in the neck and ear areas. There may also be progressive weakness in the limbs and loss of bladder and bowel control.


In the congenital form symptoms are usually first seen from 6 to 18 months of age but depending upon the degree affected could manifest at any age. In the acquired form symptoms can be seen any time after the original injury or trauma to the spine.

How does a dog get Syringohydromyelia?

Syringohydromyelia can be either acquired or congenital. If acquired, this would occur due to trauma, complications of surgery or disease such as a tumour and the syrinx would develop in the damaged segment of the spine and may expand causing difficulties. If congenital, the condition would develop in the womb or during birth as a malformation and in this case the syrinx is usually in the cervical region of the spine. In the congenital condition, there is some suspicion that it may be a genetic and there are currently studies going on to establish a familial link and hopefully understand the mode of inheritance if genetic. (Please see the section "Implications in Cavalier King Charles Spaniels" for more information)

How is it diagnosed?

Though the condition was recognized symptomatically earlier it has only been due to the advances in advanced imaging techniques through the use of Magnetic Resonance Imaging (MRI) that this disease has been understood and diagnosis been able to be confirmed.

The MRI is the leading diagnostic tool used in determining Syringohydromyelia. It has only been since 1993 that a functional MRI was developed and around 1996 when it was advanced enough and readily available to diagnose spinal conditions in dogs.

MRI images are formed by the combination of a strong magnetic field and radio wave interacting with the hydrogen protons in the body and then using a computer the information is collected and made into three dimensional pictures of the body. The MRI can then characterize and discriminate among the tissues using their physical and biochemical properties (water, fat, iron, blood and its breakdown products) allowing blood flow, cerebrospinal fluid (CSF) flow and contraction and relaxation of organs to be evaluated.

Using the MRI then allows the neurologist to study the spine for the presence of a syrinx or any other abnormality, such as a tumour which might obstruct the flow of the spinal fluid.

What is the treatment for Syringohydromyelia?

There are several different treatments for Syringohydromyelia, some more successful that others. In some less severe cases the use of cortisteroids or nonsteroidal anti-inflammatory drugs may relieve the clinical symptoms. Decompression surgery to allow the CSF to flow normally may be necessary and in some cases a shunt is installed.

Implications in Cavalier King Charles Spaniels

"I have a Cavalier, should I be concerned"?

This particular condition is known to affect less than 0.002% of Cavalier King Charles Spaniels so odds are very much in your favour that your dog would not have this condition. If your dog is scratching or displaying some of the other symptoms more than likely it is due to another cause such as allergies which is a common condition in dogs. If concerned see a veterinarian for evaluation.

Is Syringohydromyelia genetic?

There is a suspicion that Syringohydromyelia is of a genetic nature. Due to the development of the MRI and genetic mapping, diagnosis and study of diseases such as this is now being made possible. The Cavalier King Charles Club (UK) has approached Mr. Simon Platt, Head of Neurology at The Animal Health Trust to undertake research into the possible genetic nature of this condition.

If you have a Cavalier King Charles Spaniel diagnosed with this condition please send pedigree and pertinent medical records relating to the diagnosis to:

Mr.Simon Platt BVM&S MRCVS
Diplomate ACVIM (Neurology) Diplomate ECVN
Head of Neurology/Neurology Service
Centre for Small Health Studies
Kentford
Newmarket
CB8 7UU

This will hopefully help in the confirmation that this condition is of a genetic nature and allow for understanding of the mode of inheritance.

Any speculation as to the genetic nature and possible carriers of this disease is premature and cannot be made by anyone less than trained professionals such as neurologists and geneticists, so by providing records to Mr. Platt at the Animal Health Trust you are helping in the fight to understand and possibly eradicate Syringohydromyelia from Cavalier King Charles Spaniels in the future.
 


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