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Syringomyelia: Symptoms, Treatment, Info

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Karlin

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This is the current SM information sheet (latest version released 3 May 2005) from UK-based vet neurologist Clare Rusbridge, one of the leading researchers into syringomyelia in cavaliers. Information on the cavalier DNA collection project is at the end. Note that they desire blood samples from ANY cavalier, whether it has SM or not, and that the donation benefits research into MVD, epilepsy, and other conditions too. This is posted with Clare Rusbridge's permission.

Support group for people with affected cavaliers or seeking further information:
http://groups.yahoo.com/group/CKCS-SM-support

Syringomyelia

Clare Rusbridge BVMS DipECVN MRCVS


What is Syringomyelia?
Syringomyelia is a condition whereby fluid filled cavities develop within the spinal cord. Some refer to SM as “neck scratcher’s disease” because scratching in the air near the neck is a common sign.

What causes it?
Syringomyelia is a consequence of an obstruction to cerebrospinal fluid (CSF) flow. In the normal mammal, the CSF around the brain shunts back and forth with the arterial pulse. If this rapid efflux and influx is obstructed then the pressure wave is transmitted down the spinal cord distending it immediately below the blockage. This results in the formation of a cavity or syrinx. Syringomyelia can occur from any blockage in the subarachnoid space (space containing CSF around the brain and spinal cord). However, the most common cause is the cerebellum within the foramen magnum (i.e. the back of the brain poking though the hole at the back of the skull). The cerebellum is pushed (herniated) out the skull because there is not enough space since the bone at the back of the skull (occipital bone) is too small. This condition occurs in many small breeds but is common in the cavalier King Charles spaniel (CKCS) (conservative estimates at least 50% of the breed have a degree of occipital hypoplasia although only a proportion are severe enough to have syringomyelia). It is similar to the human condition Chiari malformation (some vets refer to it as Arnold Chiari syndrome which can be confusing as the original description by Arnold was of syringomyelia associated with spina bifida and this is not the case in the CKCS).

What are the clinical signs of syringomyelia?
By far the most important sign of syringomyelia is pain. This is most commonly localised to the neck region but may be difficult to define or intermittent. Owners often report that their dog is worse at night; when first getting up; during hot or cold temperature extremes; when excited; or related to posture e.g. preferring to sleep with their head elevated. They may seem to be overly sensitive to touch on one side of the neck / ear / shoulder / sternum. In addition some affected dogs scratch at one area of the shoulder, ear, neck or sternum. This is typically one side only, while the dog is moving and sometimes without making skin contact
Some dogs, more commonly younger patients, develop a scoliosis (twisted spine). Some severe cases may have other neurological deficits such as fore and hindlimb limb weakness and ataxia (wobbliness). Facial nerve paralysis and deafness have also been associated with the condition.

What age of dog is affected?
Clinical signs of syringomyelia secondary to occipital hypoplasia are usually recognized between 6 months and 3 years of age. However, dogs of any age may be presented and dogs with more severe disease tend to be presented before two years of age.

Do the signs get worse?
Progression of the disease is very variable. Some dogs have the tendency to scratch with mild pain only and other neurological signs, such as paresis, never or very slowly develop. Others can be severely disabled by pain and neurological deficits within 12 months of the first signs developing. Mild syringomyelia may also be found as an incidental finding, with no recognised clinical signs, in the investigation of another neurological disease.

Are there any diseases with similar signs to syringomyelia?
The main diseases to rule out are other causes of neck pain e.g. disc disease (uncommon in dogs less than two years of age); CNS inflammatory diseases and other malformations. If scratching or face rubbing is the main sign then skin disease should be eliminated.

How do I know if my dog has Syringomyelia?
The only way to confirm a diagnosis is by MRI (Magnetic Resonance imaging). This is essentially a picture of the water content of the body presented in a series of slices (like a loaf of bread). Nervous tissue, which contains a lot of water, is not imaged by x-rays but is shown in great detail by MRI. The syringomyelia can be easily visualised as a pocket of fluid within the spinal cord. In severe cases the syrinx is so wide that only a thin rim of spinal cord remains.

If my dog has been diagnosed with Syringomyelia what are the options?
No one can make the decision for you about what is best for your dog.

Medical management
Long-term studies of medical management of syringomyelia are not available yet. The drugs used to treat syringomyelia can be divided into 3 types:
• analgesics;
• drugs which reduce CSF production;
• corticosteroids.

Analgesics
Pain in mild cases may be controlled by non steroidal anti-inflammatory drugs (NSAIDs) e.g. Rimadyl and Metacam. In more severe cases anticonvulsants, which have a neuromodulatory effect on hyperexcitable damaged nervous system, may be useful, for example gabapentin (Neurontin Pfizer; dose rate 10-20mg/kg BID/TID – these are not licenced for dogs). Oral opioids, e.g. pethidine or methadone are also an alternative.

Drugs which reduce CSF production
Proton pump inhibitors such as omeprazole (Losec or Prilosec) can inhibit cerebrospinal fluid formation and therefore may be valuable; clinical data on their use and effectiveness for SM is currently lacking. This drug is unlikely to be useful in the long term as therapy longer than 8 weeks duration is not recommended as this may increase the risk for stomach cancer. Carbonic anhydrase inhibitors such as acetazolamide (Diamox; Lederle laboratories) also decrease CSF flow and may also be helpful in treating syringomyelia although adverse effects of abdominal pain, lethargy and weakness may limit long term use. Furosemide also decreases intracranial pressure and therefore could be useful in the treatment of syringomyelia. The mechanism of action is unknown and may just be due to diuresis and reduction in blood volume
At present the usefulness of omeprazole, acetazolamide and furosemide for treating syringomyelia is unknown.

Corticosteroids
Corticosteroids are anecdotally very effective in reducing both pain and neurological deficits although the exact mechanism is not known. It has been suggested that these drugs reduce CSF pressure however laboratory evidence of this is lacking. They possibly have a direct effect on pain mediators such as substance P. Although corticosteroids may be effective in limiting the signs and progression, most dogs require continuous therapy and subsequently develop the concomitant side effects of immunosuppression, weight gain and skin changes. If there is no alternative then the lowest possible dose that can control signs is used. Alternate day therapy is preferred. The author starts with 0.5mg/kg prednisolone / methylprednisolone daily.

Surgical management
Surgical management is indicated for dogs with significant pain or with worsening neurological signs. The aim is to restore CSF dynamics and if this can be achieved then the syrinx can resolve. The most common procedure for Chiari like malformation is suboccipital decompression where the hypoplastic occipital bone and sometimes the cranial dorsal laminae of the atlas are removed (with or without a durotomy) to decompress the foramen magnum. The success reported in the small case series varies from no improvement to post operative resolution of the syrinx. Syringo-subarachnoid shunting has also been described. In the author’s experience surgery is usually successful at significantly reducing the pain but some dogs may still show signs of discomfort /scratching. Also in the author’s experience signs may recur in a proportion of dogs after several months/years due to redevelopment of syringomyelia.
One must weigh the risks and benefits of surgery versus medication versus no intervention. Remember, progressive disease means that no action may enable further deterioration.

When to have surgery?
There is more chance of success if the surgery is done early in the course of the disease before permanent damage has occurred. Surgical management is indicated for dogs with significant pain or with worsening neurological signs

What are the risks of surgery?
There are major blood vessels in the area and if traumatised the dog could quickly bleed to death. Although not actually operating on the brain/spinal cord, it is in close proximity and there is a risk of permanent neurological injury. In reality complications from surgery seem to be rare.

Can the disease recur?
In the authors’ experience signs may recur in a proportion of dogs after several months/years due to redevelopment of syringomyelia. The newly created “space” from surgery may fill in with scar tissue. If this happens, repeat surgery may be indicated; some owner prefer to continue with medical management e.g. with NSAIDs, gabapentin or corticosteroids.

What post surgery drug treatment would you advise?
Dogs are hospitalised until comfortable enough for morphine-like-drugs to be discontinued and then discharged on a combination of non steroidal anti-inflammatory drugs (e.g. Rimadyl) and gabapentin (Neurontin). This is withdrawn when the dog is comfortable (about 2 weeks in most cases).

DNA collection programme

Our aim is to provide a comprehensive, integrated collection of cavalier King Charles spaniel DNA for the benefit of the dogs, owners, breeders and to provide insight into human disease. Surplus blood from a health check would be stored for future studies on the health of the breed. The current studies include syringomyelia (SM), mitral valve disease (MVD) and Epilepsy.

Questions & Answers

Why is blood needed?
It is easy to extract DNA from the white blood cells in a blood sample. To do this the blood must be fresh and prevented from clotting by putting it in an EDTA tube.

What will happen to the blood sample from my dog?
The DNA sample being submitted to the researchers will be anonymous once it is entered into the archive and will be kept strictly confidential. The samples and clinical data will be made to available to bona fide research groups working on these conditions and where the projects have been deemed to be ethically sound. The owner will also retain the right to remove the sample from the archive in the future if so wished. However, no information regarding tests performed on the DNA sample will be given back to the owner. It will only be possible to find out which genes and environmental factors are important by identifying patterns in large numbers of affected and unaffected animals.

What kinds of dogs are needed to give blood?
All blood from your cavaliers will be valued. The purpose of the study is to identify a gene through DNA analysis. We are therefore focusing on certain areas to be most successful in achieving our goal. We need dogs that are

• Normal healthy, especially if over 7years or MRI confirmed normal (no SM)
• Champions that often appear in pedigrees (any age)
• SM Affected –MRI confirmed or showing typical clinical signs
• Parents and siblings of affected dogs
• Offspring of affected dogs – If <3 years of age the blood may be stored in case signs develop later.
• Mates of an affected dog – is helpful if DNA from offspring is collected later
• MVD affected and their relatives (see SM above)

Remember
Your blood donation will help keep Cavaliers healthy from inherited diseases.

SM/MVD carriers can have good genes/characteristics that we need to conserve.

The more help we get the speedier will be the result.

Why do I need to provide a pedigree?
Pedigree information about your cavalier is important for our study. The relationship between affected and non affected family members can indicate the way in which a disease can be inherited. Comparisons are made between the parental genotypes and those of the offspring. Pedigree analysis is not sufficient in itself to determine if a trait is inherited as a threshold trait. There are many investigations to be made and that is why you are asked for as much blood (DNA) as possible. Linkage may be used, which means DNA from animals that link up affected individuals would be needed.

Bottom line: It is essential that we have DNA from related dogs regardless of whether their status is known.



If you want to donate cavalier blood, you can do so from the UK/Europe or the US/Canada.

For forms and info on donating from Europe, click the 'health' and 'syringomyelia' sections here: http://www.thecavalierclub.co.uk/start.html

For the US/Canada:

DNA may be in the form of EDTA blood (as much as possible ideally 5-10mls) or a DNA swab. Blood is preferred as more DNA can be extracted with this method.

USA forms: http://www.cavalierhealthfoundation.com/North American SM Collection Instructions Forms.pdf

DNA swab kits are available either through the American Kennel Club http://www.akc.org/store/detail/index.cfm or from the International Canine Semen Bank http://www.ik9sb.com/index.htm.

It is essential that pedigree information and the phenotype form are filled in for each dog sampled, otherwise the valuable DNA cannot be used.

The DNA sample and the phenotype forms should be sent to
Berge A Minassian MD, CM FRCP(C)
Room 6536B
Hospital for Sick Children
555 University Ave. Toronto, Ontario
M5G 1X8 Canada
Tel: 416-813-6291
Fax: 416-813-6334
email: [email protected]

Please post /fax or email duplicate phenotype forms with pedigree information to
Clare Rusbridge
Stone Lion Veterinary Centre
41 High Street,
Wimbledon,
SW19 5AU, UK.
Confidential fax line 00 44 208 7860525
email: [email protected]
 
Seminar on syringomyelia by UK neurologist Dr Skerritt

Crossposted from the Arnold Chiari list (on syringomyelia, located here: http://uk.groups.yahoo.com/group/ArnoldChiari_dogs/).

Barbara Reese took these notes from a seminar in Wales in early September 2004 by Dr Skerritt, one of the main UK researchers/vets working on SM in cavaliers. There is further SM information and links in the health concerns FAQ in this section.


Unfortunately Mr Skerritt does not have a transcript as his talk was
very much off the cuff with a lot of slides. I did take some notes
and will try to give the main points for you. His views on this do
differ from some of the other experts.

1. He believes that SM does have a genetic basis and said that the
incidence in cavaliers is a lot higher than the other breeds that
it appears in.

2. It is the malformation of the occipital bone that is hereditary,
the syrinx (which is syringomyelia ) is the by product of this. He
believes the malformation is congenital (present at birth).

3. There is a high incidence of hearing impairment connected to SM,
which they have found to be caused by fluid in the middle ear. This
can be drained and hearing improves.

4. Clincal signs eg scratching are due to the changes in levels of
CFS (fluid in spine) pressure. Most of the dogs they have seen have
been around 18 months of age but they have had symptoms starting as
late as 9 or 10 years. He said lifestyle can account for this. eg a
more sedentary or less excitable dog will have fewer pressure
changes and therefore show fewer clinical signs.

5. He grades them into 3.
Grade 1 ( very mildly affected) can be treated medically.
Grade 2 May need surgery eventually if medication stops working.
Grade 3 Surgery is only option.

6. His preferred method of sugery is the Shunt. He dislikes the
decompression surgery because there is a very high risk of nerve
damage and extreme blood loss. It can also cause the cerebellum to
herniate even more.

7. He does not advocate long term use of steroids because there are
too many side effects and prefers to use Frusamide ( a diretic) to
drain the fluid. He has had very good results from this and though
they monitor the blood potassium levels , because of the very low
dose used they have never had a dog show any ill effects due to
continued use of frusamide.

8. After shunting, they have found that scratching lessens within 3
to 6 weeks. Ataxia of limbs improves within 2 weeks, progression is
halted and quality of life improves within days.

9. Over a 3 year period he diagnosed 235 cases but these figures
have not been updated for 2 years and he now sees at least two
cavaliers per week that are diagnosed with SM.

10 He has MRI'd cavaliers brought in for other ailments ( not with
SM symptoms) and has found them to have NORMAL skulls.

11. He stated that we should be scanning as many cavaliers as
possible, especially young ones. Reccomended 6 months of age and
said that if the malformation was not present then it would not
develop later and the pup could be pronounced as having a normal
skull.

12. It is imperative that MRI scans are done on the same type of
machine with as high a resonance as possible for results to be
accurate.

13. He feels that as well as removing affected dogs from breeding
programmes, breeders should also be withdrawing identified carriers
and possibly their siblings too.

14. The MRI Scheme will be set up with two colleagues. One at
Cambridge University, one at Castle Donnington and his own Chestergates Hospital in Chester. The idea is to conduct a survey to find out how many have the malformation and how many don't. They are planning to do a 10 minute scan of the head and neck region , under sedation rather than a general anaestic. (sorry can't spell it !!) All have the same type of machines for accuracy.

This will show the malformation and any herniation present.


NOTE:
This scheme is now up and running in the UK. One dog is £175; five or more are £150 each at time of posting.
 
NJ syringomyelia talk by Tufts Univ. researcher (Oct 04)

Ellen Crimmel kindly has given permission for me to crosspost her clear and concise summary of the syringomyelia presentation given by Tufts vet neurologist Dr Jay McDonnell at the Cherry Hill cavalier show in Autumn 2004.

One additional note to the seminar was offered on the Arnold Chiari SM discussion list by Laura Lang, who added that Dr McDonnell said many dogs do not have the scratching symptoms at all but show others, which might lead people to think their dog can't have SM. He said only 30-40% of affected dogs he sees present with scratching.

You can view an excellent video of Chester, with Ellen explaining his symptoms, here:

http://www.youtube.com/watch?v=xWmbF3j8zbU



Hi, all, here's a report from the SM seminar held at the Cherry Hill
show this past weekend.

The Syringomyelia seminar at the CNE/COS show in Cherry Hill, NJ
this past weekend was extremely informative. Dr. Jay McDonnell,
Veterinary Neurologist, who teaches at Tufts and is also in private
practice in Bowie, Maryland, spoke for a good 3 ½ hours. Most folks
on this list already know most of this information, but I thought
I'd pass along what I learned. To summarize Dr. McDonnell's
presentation:

SM is a disorder caused by a congenital malformation of the
occipital bone of the skull. In essence, the skull is too small for
the brain. This causes the higher pressure in the brain, which
causes the cerebrospinal fluid (CSF) flow to be "backed up" and not
flow well. This causes hydrocephalus which is enlargement and
dilation of the ventricles of the brain. It also causes the
cerebellum to be pushed out the foramen magnum (the hole in the
skull through which the spinal cord comes off the brain) which is
referred to as herniation of the cerebellum. Eventually, the
pressure buildup causes fluid filled pockets called syrinxes to form
within the spinal cord. This damages the nerves of the spinal cord
which causes the symptoms. There is strong evidence that it is an
inherited condition, and males and females are equally affected.

The typical symptoms are: scratching at the neck, neck pain, head
tilt and head turn, scoliosis, weakness and incoordination of the
limbs, seizures, changes in peripheral vision, and sometimes facial
palsy. Dr. McDonnell's typical patient is young (about 2 years on
average), but the symptoms can start at any age. It is usually only
seen in small/toy breeds.

The diagnosis can strongly suspected on a clinical basis, meaning
based on the presence of typical symptoms. The "gold standard" of
diagnosis is MRI. Dr. McDonnell says he typically sees these 4
signs on MRI: the hydrocephalus or dilation of the ventricles, the
herniation of the cerebellum, the syrinxes in the spinal cord, and
a "kink" in the cervical spinal cord. He has done a preliminary
study on the use of Ultrasound to try to diagnose SM. In a limited
study of 16 dogs, 8 dogs with SM on MRI, and 8 dogs with no SM
symptoms and normal MRI, he was able to see the hydrocephalus,
cerebellar herniation, and the "kink" in the cervical spinal cord.
Ultrasound is not, however, able to see the syrinxes in the spinal
cord because ultrasound can't see through bone. Dr. McDonnell is
hopeful that further studies will prove ultrasound to be a useful
tool in the diagnosis of SM, but says that is more than likely a few
years away.

Treatment usually starts with medication, either steroids
(prednisone), or non-steroidal anti-inflammatory meds such as
Rimadyl, aspirin, or Etogesic. Dr. McDonnell told us that most dogs
will respond initially to medications, but the effect tends to wear
off over time, and the symptoms become uncontrollable on medication
alone in many dogs. He does not put in shunts, as he says they do
not work. He advocates surgical correction. The surgery
is "foramen magnum decompression and dorsal laminectomy". This
consists of cutting away bone to make the opening of the foramen
magnum larger, and cutting away bone from the first vertebra to also
take pressure off the spinal cord. The result of the surgery is
that flow of CSF is restored to normal/near normal. The goal of
surgery is to arrest progression of the disease and minimize or
eliminate pain medications. Dr. McDonnell says the dogs that have
the best outcomes are younger dogs whose symptoms have not been
present for too long. These dogs can have actual reversal of
symptoms and can return to normal. For dogs that have had symptoms
a long time, the best that can be hoped from the surgery is to keep
things from getting worse. The decision whether or not to proceed
with surgery is dependent on the severity of the symptoms, age at
presentation, and whether the symptoms are progressing over time.
Dr. McDonnell has performed this surgery on 20-25 Cavaliers. He
says the dogs usually go home 2-3 days after surgery and are kept on
steroids for a few months, and gradually tapered off.

Dr. McDonnell showed a short video of a 2 year old affected dog,
scratching while walking and with a head tilt, and the surgery
performed on that dog. He told us her symptoms completely resolved
after the surgery. He then graciously allowed me to show a video of
Chester and explain his symptoms to the audience. He then did a
brief exam of Chester. He concurred that Chester most likely DOES
have SM, despite not having an MRI. Given the (albeit very small)
risk associated with MRI, not to mention the cost, Dr. McDonnell
made the point that the decision whether to have an MRI done should
be based on "what are you going to do with the information?" If it
will make a difference in treatment, and if the dog is healthy
enough to have the general anesthesia needed for an MRI, then an MRI
should be done to confirm the diagnosis. He agreed that since
Chester's symptoms seem to be fairly well controlled and have not
really progressed much, that the decision not to MRI him was a valid
one. He told us he has other dogs similar to Chester in his
practice.

As to how to reduce the incidence of this in the breed, he spoke
about the need for incorporating genotyping into breeding
practices. He did say that it appears that the practice of trying
to breed away from MVD has increased the incidence of SM in
Cavaliers, most likely by further limiting the gene pool. He spoke
about the difference between genotype and phenotype, and the fact
that just eliminating affected dogs will not eliminate the disease.

Chester was glad to have been able to demonstrate for all the
participants and was really happy to have met everyone. Hope you
all find this information helpful.

Best regards,
Ellen Crimmel
www.crimcav.com
 
Syringomyelia Symptoms

Syringomyelia Symptoms
(Compiled by Karlin Lillington; with permission to link, crosspost, and reproduce)

Background information: Explanation of SM and current treatments:
http://www.cavaliertalk.com/phpBB2/viewtopic.php?t=22
Support group: http://groups.yahoo.com/group/CKCS-SM-support

This document is an attempt to gather together symptoms shown by syringomyelia-affected Cavalier King Charles Spaniels. Cavaliers unfortunately are affected by SM in disproportionately larger numbers to any other breed. The primary symptoms (usually at least one of these is present) are described as:

• excessive scratching (especially while on the lead, and often 'air scratching' where the dog scratches in mid-air, which may cause the dog to hop while walking)
• ongoing tenderness around the neck, head, shoulders, or hind limbs; or weakness and/or pain in limbs
• yelping as if in pain, but for no apparent reason

Understandably, such descriptions can be confusing – how much scratching is 'excessive', for example? Some people might turn to their vet with such questions, but many have found their vets either were unfamiliar or only vaguely familiar with syringomyelia. This document, an attempt to help clarify how affected dogs may act, is a compilation of descriptions of a whole range of symptoms from owners of syringo-affected cavaliers, offered by neurologists and from the "Arnold Chiari dogs" discussion list. While it might be tempting to dismiss particular symptoms because they may originate in a normal dog behaviour, true SM symptoms are distinctive and become excessive, with most affected dogs showing odd behaviour in conjunction with any of the three points above -- *with a vet unable to find any other identifiable cause for any of the behaviours*.

At this point an owner should consult with a neurologist, ideally one familiar with SM. If syringomyelia is suspected the only way to make a definite diagnosis is by an MRI of the head and neck regions. However, symptoms may be so distinct that the condition can be diagnosed from symptoms alone. At this point the dog may be treated with medication, homeopathy, or shunt or decompression (skull) surgery. Decompression surgery, while the most serious and invasive alternative, usually halts the progression of the condition but does not guarantee it will not return. However most dogs seem to significantly improve or at the very least, stabilise after surgery. About 25% of dogs develop scar tissue after surgery which can cause cerebro-spinal fluid (CSF) pressure to build again and SM to recur. Some neurosurgeons are experimenting with inserting a titanium mesh to reduce or prevent scarring, a technique used successfully in human surgeries. Information on the success rate of various treatments is at present only anecdotal.

Below, general symptoms are listed, and then a range of ways in which each has been expressed in different dogs. Keep in mind that many of these symptoms are also typical dog behaviour, so owners should look out for a range of symptoms or a gradual worsening of a single or a handful of symptoms or *excessive* behaviour. Symptoms typically show between 6 months and three years, but may appear at any time including in puppies as young as 8 weeks old. Dogs exhibiting symptoms before age 2 tend to be more severely affected. Dogs may go through good periods when the symptoms subside and bad periods when they are severe or return. Many of the symptoms typically occur when the dog is on the lead or excited. Note that although scratching and pain are considered key symptoms, some dogs never show one or the other, but may show some of the other symptoms. Fewer than 50% of dogs ever scratch as a symptom so SM should not be ruled out on the basis that the dog isn't scratching.

Scratching

Dogs display frequent to almost continuous scratching at head, shoulders or ears, often on one side only. This can progress until the dog is scratching itself raw or scratching and yelping in pain, or scratching almost manically while lying on the floor. Scratching can happen in long 'episodes' of several minutes at a time. Sometimes scratching includes biting at areas, sometimes until those areas are raw. Sometimes touching the dog's ears brings on scratching.

Air scratching, a circular scratching motion with the leg, where the dog never actually scratches itself, is another common symptom – often occurring while on the lead. Often it becomes more frequent over time until walks become difficult. This can lead to a 'bunny hop' gait as the dog tries to scratch the air with one leg and walk.

If you suspect your dog may have SM, get a harness for the dog for walks as soon as possible, as this significantly relieves the pressure at the neck that makes dogs scratch in discomfort during and right after walks. The neck is often sensitive because this is where the brain protrudes into the spinal column of affected dogs and is also where syrinxes (fluid pockets) form in the spine due to CSF pressure.

General expression of pain

Often syringomyelia is first noticed because a dog begins yelping or whining or whimpering, even shrieking or screaming, seemingly for no reason. Again, this often occurs while on the lead. Pain episodes can disappear then return even after a year or more. Some episodes can be severe with the dog shivering with pain and crying out for long periods.

Often the dog is so uncomfortable and tender that it cannot abide being touched in areas such as the head, neck or shoulders or even on an entire side. Sometimes the pain is so severe that the dog can not be picked up or held. Eyes may look pained. Dogs may pant with pain.

In some dogs weather changes such as storms or a cold front seem to bring on pain episodes (some neurologists believe this may be due to shifts in atmospheric pressure which affect the CSF fluid pressure). When in pain, some dogs go seclude themselves under tables, chairs or beds. Some seem to have episodes mostly at night. Pain can occasionally make the dog irritable with other dogs or with people.

Seeking cool areas or restlessness

Often an affected dog will shift constantly rather than sleep comfortably, and go in search of cold places such as tile or cement floors or even out in the rain, which seems to bring some relief.

Weakness in Limbs

Some dogs acquire a "rolling gait" that can worsen. They may show a lack of coordination. They may collapse easily, even falling over while standing. Sometimes brushing, grooming or bathing bring on collapsing episodes. They may fall over, usually to one side, when playing (though investigate Episodic Falling Syndrome if this happens frequently).

Dogs can start to have difficulty getting up and down stairs and couches and beds. They can have leg twitching episodes. They might not be able to balance well when set down. A paw or leg might go weak.

Feet Licking

Some dogs will lick at their paws or legs obsessively, often until raw.

Tiredness and lethargy

Some dogs become very sleepy and rest much of the day and night, often with their head elevated, which seems to be more comfortable for them.

Fly-catching, head shaking, lip-licking

Fly catching is a neurological condition in which the dog snaps at the air, as if snapping at flies, and has been reported in many syringomyelia dogs though it occurs as a separate condition on its own, as well. Dogs often will shake their heads and ears, yawn excessively (probably an attempt to clear pressure they feel in their heads), or lick at their lips excessively.

Eating and Drinking

Many dogs become uncomfortable eating with dishes placed at a low level and raising them seems to significantly help relieve discomfort. Some dogs start to choke on food or refuse food.

Head rubbing

Some dogs start to rub their head from side to side on the floor as if their heads hurt, doing this excessively (NB: normal dogs will do this with pleasure, often before rolling on the floor). They sometimes 'mush' their face against the floor. In severe cases dogs have rubbed their faces raw on the floor.

Digging or pushing

Some dogs begin to dig obsessively at carpets or sofas especially after they have experienced an episode of pain. They may run along the length of a sofa pushing themselves against it. Again, this behaviour is normal in many dogs; with SM dogs, the activity is frantic and an expression of pain.


Walking in a circle

Some dogs start to walk in circular patterns.

Nerve damage, stiffness, seizures

This can affect a dog in many ways, from loss of feeling, hearing, or muscular movement. Some dogs have neurological problems with their eyes. Nerve damage seems to be progressive with this condition though some dogs have little or no visible damage and others have severe damage. The surgery seems to halt the progression of such damage but will not reverse existing damage.

Some dogs develop a stiffness in the neck, back and/or limbs. In severe cases the neck may stiffen and bend permanently to the right or left ('neck scoliosis'), or the whole body may bend into a 'C' shape when the dog runs. The head may tilt permanently to one side or the other. The dog may have head tremors. Some dogs have begin to have seizures, in some cases, several a day and often very severe.


Keep in mind:

Many symptoms listed have nothing to do with syringomyelia so it is important for a vet to eliminate other possibilities first, including PSOM (primary secretory otitis media), or 'glue ear', which causes similar symptoms and is frequently seen in cavaliers. Allergies to many things, including diet, can also cause dogs to rub their heads on the floor. Ear infections, ear mites, skin conditions or skin irritants like mites or fleas can cause a dog to scratch obsessively or scratch or shake the head and ears. Some dogs are also yelpers, especially when excited. It's a good idea to eliminate more common possibilities first before exploring whether a dog has syringomyelia. However, early treatment – especially if opting for surgery – is also important. Do not postpone taking a dog in to see a good vet – ideally, one familiar with this condition -- if its actions seem to point towards syringomyelia. Waiting even a matter of weeks may result in permanent neurological damage as health can decline swiftly in severely affected dogs.

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** More information on PSOM:

Primary secretory otitis media in the Cavalier King Charles spaniel: a review of 61 cases
Authors: Stern-Bertholtz W.; Sjöström L.; Wallin Håkanson N.
Source: The Journal of Small Animal Practice, Volume 44, Number 6, 30 June 2003, pp. 253-256(4)
Publisher: BVA Publications

Abstract:
Sixty-one episodes of primary secretory otitis media (PSOM) were diagnosed in 43 Cavalier King Charles spaniels over a 10-year period. The principal findings were signs of moderate to severe pain localised to the head or cervical area, and/or neurological signs. Diagnosis was made by examination of the tympanic membrane and middle ear with the aid of an operating microscope under general anaesthesia. A bulging, but intact, tympanic membrane was found in most cases. Following myringotomy, a highly viscous mucus plug was found filling the middle ear. Treatment, consisting of removal of the mucus plug, flushing of the middle ear, and local and systemic medical therapy, had to be repeated between one and five times. The prognosis was good in all cases. PSOM is an important differential diagnosis in Cavalier King Charles spaniels with signs of pain involving the head and neck, and/or neurological signs.

The full article is available for purchase online here at $12.95: http://www.ingentaconnect.com/content/bva/jsap/2003/00000044/00000006/art00001
 
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