Vous trouverez ici la version française de cet article.
Published in The
"Dépêche vétérinaire", French weekly for vets,
n° 804 from march, 13th to march, 19th 2004 (if you want to get part
or integrality of this article, please ask its author D. Cauzinille or the "Dépêche
In the front page, column "Dépêchez-vous de lire..."(hurry to read), we find :
Cavaliers King Charles Spaniel: fight against syringomyelia
Syringomyelia seems affect preferentially cavalier king
charles spaniels. Fighting against this hereditary disease will get also
through a best information of vets,
Going on leafing through this paper, pages 18 & 20, we can read :
Continuous formation :
By Laurent CAUZINILLE*
Clinique vétérinaire Frégis
|*||ancien interne de
chirurgie de Maisons-Alfort;
ancien résident de neurologie et neurochirurgie de North Carolina State University;
American college of Veretinary Internal Medicin (Neurology) Diplomate;
European College of Veterinary Neurology Diplomate;
président du Groupe d'étude en neurologie de
l'Association française des vétérinaires pour animaux de compagnie.
Well known and studied in Great Britain, syringomyelia seems affect preferentially cavalier king charles spaniels and express itself by different clinical troubles. Fighting against this hereditary disease will get also through a best information of vets, breeders and through a quick reaction of the breed club.
The breed of the Cavalier King Charles Spaniel (CKCS) is known for its hereditary cardiac and ocular problems. A genetic predisposition to develop a sort of malformation known under the term of "syringomyelia" is described in United Kingdom for many years. This disease is also met in France but its incidence is not determined.
Because of a flattened and a weak opening of the occipital area that contains the cerebral trunk, a cavity (called syrinx) would develop within the cervical spinal chord (picture 1). This cavity is sometimes in relation with the central canal (hydromyelia). Others associated anomalies are described : bad closing of the spinal chord dysraphism or abnormal dilatation of cerebral ventricles hydrocephaly. The syringomyelia is also described in others breeds (French bulldog, Weimar pointer, German mastiff).
Picture 1. Macroscopic view in transverse cross of the spinal chord of a 5-years-old CKCS, shown for neck pain and weak robustness to effort. Note the extreme dilatation of the central canal and the scarcity of spinal cord tissue.
Picture2. 5-months-old CKCS, shown for stiff neck and neck pain since its firsts months.
The clinical expression of these anomalies takes on very different presentations : neck and shoulder itching (well known in humans affected by the same syndrome), stiff neck, motor troubles of the front limb, or even paralysis and social delay in the case of severe hydrocephaly (Picture2). There is no direct correlation between the importance of the anomaly and the importance of the clinical signs, some affected dogs appear totally normal. It is described, in veterinary medicine like in human one, a puppy form (severe hydrocephaly), a young form between 6 months and half and 1 year old (stiff neck, limb weakness) and an adult form (itching, cry or locomotive difficulties.
Syringomyelia shows clinical signs comparable to a back instability, slipped disc, meningomyelitis or also tumoral process of back, spinal cord or from a nerve root cervical. So complementary exams are necessary. It is not possible to diagnose a syringomyelia on a CKCS with cervical problems doing a simple auscultation!
Among the diagnosis way, the myelography shows a spinal chord abnormally wide in the cervical area (Picture 3). We can sometimes notice the cavity itself if the injected product of contrast accumulate there. The tomodensitometry (TD) or the Magnetic Resonance Imaging (MRI), when they have a very good quality, which means that they are realised by skilled vets, confirm the presence of a syrinx, a dysraphism or a hydrocephaly (Pictures 4 et 5). TD allows to take a better view of bone anomalies (deformation of the skull's caudal area) as MRI is better to take a view of tissue's anomalies.
Picture 3. Cervical myelography in lateral view realised on a 7-years-old CKCS shown for a weakness of front limb. Note the extreme dilation of the spinal chord in the cervical area.
Picture 4. tomodensitometry study in sagittal view of the spinal chord of the same CKCS as in Picture 1. Note the important dilation of the central canal, the medullary tissue only appearing at the periphery of the back canal.
Picture 5. Cervical sagittal MRI of the Pictures 3's CKCS balanced T2. Note the intensity of the obtained signal in cervical area in favour of syringomyelia when hydrocephaly is also visible at the level of brain.
Therapeutics options are limited. Some authors propose a surgical realisation of a more wide way at the level of the occipital opening. For the moment, there is no consensus on the interest of this surgery, a study on a great number of cases being necessary. The opening of the cavity to drain the liquid around the marrow or in the abdomen by a valve was also tempted. Using anti-inflammatory like steroids (prednisolon) and anhydrase carbonic inhibitor (acétazolamide) remove the inflammation and the pressure so reduce pain and the motor handicap of the dog. Some animals are improved so for months and even years.
Study of pedigrees realised in Great Britain by the Dr. Clare Rusbridge on more than a hundred of affected CKCS confirmed the hereditary component, so transmissible, of this disease. Two bitches are systematically found as common ancestors in 3 or 4 great breeding lines. This disease would be transmitted by several genetic recessive anomalies, without sexual predisposition. A sire (dam) being carrier of the genetic anomaly but apparently healthy can have in its descendants some animals displaying signs of disease. Blenheim and Ruby animals, of bigger consanguinity, seem to be more often affected.
The DNA collection scheme of healthy and affected dogs is in progress in Great Britain in order to find implicated gene(s). By waiting for the detection of the carriers by this way which is not yet available, it is advised of not use as sire (dam) animals that have a diagnose of syringomyelia. Still it is necessary to look for them and for this, realise a good exam of the nervous system and diagnostic exams of choice in case of doubt!
The English club advises to choose as parents of dogs without clinical signs which have very different previous history and without ascending, downward or collateral relatives known to have shown the signs.
Be careful : Some owners make, wrongly, a mixture between syringomyelia and "fly biting ". This latter disease is a form of epilepsy, called "psychomotor" which has nothing to do with syringomyelia. No genetic base is proved even if it is met effectively more often in some breeds, of which the CKCS.
So it is high time that CKCS or even king charles spaniels breeders were informed of this disease, which, today, take the risk of condemn in a short or middle term, a very popular breed for the general public.
It is probably while avoiding breeding lines affected by cardiac or ocular
problems that the selection of this disorder was made. If this work was done
for the two previous defects, the Club has to help in the awareness of the latter.
There is certainly many cases of dogs affected by syringomyelia in France, since they often come from British ancestors…
Little commentary from the Webmaster
FCKC : Even if, as far as I'm concerned, I think saying that a protocol
of selection against heart problem exists and is followed by breeders is very
optimistic, this article has the merit to put in light the situation in France…
and question whether the Club had really contact the Dr. Cauzinille has M. Métans
Published in a professional weekly paper for vets and written by an eminent French specialist, this article strangely shows a picture of the situation really different from the one drawn up by the C.E.N.A.
and, to resume a small sentence of its president, M. Métans : "Each of us has to draw the teaching".
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