Syringomyelia info and links

[Karlin]

From Nicki (info from UK CKCS ckub):

Description:

Syringomyelia 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.


Symptoms:

Scratching at the neck/shoulders/ears particularly when excited eg on the lead {contact is not usually made with the skin} NB it’s now thought that less than 50% of affected dogs do scratch; neck pain; hypersensitivity to grooming, being touched etc; head tilt and head turn; scoliosis; weakness and in-coordination of the limbs; seizures; changes in peripheral vision and facial palsy.

Diagnosis:

Diagnosis can be strongly suspected on a clinical basis, meaning based on the presence of the typical symptoms. Definitive diagnosis is based on MRI.

Websites:

http://website.lineone.net/~malburley/index.html
www.thecavalierclub.co.uk/start.html
www.cavaliers.co.uk/articles/syringohydromyelia.htm

Affected breeds - to date, dogs of the following breeds have been diagnosed with Syringomyelia: Boston Terrier, Cavalier King Charles Spaniel, Chihuahua, French Bulldog, Griffon Bruxellois, King Charles Spaniel, Maltese Terrier, Pomeranian, Staffordshire Bull Terrier and Yorkshire Terrier

Breeding Advice:

The UK CKCS Club advise that to reduce the incidence of MVD, the dog or bitch to be used for breeding should be at least 2 ½ years old with a clear heart, with parents with clear heart certificates issued at 5 years of age or older. As symptoms of Syringomyelia usually show by 3 years of age, it is recommended to delay breeding until then, also taking into account the status of relatives. If possible, dogs and bitches should be MRI scanned. This will determine if they have the malformation, BUT even if they are “clear�, they can still be carriers. At present this is the best that you can do to ensure healthy pups.

UK low cost MRI Scheme:

Mr Skerritt - Chester Tel: 01244 853823 SKERRVET@aol.com
Mr Robinson – Derbyshire Tel: 01332 810395
alisonrobinson@dovecoteveterinaryhospital.co.uk
Dr Nick Jeffery - Cambridge Tel: 01223 337621 ndj1000@cam.ac.uk


Similar schemes in the USA – for example the HEALEY STUDY. For details contact Joseph A. DeLucia DVM,CCRP, Valley Veterinary Rehabilitation, New Jersey 07013, Tel : 973-509-5225 ext 103,www.valleyvetrehab.com . Veterinary MRI and RT Centre of New Jersey, New Jersey 07011 973 772-9902 www.VetMRIRT.com

DNA collection program:

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. Details of the UK collection can be found on http://www.thecavalierclub.co.uk

[Karlin]

From UK researcher Clare Rusbridge:


What’s in a name?
Is it occipital hypoplasia/syringomyelia complex, caudal occipital malformation syndrome (COMS), Chiari type I malformation or Arnold Chiari malformation? In 1891 Hans von Chiari, a pathologist in Prague, described 4 types of abnormality based on autopsy of infants with hydrocephalous that died shortly after birth. In translation, Type 1 was described as “elongation of the cerebellar tonsils and the medial part of the interior cerebellar lobes into conelike projections, which accompany the medulla into the spinal canal�. Arnold’s name was subsequently added, it is rumoured by two of his students, on the basis of a description of single case and for many years the condition was referred to as Arnold-Chiari syndrome. In recent years the trend has been for simplifying the name to Chiari malformation or the more anatomically accurate term “hindbrain descent�. To use the term Chiari type I or Arnold-Chiari malformation to describe the malformation in the CKCS is not strictly correct as the condition in the dog is inconsistent with the historical description, for example dogs don’t have cerebellar tonsils. The term occipital hypoplasia with secondary syringomyelia, although longwinded, does accurately describe the condition and therefore is least likely to result in confusion. In humans, the term Chiari malformation is convenient “shorthand� for a wide range of similar abnormalities distinct from Hans von Chiari’s original description. Therefore if an eponym must be used for the dog condition, Chiari malformation is probably the most appropriate. Perhaps it would be simpler to use initials, for example OH/SM complex!

Reference: Batzdorf, U. Treatment of Syringomyelia Associated with Chiari I malformation in Syringomyelia: Current Concepts and Management eds N Tamaki, U Batzdorf, T Nagashima Springer-Verlag Tokyo 2001 pp 121-123.

[Karlin]

Support group:

http://groups.yahoo.com/group/CKCS-SM-support

General SM discussion group:

http://groups.yahoo.com/group/CKCS-SM