Contributed by Jean Brickell

In 1998, a hereditary condition called Cerebellar Ataxia, hereafter referred to as CA, was acknowledged in the Spinone Italiano, with the first suspect cases appearing in 1990. There is no treatment.  CA only affects young dogs.  To date, all the cases reported have been under one year of age.  CA appears to be restricted to one family line only and all cases reported to  date have one or more of the proven carriers on both sides of the pedigree. This lethal gene was traced to a dog imported from Italy, Trusco Dell Angelo Del Summano Del Gaesten, who's impact on the breed in Great Britain borders on the legendary. 

From the  information we have to date and by studying the pedigrees, it has been shown that the mode of inheritance of CA is that of a simple recessive gene.  In order for affected puppies to be produced, both sire and dam must be carriers of this lethal gene.  Puppies which are affected with CA rarely live past the age of one year and never reach the age to reproduce. Although, the loss of these puppies is heartbreaking, it is not these puppies who pose the threat to the breed. The CA affected puppies DIE. It is the littermates of these affected puppies, 50% of which WILL BE CARRIERS, who in turn IF bred will produce litters in which 25% will be carriers, who are the concern of all reputable breeders and breed enthusiasts.  It is the unidentifiable carriers which spread this lethal gene throughout the breed .

At present, there is no test although the Spinone Club of America and the Italian Spinone Club of Great Britain are working hand in hand to develop a genetic test which will establish carrier or non-carrier status of dogs from these lines. It is the recommendation of the Spinone Club of America that dogs from the carrier lines not be bred until a genetic test is developed. We have a unique opportunity to stop this genetic defect before it spreads through our gene-pool. In regard to CA lines, it is buyer beware and educate yourselves on pedigrees. You are buying this puppy. It is your responsibility to insist upon a pedigree which traces ancestry back far enough to ascertain that none of these known carriers are in the pedigree. Unfortunately, there tends to be a select few who choose to overlook and downplay the effects and consequences of CA. CA is not a contagious disease. It is true, your dog cannot CATCH CA. It is a HEREDITARY LETHAL SIMPLE RECESSIVE GENE passed from parent to offspring. It is passed down from one generation to the next. The tendency to overlook and downplay genetic defects is exactly how these genetic defects( i.e. PRA, epilepsy, heart abnormalities, liver and kidney disorders) become established in a breed. More's the pity because it doesn't have to be. It could be eliminated now by not breeding dogs from the CA lines and in 15 years, no one would even know what CA was. Due to the tremendous amount of erroneous and incomplete information being given in regard to CA, please don't take my word. Talk to your veterinarian and the Spinone Club of America, the official parent club for the breed in the U.S.

Early signs appear at around 4 to 6 months of age and could easily be mistaken for puppy clumsiness, therefore the importance of knowing the pedigree is paramount.  An affected  puppy may appear perfectly normal some days but not others as the early symptoms may be intermittent.  Some of the early signs are :

a)  an unsteady gait, as if drunk, consisting of a type of side ways staggering and crossing of both the hind and front legs. This is especially noticeable when the puppy wakes up and takes its first few steps.

b) a tendency to periodically drag the hind feet. The upper part of the foot actually comes in contact with the ground.

c) tripping over the front feet.   This is sometimes attributed to the puppy  being clumsy.

d) the puppy has a tendency to sit down more frequently than one would expect a normal active puppy to do.  This  may be difficult for a first time owner to recognize.

e) a tendency  to lean against furniture or walls for support.  It is important to note that at this stage, once the puppy is up and about, especially outdoors and unrestricted, it appears to be  normal.

As the condition progresses,  the puppy suffers a marked loss of co-ordination of the rear legs.  The puppy will  sway and adopt a wide based stance in order to steady itself.  The legs are lifted in an exaggerated manner with a swaying motion. At this stage, the symptom  could be mistaken for hip dysplasia.

Earlier symptoms become more frequent and more pronounced. The puppy suffers a loss of co-ordination of the front legs with the same exaggerated high stepping action as affects the rear.  When the front feet make contact with the ground they are brought down heavily.  This action can be easily heard, as well as seen, especially when  indoors.

From approximately 9 months on,  the symptoms become more severe. The
gait becomes more and more unsteady, with difficulty moving forward in a straight line.  The puppy veers from side to side in a very defined manner with  difficulty moving backwards or turning in a tight circle.  The puppy may even loose balance and fall over. The puppy's vision may or may not be affected.

The puppy will stabilize for a while, maybe for a week or two, and then deteriorate so that the progression occurs in steps. The truly heart rendering thing is that throughout the affliction,  the puppy appears to suffer no pain. He maintains a healthy appetite, normal bodily functions and temperament remains normal.  An affected puppy will play and enjoy daily walks just as a normal puppy would, although in the later stages exercise has to be reduced. But the end result will be death.
 
Of the  cases reported  to date,  none have survived past the age 12 months.   Most of the affected puppies have been euthanized between 10 and 11 months of age.

CAPRICHO BARONESSA (DEDUCED)

page added 11/27/99 updated 1/2004