NEONATAL CEREBELLAR CORTICAL DEGENERATION


"DRUNKEN" OR TUMBLING PUPPY SYNDROME



GENETICS:
The Animal Health Trust in the UK has identified a genetic marker and developed a genetic test for Neonatal Cerebellar Cortical Degeneration and this is considered to be cause of this syndrome. If you have an affected puppy then both parents are carriers. Any normal littermates can be tested to see if they are clear or carriers genetically. This test is available from many labs offering genetic testing for dogs.
Detailed information on puppies with this disorder and the effort to develop the genetic marker--Overview on FACEBOOK   VIDEO OF AFFECTED PUPPIES
A puppy is slower to walk than its littermates.  It is very unsteady on its feet and often circles to the same side or falls to the same side.  You notice a head tilt and odd side to side movement of the eyes.  By the age of 10-12 weeks the odd behaviors of circling, falling and being off balance are "just how this puppy moves".  The puppy acts uncoordinated,  like a drunk person-it staggers and falls!!  Sometimes with maturity the puppy can walk straight, but when trying to move at a fast pace such as a trot, the uncoordinated side motions returns.  The puppy seems to fall over its own feet and runs to one side.  Going up or down stairs is a challenge.

This syndrome was first reported  in 1991.    Ada Lueke included it in her book "The New Beagle".  As of the printing of that book (1998) there had been 13 cases reported, all with the same symptoms. While the numbers affected were low the pedigrees were suggestive of a genetic nature to the problem. There have been more  cases reported since then with most of those puppies being euthanized at an early age.  A couple of  beagles affected have lived well into adult hood and senior years.  One was euthanized due to arthritis and pain secondary to chronic falls.

There appears to be a range of "affectiveness". Some cannot even get up and others just stagger or fall when moving fast. Puppies surviving to adult hood seem to stabilize and even improve mildly.  But, care must be taken to protect them from challenges such as stairs, holes, or unlevel surfaces.  Homes with flat yards and limited obstacles and no stairs are best suited for these puppies.  Spaying and neutering is essential and with protective care the mildly and some moderately affected beagles can live out a happy life.  These beagles are not deaf and it does appear to be a different syndrome than the bilateral vestibular disease reported in some breeds.

VIDEO #1 OF AN ADULT   VIDEO #2



A thorough examination by a veterinary is the first and most important step in treating these puppies.  Other problems such as hydrocephalus can present with similar symptoms.

Link with Additional Information

Here is a necropsy report from a litter with three affected puppies.  They were 4-5 weeks of age when necropsy was done.

GROSS FINDINGS:  Nothing significant.  Puppies are considered to be in good nutritional condition.

MICROSCOPIC FINDINGS:The microscopic findings are nearly identical between the puppies with only minor insignificant differences. 

BRAIN:  There is a greater than expected cellularity of the outer molecular layer of the cerebellum.  There is occasional single cell loss of cerebellar Purkinje cells with vacuolation to necrosis.  Scattered bilaterally through the brainstem, at the level of the seventh cranial nerve, are small foci of increased fibrillar eosinophilia, thickening and disorder. 
CERVICAL SPINAL CORD:  No significant lesions. 
LIVER: No significant lesions
KIDNEY:  No significant lesions 
LUNG:  No significant lesions

In the most affected puppy, this statement was added to the BRAIN section:  Of the three puppies, the degree of neurofibrillary change is slightly greater in this pup.

DIAGNOSIS:

Cerebellum:  Mild, subacute, Purkinje cell vacular degeneration and necrosis

Cerebellum:  Moderate, diffuse molecular layer migrational delay

Brainstem:  Mild, bilateral, multifocal, neurofibrillary degeneration

COMMENT:  The lesions in each of these puppies are similar in regard to localization, degree and progression.  The findings indicate developmental and degenerate processes acting in tandem and strongly suggest a common genetic-based metabolic derangement.  Attempts to further characterize the derangement by specific histochemical stains (Sevier-Munger. Sevier-Munger/Luxol Fast Blue, Beilh-Sikowski, Toluidine Blue) have been unsuccessful.  There is no indication of a viral (distemper) or immunologic (vaccinal) etiology in the tissues examined.  Slides have been forwarded to another testing place for further staining procedures.  Should these stains yield any additional information an addendum will be forwarded.  (Nothing has been received since this report was issued.)

If you have a puppy or puppies that may have this disorder please contact Darlene Stewart. Please consider submitting DNA swabs to the DNA Data bank available through the Canine Health Information Center (CHIC) and to the Animal Health Trust before the puppies are euthanized or placed into homes.