Progressive Neuronal Abiotrophy

1975 USKBTC Position Paper

Below are excerpts from the controversial USKBTC 1975 position paper on PNA. The
paper might be dated but it is still a major contribution to the genetic cleansing
if the breed.

To receive a copy of the complete article (copies are free to USKBTC members),
write, email, or call:

Marie Sostmann
3811 Shawney Mission Parkway
Fairway, KS 66205
913-262-2684

PROGRESSIVE NEURONAL ABIOTROPHY
A GENETICALLY INHERITED DISEASE IN KERRY BLUE TERRIERS

Prepared Under the Direction Board of Governors
UNITED STATES KERRY BLUE TERRIER CLUB, INC.

DECEMBER, 1975

PREFACE
After much deliberation and study the Board has concluded that Progressive Neuronal
Abiotrophy (PNA) is a serious threat to the health, welfare and future of the
breed and deserves your immediate attention.

In response to many requests by members of USKBTC it was decided by the Board
of Governors to gather into one paper as much of the data and discussion as practical
that might benefit the breeders of Kerry Blue Terriers in eliminating Neuronal
Abiotrophy from the breed. On the following pages you will find complete descriptions
of this lethal disease, its symptoms, and its mode of transmission. Also included
are excerpts from several papers prepared by doctors who researched the disease
and subjects relative to it.

The Board recognizes that there are other genetic diseases in Kerry Blues which
certainly need attention, study and action, but Neuronal Abiotrophy is the first
one in which a positive program of relief has the authenticity of an in-depth,
scientific research. It is not the intent of the Board to dictate to the membership
what has to be done, but rather we do feel it is our responsibility to inform
them of all such available information and afford them the opportunity to respond
to the needs of the Kerry Blue.

BOARD OF GOVERNORS, USKBTC, INC.

INTRODUCTION
The purpose of this report is to bring to you the results of a research project
that began in 1969 in which an effort was made to determine the causative agents
and nature of a lethal disease in Kerry Blues wherein puppies eight weeks of age
or older progress through various degrees of incoordination until completely incapacitated.
The disease was originally referred to as Ataxia, but later redesignated as Progressive
Neuronal Abiotrophy.

Most of the work has been accomplished by Dr. Alexander de Lahunta, Professor
of Anatomy at Cornell University, with the collaboration and assistance of Dr.
F. B. Hutt, Professor Emeritus of Animal Genetics, Cornell. Dr. Damon Averill,
Harvard School of Medicine and Boston Children’ s Hospital and Dr. T. A. Holliday,
Head of the Division of Medicine at University of California at Davis, have assisted
in the study.

Afflicted specimens of Kerries were furnished to the Universities by members of
the USKBTC from both east and west coasts of United States. Pedigrees of some
of the pups that were clinically and pathologically examined are appended and
are representative to a sufficient degree of all the specimens examined. These
pedigrees provided by the breeders, demonstrate that there is one line common
to all.

It is the opinion of the Committee that the research projects have been conducted
in a scientific and objective manner and are not unfairly prejudiced in any
way. There has been no intent by anyone associated with the research project
or this report to malign or damage the character or reputation of any person
or dog, past or present.

This report is respectfully submitted to the Board of Governors (USKBTC) for
its serious consideration for general distribution.

William Caffey, Chairman PNA Committee
Frances Reynolds
Zipporah Fleisher
Penelope Belviso

SYMPTOMS
Onset of clinical signs is generally between 9 and 16 weeks of age. Head tremor
and pelvic limb stiffness are early signs. As the disease progresses, the limb
stiffness may appear in any or all four limbs, with notable restriction of movement
in the flexion of the limb joints. Limb movements become exaggerated and progress
to severe degrees of incoordination. Usually, the hind limbs are affected first
and the fore limbs two or three weeks later. The head tremor is more noticeable
when the dog eats or drinks. Appetite is in no way affected, nor is there fever,
nasal secretion or any other sign of infection or respiratory defect. The disease
is noted by the continued happiness of the dog and the lack of any deficit other
than in gait.

Because abiotrophy is a progressive disease, the symptoms will become more pronounced.
The puppy will fall down only to get right up again and continue to try to get
about. By such time the puppy should be diagnosed without doubt. Disposition
of the puppy (preferably to one of the recognized veterinary facilities) should
be effected at such time as there are no cures nor remissions.

Hemograms, urinalyses and cerebrospinal fluid analyses all will be within normal
limits. If your veterinarian wishes corroboration for diagnostic purposes, the
club will supply him with a reference to the proper source for a more complete
description of symptoms and testing procedures. Movie films of diseased puppies
are available for loan to chapter clubs or interested groups upon application
to the USKBTC.

pna

In order for the club to keep meaningful records of the dissemination of the
disease, it is requested that breeders and owners report all suspected instances
to the Abiotrophy Committee Chairman, Mr. William Caffey, 3375 Parker Hill Road,
Santa Rosa, CA 95404, [Editor’s note: contact the current chairperson of the
USKBTC Health & Genetics Committee who will arrange for a clinical examination
by a veterinarian qualified to make an accurate diagnosis.) Because of the rarity
of the disease, the subtleties of some of its aspects may elude your local veterinarian.

Last Updated: 12/19/2002, 4:13 pm