What  is  NCL

Canine Ceroid Lipofuscinosis is an inherited neurological disorder affecting Tibetan Terriers for which there is no known cure. It is a multi-systemic storage disease thought to be caused by an inborn error of metabolism. This metabolic error results in the accumulation of storage bodies thought to affect the ability of nerve cells to function. CCL (NCL) in Tibetan Terrier has been characterized by manifestation of at least some of the following symptoms and diagnostic observations:

- decrease in or loss of vision, particularly in dim light, changes in dark adaptation
- ataxia, a loss of normal muscular co-ordination and movement, often associated with an irregular gait, loss of balance, and a roaching of
  the back
- a progressive Alzheimer-like dementia, which can include difficulty adapting to changes in environment, confusion, aggressive behaviour
- seizures in the advanced stages of the disease

New DNA Genetic Testing Scheme for NCL

At the request of the Tibetan Terrier Clubs in England, the Kennel Club (U.K.) has recently approved a new official DNA testing scheme for Neuronal Ceriod Lipofuscinosis (NCL). The test is offered by the Orthopaedic Foundation for Animals (OFA) in America and further details can be obtained from them at

Adult-Onset Neuronal Ceroid Lipofuscinosis In Tibetan Terriers


The Animal Health Trust is pleased to be able to announce the launch of a DNA test for adult-onset neuronal ceroid lipofuscinosis (NCL) in Tibetan Terriers. The causative mutation for this devastating disease was identified and reported in 2011 by a collaboration between US and European scientists as a tiny deletion of DNA in a gene called ATP13A2.

The development of the test at the AHT has been a result of a collaboration with Tibetan Terrier breeders in the UK who generously sent samples in from their dogs to enable us to develop and validate the test.


The test will be available through our Webshop ( from 21st May, 2012 where cheek swab sampling kits can be ordered.


As an introductory offer, the NCL test is offered in combination with the test for primary lens luxation for £48 inc VAT. The NCL test is available alone for £30 inc VAT.


© Siddhartha Tibetan Terriers Ireland