Ceroid Lipofuscinosis (CL) is a metabolic disease which affects the nerve cells of the body.  Commonly referred to as Storage Disease, CL appears in cats, cattle, sheep and humans, as well as several breeds of dogs.  This condition is believed to be inherited recessively.
        Ceroid Lipofuscinosis is caused by the lack of an enzyme, which allows a waste product (ceroid lipofuscin) to accumulate in body cells.  The ceroid lipofuscin is deposited predominantly in the nervous system, particularly the neurons.  Brain cells are rather compact in their nature, and have very little space for the build up of any waste product.  As the waste deposits accumulate in a specific area of the brain, the build-up tends to lead to compression and eventual destruction of healthy brain cells.
        Dogs do not develop ceroid lipofuscinosis, they are born with this condition.  At birth, however, they are asymptomatic.  It takes time for ceroid lipofuscin to accumulate in the cells, to the point where it is detrimental to the cells.  For this reason, most affected dogs will not display signs until they are approximately 15 to 18 months of age.
        One of the first symptoms usually seen is hyperactivity with aimless wandering.  When the dog first starts showing symptoms of abnormal behavior, these displays can be episodic.  As the disease progresses, the episodes become more frequent and severe.  Behavioral changes include : apprehension of familiar objects/surroundings; snapping; biting; disproportionate responses to auditory, visual, or tactile stimulus; abnormal gait (unsteady on feet, has difficulty with jumping or climbing); demented behavior (mania, hyperactivity, rage).
        Upon the onset of symptomatic displays, this disease progresses rapidly.  Blindness often occurs in the later stages of the disease.  Most often affected animals are euthanized, as there is no treatment at this time.  Currently, there are no test available to detect this condition in a live animal.  A brain biopsy performed during a post mortem can positively identify the presence of this condition.

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