Neuronal ceroid lipofuscinosis is a type of lysosomal storage disorder that results in accumulation of lysosomal storage bodies in the cells of many tissues of the affected animal.
This leads to progressive degeneration of brain and eye cells and results in severe neurological impairment and early death.
Affected dogs appear normal at birth, but begin to exhibit symptoms early in life – around 1- 2 years of age. The age of onset and severity of the disease can vary greatly among individual dogs.
The symptoms include progressive motor decline with seizures and loss of coordinated muscle movements, cognitive decline and abnormal behavior. Visual impairment may occur.
Due to the severity of the disease, affected Border Collies rarely survive beyond 26-28 months.
There is no treatment or cure at this time.