Disorder - Krabbe disease

Organ Systems Involved
Nervous/sensory

Alternative disorders described within LIDA
    Lysosomal storage diseases

Alternative Names
Globoid cell leucodystrophy; Galactosylceramide lipidosis; Galactocerebrosiderosis

Presenting Signs
    Krabbe disease is a rare disorder causing degeneration of the nervous system. Many nerves in the body are surrounded by a protective layer called the myelin sheath. In Krabbe disease there is a mutation of the gene responsible for the development of this myelin sheath. This interferes with normal growth of the sheath so the nerves cannot function properly. Kittens with this disease usually appear normal at birth. The disease progresses rapidly, resulting in severe breakdown of neurological function. The first signs of disease are incoordination of limbs, body tremors and muscle weakness. As the disease progresses sensation of the limbs may be lost and the limbs become paralysed. Eventually the muscles responsible for breathing are paralysed, and this results in death as the cat is unable to breathe effectively.

Cats at Risk
    Most commonly affects kittens within the first few months of life.

Treatment
    No treatment available

PubMed References
Krabbe disease

Contributor
Carlie Soul