Disorder - Gangliosidosis

Organ Systems Involved
Nervous/sensory

Alternative disorders described within LIDA

Fucosidosis, alpha; Gangliosidosis, GM1; Gangliosidosis, GM2; Gangliosidosis, GM2, GM2A deficiency; Glycogen storage disease II; Glycogen storage disease IV; Krabbe disease; Mannosidosis, alpha; Mucolipidosis II; Mucopolysaccharidosis I; Mucopolysaccharidosis VI; Mucopolysaccharidosis VII; Neuronal ceroid lipofuscinosis; Ceroid lipofuscinosis; Niemann-Pick disease, type C

Alternative Names
Lysosomal storage disease

Presenting Signs

Gangliosidoses are a group of inherited metabolic disorders. They are caused by a deficiency of different proteins that are required to break down fats within cell walls. As a result, excess fats accumulate to a harmful level in the cells, particularly in the nervous system including the brain, spinal cord and nerve roots. There are two types of gangliosidosis, GM1 and GM2; these are identified and categorised according to which protein is deficient and the number of organs involved. The most noticeable clinical signs of gangliosidosis in affected cats are worsening nervous system dysfunction, such as head shaking, problems coordinating leg movement, and weakness and paralysis of the limbs leading to collapse. Gangliosidosis is typically fatal, with deaths usually occurring by eight to ten months.

Cats at Risk

Both male and female cats are equally affected, with onset of the disorder occurring at early age, i.e. before the animal reaches one year of age. It is an inherited disorder; therefore the kitten with an affected ancestor is likely to be the carrier of the disorder, and should not be use in a breeding program.

Breeds Affected
Korat


Treatment

There is no available surgical or medication treatment available at present.

PubMed References
Gangliosidosis

Contributor
Candy Wong