The detail information of Globoid cell leukodystrophy
Basic Information

Disease Description: Abnormal development and/or function of certain types of white globoid cells in the brain. Krabbe disease.

Inherit Mode: This is a rare disorder that is seen most often in the Cairn and West Highland white terriers, in which it is inherited as an autosomal recessive trait. It is also autosomal recessive in the miniature poodle, Irish setter and Australian kelpie. The mode of inheritance is unknown in other breeds.

Disease Symptom: Puppies affected with GCL are normal at birth but grow more slowly than their littermates, and begin to to show signs of incoordination by 3 to 6 months. You may see tremors, a stiff gait, weakness, poor balance (falling to one side, stumbling), changes in behaviour or attitude, and vision changes. The disease is rapidly progressive and is invariably fatal. Affected dogs generally die or are euthanized by about 12 months.

Disease Cause: -

Disease Diagnose: Your veterinarian will do a thorough neurologic exam on your dog. Diagnostic tests for other conditions that might cause your dog's signs will show no abnormalities. As the disease progresses, magnetic resonance imaging (if available) will show diffuse disease in the white matter. There is a DNAtest available through HealthGene that will identiify normal, carrier (heterozygous) and affected Cairn terriers and Westies. A DNA test is also available in some other breeds. See the Orthopedic Foundation of America's currently available tests for more information.

Treat Method: Unfortunately there is no treatment for this condition. Your veterinarian will help you decide when your dog's condition has deteriorated to the point where euthanasia is the best option.

Breeder Advice: Affected animals and their parents (who are carriers of the defective gene) should not be bred. There is a DNAtest available through HealthGene that will identiify normal, carrier (heterozygous) and affected Cairn terriers and Westies. A DNA test is also available in some other breeds. See the Orthopedic Foundation of America's currently available tests for more information.

Disease Description Source: Link

Associated Diseases
Disease Name Other Name Mode of inheritance Link ID Possible OMIM ID Gene
Krabbe disease globoid cell leukodystrophy Autosomal Recessive GALC
Associated Breeds
iDog Breed Number Breed Name Personality Height Weight Breed Source
CB23 Bavarian Mountain Scent Hound Loyal, Versatile, Reserved with Strangers 17-20.5 inches 37-66 pound
CB24 English Beagle Merry, friendly, and curious 33 cm & under 33-38.1 cm under 9.1 kg (5.9 inches & under), 9.1-13.6 kg (5.9-6.8 inches) United Kingdom (England)
CB25 Bearded Collie Bouncy, charismatic, friendly; smart, but also stubborn and independent 53.3-55.9 cm (male), 50.8-53.3 cm (female) 20.4-24.9 kg United Kingdom (Scotland)
CB40 Bluetick Coonhound Smart,Fearless 55.9-68.6 cm (male), 53.3-63.5 cm (female) 24.9-36.3 kg (male), 20.4-29.5 kg (female) United States
CB41 Boerboel(South African Mastiff) Smart, dominant, protective, confident; loving with family, especially kids 61-68.6 cm (male), 55.9-63.5 cm (female) 68-90.7 kg South Africa
CB60 Cairn Terrier Cheerful, alert, busy and independent-minded 25.4 cm (male), 24.1 cm (female) 6.4 kg (male), 5.9 kg (female) United Kingdom (Scotland)
CB61 Canaan Dog Alert, vigilant, territorial; docile with family, aloof with strangers 50.8-61 cm (male), 48.3-58.4 cm (female) 20.4-24.9 kg (male), 15.9-20.4 kg (female) Middle East Lebanon - Israel
CB84 Dalmatian Stable, Outgoing;Dignified but never shy 48.3-61 cm 20.4-31.8 kg Croatia
CB85 Dandie Dinmont Terrier Independent, proud, smart; affectionate at home, bold and tenacious in the field 20.3-27.9 cm 8.2-10.9 kg United Kingdom (Scotland)
CB131 Irish Setter Outgoing, sweet-natured, active, and trainable 68.6 cm (male), 63.5 cm (female) 31.8 kg (male), 27.2 kg (female) Ireland
CB188 Poodle Proud, active and very smart 25.4 cm & under (toy), 25.4-38.1 cm (miniature), over 38.1 cm (standard) 1.8-2.7 kg (toy), 4.5-6.8 kg (miniature), 27.2-31.8 kg (male standard), 18.1-22.7 kg (female standard) Germany, France
CB255 West Highland White Terrier Happy, smart, loyal, entertaining 27.9 cm (male). 25.4 cm, female 6.8-9.1 kg United Kingdom (Scotland)
CB256 English Whippet Calm, affectionate, and playful 48.3-55.9 cm (male), 45.7-53.3 cm (female) 11.3-18.1 kg England
CB266 Australian Kelpie Alert, eager and highly intelligent, with an inexhaustible energy, loyalty and devotion to duty. 43-51 cm Australia
CB268 Miniature Poodle Intelligent, Eager-to-please , Charming companion 25-38 cm 4.5-6.8kg France&Germany
CB270 Toy Poodle Intelligent, eager to please, quick to learn and an affectionate, loyal pet. under 25 cm
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