Globoid Cell Leukodystrophy (Krabbe's Disease/Galactocerebrosidosis)
Globoid cell leukodystrophy is a storage disease caused by a deficiency in the enzyme beta-galactocerebrosidase. This deficiency leads to the accumulation of galactocerebrosides within white matter leading to a progressive neurodegeneration.
Age of Onset: 3 years of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
Clinical signs progress from paresis to paralysis over the course of 2-3 months. Commonly, signs first begin in the thoracolumbar spinal cord and progress to cervical spinal and cerebellar signs. However, cerebellar signs may precede spinal signs.
In contrast to other breeds, Globoid cell leukodystrophy in Basset hounds primarily affects spinal cord structures while preserving the cerebrum
Clinical signs progress from paresis to paralysis over the course of 2-3 months. Commonly, signs first begin in the thoracolumbar spinal cord and progress to cervical spinal and cerebellar signs. However, cerebellar signs may precede spinal signs.
In contrast to other breeds, Globoid cell leukodystrophy in Basset hounds primarily affects spinal cord structures while preserving the cerebrum
Clinical Signs:
Behavioral/Mental Awareness
Dementia
Movement
Ataxia
Tremors
Pelvic limb weakness/paresis
Proprioception
Proprioceptive deficits (pelvic limb > thoracic limb)
Cranial Nerves
Blindness
Spinal Reflexes
Decreased spinal reflexes
Reduced muscle tone
Special Functions (e.g. respiration; urination)
Incontinence
Muscle Atrophy
Muscular atrophy
Behavioral/Mental Awareness
Dementia
Movement
Ataxia
Tremors
Pelvic limb weakness/paresis
Proprioception
Proprioceptive deficits (pelvic limb > thoracic limb)
Cranial Nerves
Blindness
Spinal Reflexes
Decreased spinal reflexes
Reduced muscle tone
Special Functions (e.g. respiration; urination)
Incontinence
Muscle Atrophy
Muscular atrophy
Diffuse central nervous system disease
Autosomal recessive mutation in galactosylceramidase 1 (beta-galactocerebrosidase)
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