Gangliosidosis Type II (GM2/Sandhoff-Like Disease)
Gangliosidosis (GM2) is a deficiency in the enzyme beta-galactosidase which causes defects in the catabolism of gangliosides resulting in their accumulation in neuronal lysosomes.
Age of Onset: Less than 18 months of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
Clinical signs worsen over time
Clinical signs worsen over time
Clinical Signs:
Behavioral/Mental Awareness
Anxiety
Decreased responsiveness to verbal commands
Posture and Appearance
Wide-based stance
Altered tail carriage – tail down
Movement
Stiff gait
Falling
Ataxia
Tetraparesis
Intention tremor
Inability to climb up or down stairs
Cranial Nerves
Decreased bilateral corneal reflex
Decreased/Absent bilateral menace response
Impaired vision
Special Functions (e.g. respiration; urination)
Vomiting
Inappetence
Behavioral/Mental Awareness
Anxiety
Decreased responsiveness to verbal commands
Posture and Appearance
Wide-based stance
Altered tail carriage – tail down
Movement
Stiff gait
Falling
Ataxia
Tetraparesis
Intention tremor
Inability to climb up or down stairs
Cranial Nerves
Decreased bilateral corneal reflex
Decreased/Absent bilateral menace response
Impaired vision
Special Functions (e.g. respiration; urination)
Vomiting
Inappetence
Brain and spinal cord
Autosomal recessive inheritance - mutation in the HEXB gene
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