Neuronal Ceroid Lipofuscinosis
Neuronal ceroid lipofuscinosis are a group of lysosomal storage disorders characterized by intracellular accumulations resulting in progressive neuronal degeneration.
Age of Onset: 1-9 years (mean of 3.5 years)
Sex Predisposition: Any sex of dog can be affected
Clinical Course:
Clinical signs slowly progress over time. After years of progression the dog may be unable to stand or walk.
Clinical signs slowly progress over time. After years of progression the dog may be unable to stand or walk.
Clinical Signs:
Behavioral/Mental Awareness
Seizures
Personality change
Aggression
Hyperactivity
Loss of learned behavior
Self-mutilation
Disorientation
Movement
Ataxia
Head tremors
Tetraparesis
Hypermetria
Proprioception
Proprioceptive deficits
Cranial Nerves
Visual impairment
Behavioral/Mental Awareness
Seizures
Personality change
Aggression
Hyperactivity
Loss of learned behavior
Self-mutilation
Disorientation
Movement
Ataxia
Head tremors
Tetraparesis
Hypermetria
Proprioception
Proprioceptive deficits
Cranial Nerves
Visual impairment
Intracranial- diffuse
CLN10 phenotype -Autosomal recessive (CTSD gene mutation)
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