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Neuronal Ceroid Lipofuscinosis
Video 1
  • Description
  • Signalment
  • Clinical Features
  • Neurolocalization
  • Genetics
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​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 (most are less than 2 years of age)
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:
Behavioral/Mental Awareness
Seizures
Personality change
Aggression
Hyperactivity
Loss of learned behavior
Self-mutilation
Anxiety

Movement
Ataxia
Head tremors
Tetraparesis
Jaw chattering


Proprioception
Proprioceptive deficits

Cranial Nerves
Visual impairment
Intracranial - diffuse
Autosomal recessive inheritance of CLN8 nonsense variant

For breed specific genetic tests, follow the links below:
https://cvm.missouri.edu/research/canine-genetics-laboratory/canine-genetics-laboratory-testing/dna-tests-by-disease/​
To read more about this disease click below:
References
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