Cerebellar Abiotrophy/Cerebellar Cortical Abiotrophy/Cerebellar Cortical Degeneration/Cerebellar Degeneration
Cerebellar abiotrophy results from a loss of vital substance for continued life of the neuron. Many of these diseases are associated with loss of purkinje cells.
Age of Onset: Clinical signs occur around 4-6 years of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
Clinical signs typically worsen over time
Clinical signs typically worsen over time
Clinical Signs:
Behavioral/Mental Awareness
Incessant crying
Posture and Appearance
Periodic opisthotonus
Wide-based stance
Movement
Hypermetria
Pelvic limb stiffness
Inability to stand
Ataxia
Rolling on side
Unable to remain in a sternal position
Intention tremor
Dysmetria
Mild spasticity
“lurching” or forward-falling movements
Loss of muscle tone
Incoordination
Proprioception
Proprioceptive deficits
Cranial Nerves
Absent menace response
Vertical nystagmus
Positional nystagmus
Episodic facial paralysis (some reports)
Other
May be associated with hydrocephalus
May be associated with storage diseases (Niemann-Pick type C)
Behavioral/Mental Awareness
Incessant crying
Posture and Appearance
Periodic opisthotonus
Wide-based stance
Movement
Hypermetria
Pelvic limb stiffness
Inability to stand
Ataxia
Rolling on side
Unable to remain in a sternal position
Intention tremor
Dysmetria
Mild spasticity
“lurching” or forward-falling movements
Loss of muscle tone
Incoordination
Proprioception
Proprioceptive deficits
Cranial Nerves
Absent menace response
Vertical nystagmus
Positional nystagmus
Episodic facial paralysis (some reports)
Other
May be associated with hydrocephalus
May be associated with storage diseases (Niemann-Pick type C)
Cerebellum
Autosomal recessive inheritance
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