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 may be present from birth to 6 months of age (most commmonly from 5-7 weeks or greater than 12 weeks 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
Seizures
Movement
Dysmetria/Hypermetria
Pelvic limb stiffness
Inability to stand
Ataxia (pelvic limbs > throacic limbs)
Head (intention) tremor
Rolling on side
Unable to remain in a sternal position
Dysmetria
Mild spasticity
“lurching” or forward-falling movements
Staggering
Falling
Circling
Loss of muscle tone
Proprioception
Proprioceptive deficits
Cranial Nerves
Absent menace
Other
May be associated with hydrocephalus
May be associated with storage diseases (Niemann-Pick type C)
Behavioral/Mental Awareness
Seizures
Movement
Dysmetria/Hypermetria
Pelvic limb stiffness
Inability to stand
Ataxia (pelvic limbs > throacic limbs)
Head (intention) tremor
Rolling on side
Unable to remain in a sternal position
Dysmetria
Mild spasticity
“lurching” or forward-falling movements
Staggering
Falling
Circling
Loss of muscle tone
Proprioception
Proprioceptive deficits
Cranial Nerves
Absent menace
Other
May be associated with hydrocephalus
May be associated with storage diseases (Niemann-Pick type C)
Cerebellum
Suspected autosomal recessive inheritance (CFA20 locus associated with early onset disease and CFA9 locus assocaited with late-onset disease)
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