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Hereditary Ataxia/Spinocerebellar Ataxia and Neuromyotonia
  • Description
  • Signalment
  • Clinical Features
  • Neurolocalization
  • Genetics
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Hereditary ataxia is a degenerative spinal disease affecting spinal cord white matter, central auditory pathways, and peripheral nerves.

​Neuromyotonia is characterized by repetitive contraction of muscle fibers secondary to hyperexcitability of motor neurons. Muscles affected may be focal to one limb or diffusely affecting all limbs.

These two diseases have been reported to occur together, potentially reflecting a shared ion channelopathy between the two diseases.
Age of Onset: 2-6 months of age
Sex Predisposition: Any sex of animal can be affected
Clinical Course:
​
Clinical signs progressed for a period of time then became stable
Clinical Signs:
Behavioral/Mental Awareness
Abnormal vocalization
Aggression
Anxiety
Excess facial rubbing
Seizures

Posture and Appearance
Myokymia (veriform muscle movement)
Progressive generalized muscle rigidity


Movement
Ataxia
Hypermetria
Intention tremor

Cranial Nerves
Absent/decreased menace response

Spinal cord
Autosomal recessive KCNJ10 gene mutation
To read more about this disease click below:
References
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