Spinocerebellar ataxia type 3 (SCA3): management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Spinocerebellar ataxia type 3 (SCA3): management. Severe olivopontocerebellar atrophy. Fully referenced neurology checklist.

Magnetic resonance imaging (MRI) brain: features

  • Severe olivopontocerebellar atrophy
  • Reduced volume of putamen and caudate nuclei
  • Reduced brainstem volume: especially of the pons
  • Reduced cervical spinal cord volume: especially at C2 and C3 segment

Electromyogram (EMG): features

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Pathology

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Treatments

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Investigational treatments

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Synonym

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References

  1. Faber J, Schaprian T, Berkan K, et al. Regional brain and spinal cord volume loss in spinocerebellar ataxia type 3. Mov Disord 2021; 36:2273-2281.
  2. Whaley NR, Fujioka S, Wszolek ZK. Autosomal dominant cerebellar ataxia type I: a review of the phenotypic and genotypic characteristics. Orphanet J Rare Dis 2011; 6:33.
  3. Fujioka S, Sundal C, Wszolek ZK. Autosomal dominant cerebellar ataxia type III: a review of the phenotypic and genotypic characteristics. Orphanet J Rare Dis 2013; 8:14. 
  4. Bürk K, Fetter M, Abele M, et al. Autosomal dominant cerebellar ataxia type I: oculomotor abnormalities in families with SCA1, SCA2, and SCA3. J Neurol 1999; 246:789-797.
  5. Bürk K, Abele M, Fetter M, et al. Autosomal dominant cerebellar ataxia type I clinical features and MRI in families with SCA1, SCA2 and SCA3. Brain 1996; 119:1497-1505.
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