Spinocerebellar ataxia type 3 (SCA3): management

Evidence-based neurology checklist on spinocerebellar ataxia type 3 (sca3): management: 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…

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

Pathology

Treatments

Investigational treatments

Synonym

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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