Cerebellar cognitive affective syndrome (CCAS): pathology and causes

Evidence-based neurology checklist on cerebellar cognitive affective syndrome (ccas): pathology and causes: Pathology CCAS is caused by damage to the proximal efferent cerebellar pathways It is most prominent with posterior cerebellar lobe and vermis lesions The damage disrupts the cerebellar…

Pathology

  • CCAS is caused by damage to the proximal efferent cerebellar pathways
  • It is most prominent with posterior cerebellar lobe and vermis lesions
  • The damage disrupts the cerebellar connections with the cerebral cortex
  • It results in dysmetria of thought

Hereditary causes

Acquired causes

Synonym

References

  1. Argyropoulos GPD, van Dun K, Adamaszek M, et al. The cerebellar cognitive affective/Schmahmann syndrome: a task force paper. Cerebellum 2020; 19:102-125.
  2. Ahmadian N, van Baarsen K, van Zandvoort M, Robe PA. The cerebellar cognitive affective syndrome-a meta-analysis. Cerebellum 2019; 18:941-950.
  3. Verhoeven WM, Egger JI, Ahmed AI, Kremer BP, Vermeer S, van de Warrenburg BP. Cerebellar cognitive affective syndrome and autosomal recessive spastic ataxia of charlevoix-saguenay: a report of two male sibs. Psychopathology 2012; 45:193-199.
  4. Braga-Neto P, Pedroso JL, Alessi H, et al. Cerebellar cognitive affective syndrome in Machado Joseph disease: core clinical features. Cerebellum 2012; 11:549-556.
  5. Hoche F, Daly MP, Chutake YK, Valera E, Sherman JC, Schmahmann JD. The cerebellar cognitive affective syndrome in ataxia-telangiectasia. Cerebellum 2019; 18:225-244.
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