Cognition is affected in many of the inherited ataxias, and sometimes that is the main problem patients have to deal with. We recently teamed up with colleagues in Brazil to draw attention to the existence of the cerebellar cognitive affective syndrome (CCAS) in Spinocerebellar Ataxia Type 21 (SCA 21). This is described in our letter and reply just published in the journal, Brain; Cognition in SCA21 reflects developmental and adult onset cerebellar cognitive affective syndrome, REPLY-Cognition in SCA21 reflects developmental and adult onset cerebellar cognitive affective syndrome. The reason this is important is it allows patients and their families to recognize cognitive problems in the ataxic disorders, and it enables doctors and treating clinicians to understand these problems and take steps to treat them and help enhance quality of life.
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