COGNITIVE OR behavioral abnormalities are not generally considered to be important features of the autosomal dominant cerebellar ataxias (ADCAs). However, Harding1 observed significant cognitive impairment in more than 25% of patients with ADCA, and others have documented attentional and frontal-executive dysfunction related to disease duration in a large cohort of patients with clinically diagnosed ADCA.2 However, few studies have specifically focused on documenting the nature of cognitive impairment in patients with ADCA. The discovery of the genetic abnormality responsible for an increasing proportion of patients with ADCA offers the opportunity to study in a more precise manner the interaction of the identified spinocerebellar ataxia (SCA) mutations, other familial factors, and the environment and their relationship to cognitive functioning.
Geschwind DH. Focusing Attention on Cognitive Impairment in Spinocerebellar Ataxia. Arch Neurol. 1999;56(1):20-22. doi:10.1001/archneur.56.1.20