Cognitive findings in spinocerebellar ataxia type 2: relationship to genetic and clinical variables.

摘要: Several authors have recently reported a broad cognitive impairment in autosomal dominant cerebellar ataxias (ADCAs) patients. However, only few studies on neuropsychological features spinocerebellar ataxia type 2 (SCA2) patients are present the current literature. The aim of this study is to evaluate wide sample SCA2 and verify role different disease-related factors (age onset, disease duration, clinical severity) intellectual abilities. We administered battery tests assessing handedness, attention, short- long-term verbal visuo-spatial memory, executive functions, constructive abilities, general abilities depression 18 belonging eight families who came our observation. Evidence impaired functions attention was found. status partially related severity rather than duration or age at onset symptoms. confirmed data defects already by others but we also found defective skills as well significant lower performances nonverbal intelligence task.