Teive Helio Afonso Ghizoni, Arruda Walter Oleschko
Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil.
Dement Neuropsychol. 2009 Jul-Sep;3(3):180-187. doi: 10.1590/S1980-57642009DN30300002.
Spinocerebellar ataxias (SCAs) comprise a heterogeneous group of complex neurodegenerative diseases, characterized by the presence of progressive cerebellar ataxia, associated or otherwise with ophthalmoplegia, pyramidal signs, extrapyramidal features, pigmentary retinopathy, peripheral neuropathy, cognitive dysfunction and dementia.
To verify the presence of cognitive dysfunction among the main types of SCA described in the literature.
the review was conducted using the search system of the PUBMED and OMIM databases.
Cognitive dysfunction occurs in a considerable proportion of SCA, particularly in SCA 3, which is the most frequent form of SCA worldwide. Dementia has been described in several other types of SCA such as SCA 2, SCA 17 and DRPLA. Mental retardation is a specific clinical feature of SCA 13.
The role of the cerebellum in cognitive functions has been observed in different types of SCAs which can manifest varying degrees of cognitive dysfunction, dementia and mental retardation.
脊髓小脑共济失调（SCAs）是一组异质性的复杂神经退行性疾病，其特征为进行性小脑共济失调，可伴有或不伴有眼肌麻痹、锥体束征、锥体外系特征、色素性视网膜病变、周围神经病变、认知功能障碍和痴呆。
验证文献中描述的主要类型脊髓小脑共济失调中认知功能障碍的存在情况。
使用PUBMED和OMIM数据库的检索系统进行综述。
相当一部分脊髓小脑共济失调患者存在认知功能障碍，尤其是在脊髓小脑共济失调3型中，这是全球最常见的脊髓小脑共济失调类型。在其他几种脊髓小脑共济失调类型中也有痴呆的描述，如脊髓小脑共济失调2型、脊髓小脑共济失调17型和齿状核红核苍白球路易体萎缩症（DRPLA）。智力发育迟缓是脊髓小脑共济失调13型的一个特定临床特征。
在不同类型的脊髓小脑共济失调中均观察到小脑在认知功能中的作用，这些类型可表现出不同程度的认知功能障碍、痴呆和智力发育迟缓。
