Agarwal Ayush, Faruq Mohd, Garg Ajay, Srivastava Achal K
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Department of NMR, All India Institute of Medical Sciences, New Delhi, India.
Ann Indian Acad Neurol. 2022 Jul-Aug;25(4):601-605. doi: 10.4103/aian.aian_63_22. Epub 2022 Sep 9.
Spinocerebellar ataxias (SCAs) comprise a group of complex and heterogeneous hereditary neurodegenerative disorders characterized by cerebellar ataxia, with ophthalmoplegia, pyramidal and extrapyramidal features, peripheral neuropathy, motor neuron disease, pigmentary retinopathy, epilepsy, and dementia in varying proportions. Cognitive impairment is not frequent in SCAs but is rarely noticed since it gets camouflaged behind the exorbitant ataxic manifestations of the disease. The exact incidence and extent of cognitive impairment in these rare disorders are not known due to the heterogeneity between different SCA types and different modalities of testing employed in different studies. Through our review, we have summarized the cognitive aspects of SCA and can safely conclude that cognitive dysfunction is common in some SCA types when compared to others. Not only is it important to appreciate its presence as a symptom complex in SCA but also is the need to actively search and treat it to improve the patients' quality of life.
脊髓小脑共济失调（SCAs）是一组复杂且异质性的遗传性神经退行性疾病，其特征为小脑性共济失调，并伴有不同比例的眼肌麻痹、锥体束和锥体外系特征、周围神经病变、运动神经元病、色素性视网膜病变、癫痫和痴呆。认知障碍在脊髓小脑共济失调中并不常见，但由于它被该疾病严重的共济失调表现所掩盖，所以很少被注意到。由于不同脊髓小脑共济失调类型之间存在异质性，且不同研究采用的检测方式不同，这些罕见疾病中认知障碍的确切发病率和程度尚不清楚。通过我们的综述，我们总结了脊髓小脑共济失调的认知方面，并可以有把握地得出结论，与其他类型相比，认知功能障碍在某些脊髓小脑共济失调类型中很常见。认识到它作为脊髓小脑共济失调中的一种症状复合体的存在不仅很重要，积极寻找并治疗它以提高患者的生活质量也很有必要。
