Center of Hereditary Ataxias, Department of Neurology, 2nd Faculty of Medicine and Motol University Hospital, Charles University, Prague, Czech Republic.
Hotchkiss Brain Institute and O'Brien Institute of Public Health, University of Calgary, Calgary, Alberta, Canada; Departments of Psychiatry, Clinical Neurosciences, and Community Health Sciences, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
Neurosci Biobehav Rev. 2023 Jul;150:105205. doi: 10.1016/j.neubiorev.2023.105205. Epub 2023 May 1.
Apart from its role in motor coordination, the importance of the cerebellum in cognitive and affective processes has been recognized in the past few decades. Spinocerebellar ataxias (SCA) and Friedreich ataxia (FRDA) are rare neurodegenerative diseases of the cerebellum presenting mainly with a progressive loss of gait and limb coordination, dysarthria, and other motor disturbances, but also a range of cognitive and neuropsychiatric symptoms. This narrative review summarizes the current knowledge on neuropsychiatric impairment in SCA and FRDA. We discuss the prevalence, clinical features and treatment approaches in the most commonly reported domains of depression, anxiety, apathy, agitation and impulse dyscontrol, and psychosis. Since these symptoms have a considerable impact on patients' quality of life, we argue that further research is mandated to improve the detection and treatment options of neuropsychiatric co-morbidities in ataxia patients.
除了在运动协调中的作用外,小脑在认知和情感过程中的重要性在过去几十年中得到了认可。脊髓小脑共济失调(SCA)和弗里德里希共济失调(FRDA)是罕见的小脑退行性疾病,主要表现为步态和肢体协调进行性丧失、构音障碍和其他运动障碍,但也存在一系列认知和神经精神症状。本综述总结了目前关于 SCA 和 FRDA 神经精神障碍的知识。我们讨论了在最常报告的抑郁、焦虑、淡漠、激越和冲动控制障碍以及精神病等领域中,患病率、临床特征和治疗方法。由于这些症状对患者的生活质量有相当大的影响,我们认为需要进一步研究,以改善对共济失调患者神经精神合并症的检测和治疗选择。