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.

Center of Hereditary Ataxias Department of Neurology 2nd Faculty of Medicine and Motol University Hospital Charles University Prague Czech Republic

Departments of Psychiatry Clinical Neurosciences and Community Health Sciences Cumming School of Medicine University of Calgary Calgary Alberta Canada

Hotchkiss Brain Institute and O'Brien Institute of Public Health University of Calgary Calgary Alberta Canada

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