| Main clinical features
|
neurodegenerative disorder characterized by onset of gait ataxia and/or cognitive-affective symptoms in midadulthood
gait ataxia, dysarthria, and dysphagia, as well as cognitive decline, deficits in executive function, and psychiatric or affective manifestations, such as depression, anxiety, and apathy
brain imaging shows selective atrophy of the posterior areas of the cerebellar vermis, often with bilateral T2-weighted hyperintensities in the dentate nuclei (the 'crab sign') |