Abstract
BackgroundThe spinocerebellar ataxias (SCA) are a heterogeneous group of progressive neurodegenerative disorders that are associated with diffuse cerebellar atrophy. While the physical symptoms of this condition have long been studied, more attention has been given to cognitive changes in recent years. We describe a case series of four adults with various genetically-confirmed subtypes of SCA.Case presentationPatients with SCA types 2, 3, and 6 presented with impaired cognitive profiles consistent with the existing literature while the reported patient with SCA-14 showed notable impairment inconsistent with the only published case controlled study.ConclusionsComparisons were made between the four patients with a common pattern of slowed processing speed, poor memory retrieval, and reduced mental flexibility. Confrontation naming and consolidation-based memory were intact across all patients. These findings are discussed in light of the relevant literature on cerebellar cognitive affective syndrome.
Highlights
ConclusionsComparisons were made between the four patients with a common pattern of slowed processing speed, poor memory retrieval, and reduced mental flexibility
The spinocerebellar ataxias (SCA) are a heterogeneous group of progressive neurodegenerative disorders that are associated with diffuse cerebellar atrophy
Confrontation naming and consolidation-based memory were intact across all patients. These findings are discussed in light of the relevant literature on cerebellar cognitive affective syndrome
Summary
SCA is a progressive neurodegenerative disease that selectively targets the cerebellum. Given the recent exploration of the cerebellum’s role in cognition, the neurocognitive profile of this disease is of interest. This study provided support for the growing literature of the neurocognitive profiles associated with SCA2, SCA3, and SCA6, while providing the first report of a pattern of deficits in SCA14. Amongst all four subtypes, slowed processing speed was found. This likely reflects the important role of the cerebellum in efficient information processing. It was noted that no patients were anomic, a finding contrary to that expected from the CCAS literature and likely indicative of less advanced disease
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