Highlights•Cognitive impairment in SCAs is well-established, but not universal in all
patients.•The CCAS Scale is the current standard for identifying cognitive impairments in …

Abstract Purpose of Review Spinocerebellar ataxias (SCAs) are autosomal dominant
degenerative syndromes that present with ataxia and brain stem abnormalities. This review …

The role of the cerebellum in cognition, both in healthy subjects and in patients with
cerebellar diseases, is debated. Neuropsychological studies in spinocerebellar ataxia type 1 …

Abstract Spinocerebellar ataxia type 3 (SCA3), the commonest dominantly inherited ataxia
worldwide, is characterized by disruption in the cerebellar-cerebral and striatal-cortical …

Background The spinocerebellar ataxias (SCA) are a heterogeneous group of progressive
neurodegenerative disorders that are associated with diffuse cerebellar atrophy. While the …

Abstract The Cerebellar Cognitive Affective/Schmahmann Syndrome (CCAS) manifests as
impaired executive control, linguistic processing, visual spatial function, and affect …

A subtype-specific impairment of cognitive functions in spinocerebellar ataxia (SCA) patients
is still debated. Thirty-two SCA patients (SCA1, 6; SC2, 3; SCA3, 15; SCA6, 8) and 14 …

The aim of this study is to determine whether the initial symptom associates with motor
progression in spinocerebellar ataxias (SCAs). SCAs are clinically heterogeneous and the …

We administered a large battery of neuropsychological tests to an heterogeneous cohort of
genetically defined spinocerebellar ataxia (SCA) patients in order to assess their cognitive …

Background The natural history of clinical symptoms in the spinocerebellar ataxias (SCA) s
has been well characterised. However there is little longitudinal data comparing cognitive …