Executive Dysfunction in Spinocerebellar Ataxia Type 1

Abstract

Fourteen patients with spinocerebellar ataxia 1 (SCA1) and 11 controls with similar mean age and IQ estimates were submitted to a neuropsychological test battery comprising tests for IQ, attention, verbal and visuospatial memory as well as executive functions. Neuropsychological assessment yielded verbal memory and executive dysfunction while tests of visuospatial memory and attention were not significantly impaired in SCA1 as compared to controls. Test performance was neither related to the repeat length, the age of onset nor the disease duration. The profile of cognitive impairment in SCA1 with prominent executive dysfunction corresponds to the concept of ‘frontal-subcortical’ dementia that is likely to be contingent upon disruption of a cerebrocerebellar circuitry that consists of afferent and efferent connections between the prefrontal cortex and the cerebellum.

Prefrontal hypoperfusion and cognitive dysfunction correlates in spinocerebellar ataxia type 6

“…In this study, we reveal visual memory deficits, impairments of verbal fluency, and executive dysfunction in SCA6 patients, which are similar to those described in patients with spinocerebellar ataxia type 1 [23], type 2 [24,25], and Machado-Joseph disease [26,27]. Cognitive dysfunction in these diseases is considered to be derived from the disruption of the cortico-cerebellar loop [23,24,26,27].…”

“…Cognitive dysfunction in these diseases is considered to be derived from the disruption of the cortico-cerebellar loop [23,24,26,27]. However, these diseases have extracerebellar involvement with degeneration of frontal lobes, thalamus, brainstem or basal ganglia, [28,29].…”

Cognitive Impairment in Spinocerebellar Degeneration

“…Tang et al [10] found that clinically, dementia was less frequent in SCA1 than in the other SCAs. The first comprehensive study of cognitive function in genetically confirmed SCA1 was described by Bürk et al [11]. They examined 14 patients with SCA1 using a neuropsychological test battery.…”

The spinocerebellar ataxias: Order emerges from chaos

“…Frank dementia is occasionally observed, but more subtle cognitive abnormalities are probably common, if not universal. Executive dysfunction is especially notable [73,74] and may cause substantial impairment yet escape clinical attention during routine neurologic evaluations. Psychiatric disorders, including affective syndromes, personality change, and psychotic syndromes, may be present in as many as 80% of individuals with SCA [75••].…”