Cognitive Impairment in ARCA-1, a Newly Discovered Pure Cerebellar Ataxia Syndrome |
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Authors: | Jr" target="_blank">Robert LaforceJr James P Buteau Jean-Pierre Bouchard Guy A Rouleau Rémi W Bouchard Nicolas Dupré |
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Institution: | 1.Département des Sciences Neurologiques Centre Hospitalier Affilié (Enfant-Jésus),Université Laval,Laval,Canada;2.Centre d’excellence en neuromique de l’Université de Montréal, Centre de Recherche du CHUM (Notre-Dame), Faculté de médecine,Université de Montréal,Montréal,Canada |
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Abstract: | Cerebellar contribution to non-motor functions has been supported by several animal, human and functional neuroimaging studies.
Which cognitive skills and to what extent the cerebrocerebellar loops contribute remain unclear, however. Among other reasons,
this may be explained by the fact that authors have studied patients with extracerebellar lesions. The goal of this study
was to explore the role of the cerebellum in cognition and affect in patients with autosomal recessive cerebellar ataxia type
1 (ARCA-1), a newly described inherited cerebellar disease characterised by middle-age onset of ataxia as well as pure, severe
and diffuse cerebellar atrophy. To this end, the performance of 21 ARCA-1 patients was compared to that of 21 normal controls
paired for age and education on a 3-h battery of attention, executive, visuospatial and memory skills. Results indicated similar
IQ, naming and declarative memory abilities between groups. ARCA-1 patients showed significant deficits in attention (attention
span, speed of information processing, sustained attention), verbal working memory and visuospatial/visuoconstructional skills
(3-D drawings, copy of a complex figure). Functional brain imaging in a subset of patients showed diffuse severe cerebellar
hypometabolism associated with a small area of right parietal hypometabolism. None of the patients presented a significant
affective syndrome. Correlational analyses suggested that cognitive deficits could not be explained by the severity of motor
deficits, duration of disease or mood. Altogether, this study confirms that pure cerebellar damage as seen in ARCA-1 is associated
with significant cognitive impairments but not with psychiatric comorbidity. These deficits are correlated with an overall
moderate impact on patient’s autonomy. Our data favour an indirect participation of the dorsolateral prefrontal and posterior
parietal cortical areas to the cerebrocerebellar circuit. |
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