Prefrontal hypoperfusion and cognitive dysfunction correlates in spinocerebellar ataxia type 6

• Published in: Journal of the neurological sciences 2008-08, Vol.271 (1), p.68-74
• Main Authors: Kawai, Y, Suenaga, M, Watanabe, H, Ito, M, Kato, K, Kato, T, Ito, K, Tanaka, F, Sobue, G
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Brain Mapping; Case-Control Studies; Cerebellum; Cerebral blood flow; Cognition Disorders - diagnostic imaging; Cognition Disorders - etiology; Cognition Disorders - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Higher nervous activity; Humans; Medical sciences; Neurology; Neuropsychological Tests; Prefrontal cortex; Prefrontal Cortex - diagnostic imaging; Prefrontal Cortex - pathology; Severity of Illness Index; SPECT; Spinocerebellar ataxia type 6; Spinocerebellar Ataxias - complications; Spinocerebellar Ataxias - diagnostic imaging; Spinocerebellar Ataxias - pathology; Tomography, Emission-Computed, Single-Photon - methods
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2008.03.018

Abstract Objective The aim of this study is to evaluate the correlation between brain perfusion and cognitive dysfunction in spinocerebellar ataxia type 6 (SCA6) patients. Methods Thirteen genetically confirmed SCA6 patients and 21 age- and education-matched control subjects were subjected to single photon emission computed tomography (SPECT) and neuropsychological tests. Brain perfusion was examined with SPECT analysis, while general cognition, verbal and visual memory, attention, visuospatial ability, language, executive function, depression, and anxiety were examined with the neuropsychological tests. Results SCA6 patients showed prefrontal hypoperfusion, and impairments of visual memory, verbal fluency, and executive function compared to control subjects. These neuropsychological impairments in SCA6 patients were significantly correlated with a decrease in prefrontal perfusion. This relation was not correlated to other factors, such as age, education and severity of cerebellar ataxia, which are possible relevant factors associated with cognitive performance. Conclusions SCA6 patients have mild cognitive impairment, and correlating prefrontal hypoperfusion. These results indicate cognitive impairment in SCA6 patients resulting from prefrontal hypoperfusion.