Brain Advance Access originally published online on January 4, 2008
Brain 2008 131(2):438-446; doi:10.1093/brain/awm328
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Effects of disease duration on the clinical features and brain glucose metabolism in patients with mixed type multiple system atrophy
1Department of Neurology, Youngdong Severance Hospital, Yonsei University College of Medicine, Seoul, 2Department of Brain and Bioengineering, KAIST, Daejeon, 3Department of Radiology, Youngdong Severance Hospital, Yonsei University College of Medicine, Seoul, Korea and 4Turku PET Center, University of Turku, Turku, Finland
Correspondence to: Myung Sik Lee, MD, PhD, Professor, Department of Neurology, Youngdong Severance Hospital, 612 Eonjuro, Gangnam-gu, Seoul, Korea E-mail: mslee{at}yuhs.ac
To study the effect of disease duration on the clinical, neuropsychological and [18F]-deoxyglucose (FDG) PET findings in patients with mixed type multiple system atrophy (MSA), this study included 16 controls and 37 mixed-type MSA patients with a shorter than a 3-year history of cerebellar or parkinsonian symptoms. We classified the patients into three groups according to the duration of parkinsonian or cerebellar symptoms (Group I = 
1 year; II = 13–24 months; III = 25–36 months). We performed UPDRS, international cooperative ataxia rating scale (ICARS), and a neuropsychological test battery. We compared the FDG PET findings of each group of patients with controls. Group I patients frequently had memory and frontal executive dysfunction. They showed hypometabolism in the frontal cortex, anterior cerebellar hemisphere and vermis. They had parkinsonian motor deficits, but no basal ganglia hypometabolism. Group II and III patients frequently had multiple domain cognitive impairments, and showed hypometabolism in the frontal and parieto-temporal cortices. Hypometabolism of the bilateral caudate and the left posterolateral putamen was observed in Group II, and whole striatum in Group III. In summary, the cortical hypometabolism begins in the frontal cortex and spreads to the parieto-temporal cortex in MSA. This spreading pattern coincides with the progressive cognitive decline. Early caudate hypometabolism may also contribute to the cognitive impairment. Parkinsonian motor deficits precede putaminal hypometabolism that begins in its posterolateral part. Cerebellar hypometabolism occurs early in the clinical courses and seems to be a relevant metabolic descriptor of cerebellar deficits.
Key Words: multiple system atrophy; cognitive impairment; positron emission tomography
Abbreviations: COWAT, controlled oral word association test; FDG, [18F]-deoxyglucose; FWHM, full-width half-maximum; GCI, glial cytoplasmic inclusion; ICARS, international cooperative ataxia rating scale; IQR, interquartile range; K-BNT, Korean version of the Boston naming test; MSA, multiple system atrophy; PET, positron emission tomography; RCFT, Rey–Osterrieth complex figure test; SVLT, Seoul verbal learning test; TE, echo time; TR, repetition time; UPDRS, unified Parkinson's disease rating scale.
Received September 10, 2007. Revised November 8, 2007. Accepted December 11, 2007.