Representative images of spatially normalized ratios using study-specific templates of normal controls (A) and patients with crossed cerebellar diaschisis (B), multiple system atrophy of the cerebellar type (C), Spinocerebellar Ataxia (SCA) type 2 (D), and SCA type 6 (E).

<p>Representative images of spatially normalized ratios using study-specific templates of normal controls (A) and patients with crossed cerebellar diaschisis (B), multiple system atrophy of the cerebellar type (C), Spinocerebellar Ataxia (SCA) type 2 (D), and SCA type 6 (E).</p

Demographic features of the study groups.

<p>Demographic features of the study groups.</p

Different subregional metabolism patterns in patients with cerebellar ataxia by ¹⁸F-fluorodeoxyglucose positron emission tomography

<div><p>We evaluated cerebellar subregional metabolic alterations in patients with cerebellar ataxia, a representative disease involving the spinocerebellum. We retrospectively analyzed ¹⁸F-fluorodeoxyglucose positron emission tomography (¹⁸F-FDG PET) images in 44 patients with multiple system atrophy of the cerebellar type (MSA-C), 9 patients with spinocerebellar ataxia (SCA) type 2, and 14 patients with SCA type 6 and compared with 15 patients with crossed cerebellar diaschisis (CCD) and 89 normal controls. Cerebellar subregional metabolism was assessed using 13 cerebellar subregions (bilateral anterior lobes [ANT], superior/mid/inferior posterior lobes [SUPP/MIDP/INFP], dentate nucleus [DN], anterior vermis [ANTV], and superior/inferior posterior vermis [SUPV/INFV]) to determine FDG uptake ratios. MSA-C and SCA type 2 showed severely decreased metabolic ratios in all cerebellar subregions compared to normal controls (ANT, 0.58 ± 0.08 and 0.50 ± 0.06 vs. 0.82 ± 0.07, respectively, <i>p</i> < 0.001). SCA type 6 showed lower metabolic ratios in almost all cerebellar subregions (ANT, 0.57 ± 0.06, <i>p</i> < 0.001) except INFV. Anterior-posterior lobe ratio measurements revealed that SCA type 2 <b>(</b>Right, 0.81 ± 0.05 vs. 0.88 ± 0.04, <i>p</i> < 0.001; Left, 0.83 ± 0.05 vs. 0.88 ± 0.04, <i>p</i> = 0.003) and SCA type 6 (Right, 0.72 ± 0.05 vs. 0.88 ± 0.04, <i>p <</i> 0.001; Left, 0.72 ± 0.05 vs. 0.88 ± 0.04, <i>p</i> < 0.001) showed preferential hypometabolism in the anterior lobe compared to normal controls, which was not observed in CCD and MSA-C. Asymmetric indices were higher in CCD and MSA-C than in normal controls (<i>p</i> < 0.001), whereas such differences were not found in SCA types 2 and 6. In summary, quantitative analysis of cerebellar subregional metabolism ratios revealed preferential involvement of the anterior lobe, corresponding to the spinocerebellum, in patients with cerebellar ataxia, whereas patients with CCD and MSA-C exhibited more asymmetric hypometabolism in the posterior lobe.</p></div

Volume of Interest (VOI) template on the cerebellum of a normal sagittal ¹⁸F-FDG PET image includes 13 VOIs in bilateral anterior cortex, superior/mid/inferior posterior cortex, dentate nuclei (A), anterior vermis, and superior/inferior posterior vermis (B).

<p>Volume of Interest (VOI) template on the cerebellum of a normal sagittal ¹⁸F-FDG PET image includes 13 VOIs in bilateral anterior cortex, superior/mid/inferior posterior cortex, dentate nuclei (A), anterior vermis, and superior/inferior posterior vermis (B).</p

Coronal, sagittal, and transverse statistical parametric mapping images in patients with ataxia compared to normal controls (<i>p</i> < 0.001, FWE).

<p>Patients with multiple system atrophy of the cerebellar type (MSA-C, A) and spinocerebellar ataxia (SCA) type 2 (B) show decreased metabolism of the entire cerebellum. Patients with SCA type 6 (C) show decreased metabolism mainly in the anterior and superior posterior lobes of the cerebellum.</p

Anterior-posterior lobe ratios in cerebellar cortex and vermis, asymmetric indices of cortex and dentate nuclei, and their correlation with disease duration in controls, patients with Crossed Cerebellar Diaschisis (CCD), Multiple System Atrophy (MSA), and Spinocerebellar Atrophy (SCA) type 2 and type 6.

<p>Anterior-posterior lobe ratios in cerebellar cortex and vermis, asymmetric indices of cortex and dentate nuclei, and their correlation with disease duration in controls, patients with Crossed Cerebellar Diaschisis (CCD), Multiple System Atrophy (MSA), and Spinocerebellar Atrophy (SCA) type 2 and type 6.</p

Striatofrontal Deafferentiation in MSA-P: Evaluation with [¹⁸F]FDG Brain PET

<div><p>Background</p><p>Although cognitive impairment is not a consistent feature of multiple system atrophy (MSA), increasing evidence suggests that cognitive impairment is common in MSA with predominant parkinsonism (MSA-P). It is assumed that the cognitive impairment in MSA-P is caused by the striatal dysfunction and disruption of striatofrontal connections. The aim of this study was to evaluate the relationship between regional glucose metabolism in the frontal cortex and striatum in patients with MSA-P using [¹⁸F]FDG brain PET.</p><p>Methods</p><p>Twenty-nine patients with MSA-P and 28 healthy controls underwent [¹⁸F]FDG brain PET scan. The [¹⁸F]FDG brain PET images were semiquantitatively analyzed on the basis of a template in standard space. The regional glucose metabolism of the cerebral cortex and striatum were compared between MSA-P and healthy control groups. The correlations between age, symptom duration, H&Y stage, UPDRS III score, MMSE score, and glucose metabolism in the cerebellum and striatum to glucose metabolism in the frontal cortex were evaluated by multivariate analysis.</p><p>Results</p><p>The glucose metabolism in the frontal cortex and striatum in MSA-P patients were significantly lower than those in healthy controls. Glucose metabolism in the striatum was the most powerful determinant of glucose metabolism in the frontal cortex in MSA-P. Only age and glucose metabolism in the cerebellum were independent variables affecting the glucose metabolism in the frontal cortex in healthy controls.</p><p>Conclusion</p><p>The decrease in frontal glucose metabolism in MSA-P is related to the decrease in striatal glucose metabolism. This result provided evidence of striatofrontal deafferentiation in patients with MSA-P.</p></div

Result of linear regression analysis for determinants of frontal glucose metabolism in MSA-P.

<p>Result of linear regression analysis for determinants of frontal glucose metabolism in MSA-P.</p

Relationships between left frontal glucose metabolism, and age, cerebellar and striatal glucose metabolism in healthy control.

<p>Relationships between left frontal glucose metabolism, and age, cerebellar and striatal glucose metabolism in healthy control.</p

Comparison of metabolic ratios between healthy control and MSA-P.

<p>Comparison of metabolic ratios between healthy control and MSA-P.</p