PMC5852964_01
a Sagittal brain MRI (T2-weighted) disclosing gross and diffuse atrophy involving the cerebellum (A). Coronal brain MRI (T1-weighted) showing symmetrical atrophy of cerebellum and parietal cortex (B). Axial brain MRI (FLAIR) showing preservation of basal ganglia (C). b DAT-SPECT with I-123 ioflupane showed globally normal standardized binding ratios with only mild reduction of the putamen-to-caudate ratio in the left hemisphere (0.64). Semi quantification was achieved through the Basal ganglia software V2 (Calvini et al.,). c 18F- FDG PET showed severely decreased uptake in both cerebellar hemispheres (A) and moderate decreased uptake in the two putamen, mainly in the left hemisphere (B). No cortical uptake deficit was found (B, C). Hot colors (white, red, yellow) report higher uptake than cold colors (green, blue)
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PMC5852964_01_40673_2018_86_Fig1_HTML.jpg
Fig1
Brain MRI showed advanced cerebellar atrophy, involving both hemispheres and vermis, with brainstem sparing (Fig. 1a). Brain single photon emission computed tomography (SPECT) using the Dopamine Transporter (DAT) ligand I-123 Ioflupane (DAT-SPECT) showed mild reduced uptake in left posterior putamen but was otherwise normal (Fig. 1b). F-18 Fluorodeoxyglucose positron emission tomography (FDG-PET) showed severely decreased glucose metabolism of both cerebellar hemispheres and putamen (Fig. 1c).