Fig. 1. Pedigree of the patient's family and brain MR images of the cousin of the patient and MR and Pittsburgh compound-B (PiB) positron emission tomography (PET) images of the patient. (A) Pedigree of the patient. I-2: grandmother of the patient, dementia; III-2: cousin of the patient, late onset cerebellar ataxia; III-10: the patient, dementia, spastic paraparesis, cerebellar ataxia. (B) Fluid attenuated inversion recovery (FLAIR) MR images of the cousin show severe ponto-medullo-cerebellar atrophy without atrophy in the medial temporal lobes. (C) T2-weighted axial MR images of the patient (III-10 in the pedigree) showing atrophy bilaterally of the medial temporal lobes and prominent volume loss in the cerebellum with enlargement of the fourth ventricle. (D) Increased amyloid deposition in the bilateral striatum, frontal and posterior cingulate cortices, and precuneus, but not in the cerebellum.
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