PT - JOURNAL ARTICLE AU - Shinotoh, Hitoshi AU - Shimada, Hitoshi AU - Kokubo, Yasumasa AU - Tagai, Kenji AU - Niwa, Fumitoshi AU - Kitamura, Soichiro AU - Endo, Hironobu AU - Ono, Maiko AU - Kimura, Yasuyuki AU - Hirano, Shigeki AU - Mimuro, Maya AU - Ichise, Masanori AU - Sahara, Naruhiko AU - Zhang, Ming-Rong AU - Suhara, Tetsuya AU - Higuchi, Makoto TI - Tau imaging detects distinctive distribution of tau pathology in ALS/PDC on the Kii Peninsula AID - 10.1212/WNL.0000000000006736 DP - 2019 Jan 08 TA - Neurology PG - e136--e147 VI - 92 IP - 2 4099 - http://n.neurology.org/content/92/2/e136.short 4100 - http://n.neurology.org/content/92/2/e136.full SO - Neurology2019 Jan 08; 92 AB - Objective To characterize the distribution of tau pathology in patients with amyotrophic lateral sclerosis/parkinsonism dementia complex on the Kii Peninsula (Kii ALS/PDC) by tau PET using [11C]PBB3 as ligand.Methods This is a cross-sectional study of 5 patients with ALS/PDC and one asymptomatic participant with a dense family history of ALS/PDC from the Kii Peninsula who took part in this study. All were men, and their age was 76 ± 8 (mean ± SD) years. Thirteen healthy men (69 ± 6 years) participated as healthy controls (HCs). Dynamic PET scans were performed following injection of [11C]PBB3, and parametric PET images were generated by voxel-by-voxel calculation of binding potential (BP*ND) using a multilinear reference tissue model. [11C] Pittsburgh compound B (PiB) PET, MRI, and cognitive tests were also performed.Results A voxel-based comparison of [11C]PBB3 BP*ND illustrated PET-detectable tau deposition in the cerebral cortex and white matter, and pontine basis including the corticospinal tract in Kii ALS/PDC patients compared with HCs (uncorrected p < 0.05). Group-wise volume of interest analysis of [11C]PBB3 BP*ND images showed increased BP*ND in the hippocampus and in frontal and parietal white matters of Kii ALS/PDC patients relative to HCs (p < 0.05, Holm-Sidak multiple comparisons test). BP*ND in frontal, temporal, and parietal gray matters correlated with Mini-Mental State Examination scores in Kii ALS/PDC patients (p < 0.05). All Kii ALS/PDC patients were negative for [11C]PiB (β-amyloid) except one with marginal positivity.Conclusion [11C]PBB3 PET visualized the characteristic topography of tau pathology in Kii ALS/PDC, corresponding to clinical phenotypes of this disease.Aβ=β-amyloid; AD=Alzheimer disease; ALS=amyotrophic lateral sclerosis; [11C]PBB3=2-([1E,3E]-4-[6-([11C]methylamino)pyridin-3-yl]buta-1,3-dienyl)benzo[d]thiazol-6-ol; CDR=Clinical Dementia Rating; FAB=Frontal Assessment Battery; HC=healthy control; MAO-B=monoamine oxidase B; MMSE=Mini-Mental State Examination; NFT=neurofibrillary tangle; NPI=Neuropsychological Inventory; PDC=parkinsonism dementia complex; PiB=Pittsburgh compound B; SOB=sum of boxes; SUVR=standardized uptake value ratio; UPDRS=Unified Parkinson's Disease Rating Scale; VOI=volume of interest