Introduction 4 17 19 25 28 30 4 11 27 28 28 28 4 28 Materials and methods Selection of subjects and the clinical assessment 1 33 4 28 Fig. 1 a b 4 28 c 28  29 9 Neuropathological assessment 1–140 4 28 1 6 28 Results Assessment of clinical data The 226 αS-positive subjects examined included 114 (50%) patients with a clinical diagnosis of a neurodegenerative disorder, 15 (7%) patients with other neurological disorders and 97 (43%) individuals in whom the clinical information indicated that they had no neurological impairment. The mean age at death was 77 ± 0.6 years, ranging from 44 to 98 years and the gender was rather evenly distributed (108 females/118 males). Frequency of αS-immunoreactivity in the most vulnerable anatomical regions The most frequently affected regions in the 226 αS-positive subjects were dmV (197/223) and SN (197/225) where the αS-IR was seen in 88% of the analyzed subjects. The LC was affected in 81% (181/223) of subjects, making this area the second most vulnerable nuclei. The large neurons in the nbM were αS-immunopositive in 78% of cases (171/219), whereas the involvement of AC, particularly the cortico-medial nuclear group, was seen in 73% (162/223) of subjects. Staging of Parkinson disease-related αS pathology according to Braak and the frequency of dementia and extrapyramidal symptoms 4 28 1 Table 1 Applicability of Braak staging and the incidence of dementia and extrapyramidal signs (EPS) in each stage  Categorization of the distribution of αS pathology following the recommendations by the consortium on DLB international workshop, i.e. McKeith’s categorization, and the frequency of dementia and extrapyramidal symptoms 2 Table 2 Applicability of neuropathological categorization as recommended by the consortium on DLB international workshop, i.e. McKeiths categorization and the incidence of dementia and extrapyramidal signs (EPS) in each category  2 28 3 Table 3 Assessment of likelihood that the pathological findings are associated with a DLB clinical syndrome Category of Lewy body type pathology Alzheimer type pathology Braak stage 0-II Braak stage III-IV Braak stage V-VI  n  n    n    n    n  n    n    n    n  n    n    n   Atypical cases i.e. cases that could not be classified as recommended 4 Table 4 Topographic distribution of αS immunoreactive lesions in the 39 atypical cases, i.e. not classifiable following current recommendations ID Age Sex EPS Dem AD stage Topographical distribution of αS-immunoreactive structures dmV RN LC SN nbM AC TrEnt Cx CA2 Temp-Occ Ins Cx CG TCx FCx PCx 1 79 F No Yes 4 0 0 0 0 0 4 1 0 0 0 0 0 0 0 2 62 F No Yes 6 0 0 0 0 0 4 2 0 1 0 0 0 0 0 3 74 F No Yes 2 0 0 0 0 + 4 2 0 2 0 1 1 0 0 4 79 F Yes Yes 6 0 0 0 0 + 3 0 0 0 1 1 1 0 0 5 86 F No No 4 0 0 0 + 0 0 0 0 0 0 0 0 0 0 6 44 M No No 0 0 0 0 + 0 0 0 0 0 0 0 0 0 0 7 68 M No No 1 0 0 0 + 0 0 0 0 0 0 0 0 0 0 8 83 M No No 2 0 0 0 + 0 0 0 0 0 0 0 1 0 0 9 83 M No No 1 0 + 0 + 0 0 0 0 0 0 0 0 0 0 10 75 F No No 0 0 0 0 + + 0 0 0 0 0 0 0 0 0 11 76 M No No 1 0 0 0 + + 0 0 0 0 0 0 2 0 0 12 70 M No No 0 0 0 0 + + 0 0 0 0 0 0 0 0 0 13 77 F No No 2 0 0 0 + + 2 1 0 0 0 0 0 0 0 14 73 M Yes Yes 2 0 0 0 ++ + 0 0 0 0 0 0 1 0 0 15 71 F No Yes 6 0 0 0 +++ + 0 0 0 1 1 1 1 0 0 16 79 F No Yes 5 0 0 0 + 0 3 3 0 0 1 0 0 0 0 17 87 F No Yes 2 0 0 0 + + 4 na na 0 0 0 0 0 0 18 74 F No Yes 6 0 0 0 + na 4 na + 1 0 0 0 0 0 19 62 M Yes Yes 0 0 0 + + 0 0 0 0 0 0 0 0 0 0 20 79 M No Yes 0 0 0 + + + 0 0 0 0 0 0 0 0 0 21 84 F No Yes 2 0 0 + + ++ 0 0 0 0 0 0 0 0 0 22 85 F No No 0 0 0 + + + 1 1 na na 0 0 0 0 0 23 82 F No Yes 3 0 0 + + ++ na 2 0 0 1 0 0 0 0 24 58 M No No 0 0 0 + +++ ++ 0 0 0 0 0 0 0 0 0 25 84 M No No 3 0 0 + +++ + 3 2 0 1 0 2 0 0 0 26 76 M No No 1 + 0 0 + 0 0 0 0 0 0 0 0 0 0 27 87 F No No 1 + 0 0 + + 0 0 0 0 na 0 0 0 0 28 78 M No No 1 + 0 0 + + 2 2 0 0 0 0 0 0 0 29 84 F No Yes 5 + 0 0 + 0 1 na + 1 0 0 0 0 0 30 75 F Yes Yes 5 ++ 0 0 + 0 1 0 0 0 0 0 0 0 0 31 60 F No No 0 +++ 0 0 + 0 0 0 0 0 0 0 0 0 0 32 80 M No Yes 6 + 0 0 ++ 0 0 0 0 0 0 1 0 0 0 33 69 M No No 1 ++ + 0 ++ ++ 1 1 0 0 0 1 0 0 0 34 80 M Yes Yes 5 ++ 0 0 ++ + 3 2 + 1 1 1 0 0 0 35 86 F No Yes 6 + + 0 + 0 4 4 + 3 0 0 1 0 0 36 64 F No Yes 6 + 0 0 + na 4 na + na 0 0 1 0 0 37 74 F No Yes 6 + 0 0 + + 4 4 0 na 0 1 2 0 0 38 78 M No Yes 5 + 0 0 + + 3 3 + 1 2 1 1 0 0 39 89 M No No 2 + 0 0 + + 2 2 0 0 0 1 1 1 0 EPS Dem AD stage dmV RN LC SN nbM AC TrEntCx CA2 Temp-Occ Ins Cx CG TCx FCx PCx na 1 2 3 4 + ++ +++ + ++ +++ Discussion 4 28 4 5 5 4 5 5 27 4 28 1 18 21 26 8 10 24 32 33 28 2 4 11 16 22 35 4 11 4 20 4 12 6 35 23 35 13 3 14 34 7 15 36 31 34