title: Lewy body variant of Alzheimer's disease or cerebral type lewy body disease? Two autopsy cases of presenile onset with minimal involvement of the brainstem.
abstract: Lewy bodies (LB) usually extend from the brainstem to the cerebrum in patients with Parkinson's disease. However, whether the patterns of progression of LB and neuronal loss in Parkinson's disease are identical to those in other Lewy body diseases (LBD) remains unclear. In addition, pathological data on the autonomic nervous system involvement in LBD are limited. We present here the clinicopathological characteristics of two autopsy cases with both Alzheimer's disease and dementia with Lewy bodies (DLB), possibly diagnosed as having Lewy body variant of Alzheimer's disease (LBV/AD). Our patients presented clinically with dementia without parkinsonism. Histopathologically, phosphorylated alpha-synuclein-positive LB and Lewy neurites were abundant in the limbic system, especially in the amygdala, and to a lesser degree, in the neocortex, including the primary motor cortex. The amygdala was also most severely affected by neuronal loss, and the other limbic areas and neocortex were affected to a lesser degree. Despite the existence of a small number of LB and many Lewy neurites, neurons in the brainstem nuclei were relatively well preserved. The Braak stages of concurrent neurofibrillary changes and senile plaques were stage V and C, respectively, in both cases. Tyrosine hydroxylase-positive nerve fibers were relatively well spared in one case examined compared with Parkinson's disease cases. Furthermore, many Lewy neurites immunopositive for phosphorylated a-synuclein were found in the nerve fascicles of the epicardium in one case examined and in Parkinson's disease cases to a lesser degree. These findings suggest that: (i) in at least some LBV/AD cases, the amygdala develops neuronal loss and Lewy-related pathology prior to the brainstem nuclei; and (ii) the depletion of nerves in the heart tissue of LBV/AD is not necessarily complete despite the development of Lewy-related pathology.
authors.affiliation: Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama 700-8558, Japan. oyokota1@yahoo.co.jp
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokota
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Toshiki
authors.initials: T
authors.lastname: Uchihara
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ujike
authors.forename: Seishi
authors.initials: S
authors.lastname: Terada
authors.forename: Mafuyu
authors.initials: M
authors.lastname: Takahashi
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kimura
authors.forename: Hideki
authors.initials: H
authors.lastname: Ishizu
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.forename: Shigetoshi
authors.initials: S
authors.lastname: Kuroda
mesh_terms: D017668:Age of Onset; D000544:Alzheimer Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D001057:Apolipoproteins E / Q000235:genetics; D001344:Autopsy; D001933:Brain Stem / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology*; D003704:Dementia / Q000209:etiology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D016631:Lewy Bodies / Q000473:pathology; D020961:Lewy Body Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D016874:Neurofibrillary Tangles / Q000473:pathology; D058225:Plaque, Amyloid / Q000473:pathology; D016133:Polymerase Chain Reaction; D012150:Polymorphism, Restriction Fragment Length; D053764:Presenilin-1 / Q000235:genetics; D051844:alpha-Synuclein / Q000378:metabolism
chemical_list: D001057:Apolipoproteins E; D053764:Presenilin-1; D051844:alpha-Synuclein