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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.
Neuropathology 2007; 27(1):21-35N

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+Show Affiliations

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.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

17319280

Citation

Yokota, Osamu, et al. "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." Neuropathology : Official Journal of the Japanese Society of Neuropathology, vol. 27, no. 1, 2007, pp. 21-35.
Yokota O, Tsuchiya K, Uchihara T, et al. 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. Neuropathology. 2007;27(1):21-35.
Yokota, O., Tsuchiya, K., Uchihara, T., Ujike, H., Terada, S., Takahashi, M., ... Kuroda, S. (2007). 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. Neuropathology : Official Journal of the Japanese Society of Neuropathology, 27(1), pp. 21-35.
Yokota O, et al. 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. Neuropathology. 2007;27(1):21-35. PubMed PMID: 17319280.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Yokota,Osamu, AU - Tsuchiya,Kuniaki, AU - Uchihara,Toshiki, AU - Ujike,Hiroshi, AU - Terada,Seishi, AU - Takahashi,Mafuyu, AU - Kimura,Yuji, AU - Ishizu,Hideki, AU - Akiyama,Haruhiko, AU - Kuroda,Shigetoshi, PY - 2007/2/27/pubmed PY - 2007/3/30/medline PY - 2007/2/27/entrez SP - 21 EP - 35 JF - Neuropathology : official journal of the Japanese Society of Neuropathology JO - Neuropathology VL - 27 IS - 1 N2 - 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. SN - 0919-6544 UR - https://www.unboundmedicine.com/medline/citation/17319280/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_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0919-6544&date=2007&volume=27&issue=1&spage=21 DB - PRIME DP - Unbound Medicine ER -