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Alzheimer's disease: report of two autopsy cases with a clinical diagnosis of corticobasal degeneration.
Neuropathology. 2010 Apr; 30(2):140-8.N

Abstract

Alzheimer's disease (AD) is the most common cause of dementia in the elderly. Corticobasal degeneration (CBD) is a rare neurodegenerative disease affecting adults, being characterized clinically by a combination of extrapyramidal signs and focal cortical syndromes. In both diseases, tau deposits are a characteristic neuropathological feature. We report two new patients with autopsy-proven AD, in whom clinical diagnoses of CBD were made during life. The ages of the patients at onset were 52 and 67 years, and the disease durations were 9 and 15 years, respectively. At autopsy, both cases exhibited marked cortical atrophy with evident neuronal loss in the convex areas of the frontal and parietal lobes. Immunohistochemically, AT8-positive neurofibrillary tangles (NFTs) and Abeta-positive senile plaques (SPs) were widespread and abundant in the cerebral cortex (Alzheimer pathology stage VI/C of Braak and Braak), leading us to the final pathological diagnosis of AD. No tau lesions suggestive of CBD were observed, and the deep gray matter areas, including the substantia nigra, were unremarkable (exceptionally, only mild neuronal loss was noted in the putamen in case 2). These findings further strengthen the idea that in AD, neurodegeneration with tau and Abeta deposits may begin in the fronto-parietal neocortical areas, which are often preferentially affected in CBD, earlier than, or as early as the medial temporal lobe, and that extrapyramidal signs, such as rigidity and tremor, can occur in the absence of neuronal loss in the basal ganglia and substantia nigra.

Authors+Show Affiliations

Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan. keniti-o@bri.niigata-u.ac.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

19780981

Citation

Okazaki, Kenichi, et al. "Alzheimer's Disease: Report of Two Autopsy Cases With a Clinical Diagnosis of Corticobasal Degeneration." Neuropathology : Official Journal of the Japanese Society of Neuropathology, vol. 30, no. 2, 2010, pp. 140-8.
Okazaki K, Fu YJ, Nishihira Y, et al. Alzheimer's disease: report of two autopsy cases with a clinical diagnosis of corticobasal degeneration. Neuropathology. 2010;30(2):140-8.
Okazaki, K., Fu, Y. J., Nishihira, Y., Endo, M., Fukushima, T., Ikeuchi, T., Okamoto, K., Onodera, O., Nishizawa, M., & Takahashi, H. (2010). Alzheimer's disease: report of two autopsy cases with a clinical diagnosis of corticobasal degeneration. Neuropathology : Official Journal of the Japanese Society of Neuropathology, 30(2), 140-8. https://doi.org/10.1111/j.1440-1789.2009.01062.x
Okazaki K, et al. Alzheimer's Disease: Report of Two Autopsy Cases With a Clinical Diagnosis of Corticobasal Degeneration. Neuropathology. 2010;30(2):140-8. PubMed PMID: 19780981.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Alzheimer's disease: report of two autopsy cases with a clinical diagnosis of corticobasal degeneration. AU - Okazaki,Kenichi, AU - Fu,Yong-Juan, AU - Nishihira,Yasushi, AU - Endo,Minoru, AU - Fukushima,Takao, AU - Ikeuchi,Takeshi, AU - Okamoto,Kouichirou, AU - Onodera,Osamu, AU - Nishizawa,Masatoyo, AU - Takahashi,Hitoshi, Y1 - 2009/09/22/ PY - 2009/9/29/entrez PY - 2009/9/29/pubmed PY - 2010/8/14/medline SP - 140 EP - 8 JF - Neuropathology : official journal of the Japanese Society of Neuropathology JO - Neuropathology VL - 30 IS - 2 N2 - Alzheimer's disease (AD) is the most common cause of dementia in the elderly. Corticobasal degeneration (CBD) is a rare neurodegenerative disease affecting adults, being characterized clinically by a combination of extrapyramidal signs and focal cortical syndromes. In both diseases, tau deposits are a characteristic neuropathological feature. We report two new patients with autopsy-proven AD, in whom clinical diagnoses of CBD were made during life. The ages of the patients at onset were 52 and 67 years, and the disease durations were 9 and 15 years, respectively. At autopsy, both cases exhibited marked cortical atrophy with evident neuronal loss in the convex areas of the frontal and parietal lobes. Immunohistochemically, AT8-positive neurofibrillary tangles (NFTs) and Abeta-positive senile plaques (SPs) were widespread and abundant in the cerebral cortex (Alzheimer pathology stage VI/C of Braak and Braak), leading us to the final pathological diagnosis of AD. No tau lesions suggestive of CBD were observed, and the deep gray matter areas, including the substantia nigra, were unremarkable (exceptionally, only mild neuronal loss was noted in the putamen in case 2). These findings further strengthen the idea that in AD, neurodegeneration with tau and Abeta deposits may begin in the fronto-parietal neocortical areas, which are often preferentially affected in CBD, earlier than, or as early as the medial temporal lobe, and that extrapyramidal signs, such as rigidity and tremor, can occur in the absence of neuronal loss in the basal ganglia and substantia nigra. SN - 1440-1789 UR - https://www.unboundmedicine.com/medline/citation/19780981/Alzheimer's_disease:_report_of_two_autopsy_cases_with_a_clinical_diagnosis_of_corticobasal_degeneration_ L2 - https://doi.org/10.1111/j.1440-1789.2009.01062.x DB - PRIME DP - Unbound Medicine ER -