| Frontotemporal lobar degeneration with motor neuron disease showing severe and circumscribed atrophy of anterior temporal lobes. | |
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MedLine Citation:
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PMID: 20674934 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Frontotemporal lobar degeneration (FTLD) is characterized by a variety of behavioral and psychiatric symptoms based on the dysfunction of frontal and/or temporal lobes. A 63-year-old Japanese man without a family history of neurological diseases developed progressive symptoms of frontotemporal dementia, followed by motor neuron disease (MND). Brain magnetic resonance images demonstrated severe atrophy in the anterior temporal lobes from early clinical stage. The symptoms got rapidly worsened and the patient died of respiratory failure 1year 8months after the disease onset. A postmortem study revealed severe and circumscribed atrophy in the anterior temporal lobes, and histological examination disclosed marked neuronal loss with many neuronal cytoplasmic inclusions which were immunoreactive for ubiquitin antibodies and phosphorylated TAR DNA-binding protein of 43kDa (TDP-43) antibodies in hippocampal dentate granule cells and amygdalae, as well as a few neuronal cytoplasmic inclusions without dystrophic neurites in the temporal neocortex. This case report showed typical features of FTLD-MND in clinical course and TDP-43 pathology with unusual severity and distribution of cerebral atrophy, suggesting a unique manifestation of FTLD-MND. |
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Authors:
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Hiroya Kuwahara; Kuniaki Tsuchiya; Yukinobu Saito; Zen Kobayashi; Hiroshi Miyazaki; Yoko Izumiyama; Haruhiko Akiyama; Tetsuaki Arai; Hidehiro Mizusawa |
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Publication Detail:
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Type: Journal Article Date: 2010-08-02 |
Journal Detail:
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Title: Journal of the neurological sciences Volume: 297 ISSN: 1878-5883 ISO Abbreviation: J. Neurol. Sci. Publication Date: 2010 Oct |
Date Detail:
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Created Date: 2010-09-13 Completed Date: 2010-12-28 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: Netherlands |
Other Details:
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Languages: eng Pagination: 92-6 Citation Subset: IM |
Copyright Information:
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Copyright 2010 Elsevier B.V. All rights reserved. |
Affiliation:
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Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. h-kuwahara.nuro@tmd.ac.jp |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Asian Continental Ancestry Group Atrophy DNA-Binding Proteins / metabolism Frontotemporal Lobar Degeneration / complications* Humans Magnetic Resonance Imaging / methods Male Melanins / metabolism Middle Aged Motor Neuron Disease / complications* Temporal Lobe / pathology* Ubiquitin / metabolism |
| Chemical | |
Reg. No./Substance:
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0/DNA-Binding Proteins; 0/Melanins; 0/Ubiquitin; 0/protein TDP-43 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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