Document Detail

Frontotemporal dementia with co-occurrence of astrocytic plaques and tufted astrocytes, and severe degeneration of the cerebral white matter: a variant of corticobasal degeneration?
MedLine Citation:
PMID:  15841415     Owner:  NLM     Status:  MEDLINE    
We report two patients who exhibited frontotemporal dementia (FTD) with unusual neuropathological features. The ages of the patients at death were 65 and 67 years, the disease durations were 6 and 5 years, and the clinical diagnoses were Pick's disease and corticobasal degeneration (CBD), respectively. At autopsy, both cases exhibited neuropathological findings compatible with those of CBD, including atrophy of the frontal and parietal lobes, neuronal loss and gliosis in the cortical and subcortical regions, and presence of cortical ballooned neurons and astrocytic plaques (APs). In both cases, immunoblotting of insoluble tau exhibited the pattern of selective accumulation of four-repeat tau, a finding that is also compatible with CBD. However, severe degeneration was evident in the frontal and parietal white matter in both cases. Moreover, a striking finding was the widespread presence in the affected cortex of tufted astrocytes (TAs), which are characteristic of progressive supranuclear palsy (PSP). Neither co-occurrence of APs and TAs nor severe degeneration of the cerebral white matter is a feature of either CBD or PSP. No mutations were found in the tau gene in either case. In conclusion, the possibility that these two cases represent a new neuropathological phenotype of non-familial FTD rather than simply a variant of CBD cannot be completely excluded.
Chun-Feng Tan; Yue-Shan Piao; Akiyoshi Kakita; Mitsunori Yamada; Hiroki Takano; Masaharu Tanaka; Atsushi Mano; Kunihiko Makino; Masatoyo Nishizawa; Koichi Wakabayashi; Hitoshi Takahashi
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't     Date:  2004-11-12
Journal Detail:
Title:  Acta neuropathologica     Volume:  109     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  2005 Mar 
Date Detail:
Created Date:  2005-04-20     Completed Date:  2005-09-01     Revised Date:  2007-11-09    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  329-38     Citation Subset:  IM    
Department of Pathology, Brain Research Institute, University of Niigata, 1-757 Asahimachi, 951-8585, Niigata, Japan.
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MeSH Terms
Amyloid beta-Protein / metabolism
Astrocytes / metabolism,  pathology*
Atrophy / pathology
Basal Ganglia Diseases / complications,  metabolism,  pathology*
Blotting, Western / methods
Dementia / complications,  metabolism,  pathology*
Frontal Lobe / pathology*
Glial Fibrillary Acidic Protein / metabolism
Magnetic Resonance Imaging / methods
Middle Aged
Nerve Tissue Proteins / metabolism
Neurons / pathology
Pick Disease of the Brain / complications,  metabolism,  pathology*
Senile Plaques / pathology*
tau Proteins / genetics,  metabolism
Reg. No./Substance:
0/Amyloid beta-Protein; 0/Glial Fibrillary Acidic Protein; 0/Nerve Tissue Proteins; 0/Synucleins; 0/Tropanes; 0/tau Proteins; 87-00-3/homatropine

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