Document Detail


Frontotemporal lobar degeneration without lobar atrophy.
MedLine Citation:
PMID:  17101834     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal inclusions (FTLD-U) is the most common form of frontotemporal dementia. Neuronal loss and gliosis in cornu ammonis 1 and the subiculum of the hippocampus are features of hippocampal sclerosis (HpScl), which occurs in many cases of FTLD-U. OBJECTIVE: To determine if there were any clinical or magnetic resonance imaging correlates of HpScl in FTLD-U. DESIGN: We reviewed demographics and clinical features of 24 cases of FTLD-U and subjectively assessed the severity of neuronal loss and frequency of ubiquitin-positive neuronal lesions in the frontal and temporal cortices and the dentate gyrus of the hippocampus. SETTING: Mayo Clinic, Rochester, Minn. Patients Twenty-six cases were identified from the medical records linkage system query that met clinical criteria and had autopsy material available for additional studies. Two cases were excluded from further analysis after pathologic studies revealed coexisting Alzheimer disease, leaving 24 cases included in the study. Cases were subdivided based on the presence or absence of HpScl. MAIN OUTCOME MEASURES: Patterns of gray matter atrophy were assessed in cases of FTLD-U with and without HpScl using voxel-based morphometry. RESULTS: Six of the 24 cases of FTLD-U did not have HpScl. No differences were found in demographic or clinical features, including disease duration, between cases with and without HpScl; however, voxel-based morphometry analysis revealed minimal cortical atrophy in cases without HpScl, which was significantly different from the pattern of moderate to severe frontal and temporal lobe atrophy in FTLD-U with HpScl. This finding was in keeping with histopathologic observations. CONCLUSIONS: Despite similar clinical features, cases of FTLD-U with HpScl differ from those without HpScl with respect to pathologic findings and structural imaging. Specifically, FTLD-U without HpScl showed on average minimal or no cortical atrophy, even at end-stage disease. Consequently, FTLD-U without HpScl does not conform to the proposed FTLD staging scheme, is underrecognized, and may have different genetic and environmental underpinnings.
Authors:
Keith A Josephs; Jennifer L Whitwell; Clifford R Jack; Joseph E Parisi; Dennis W Dickson
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Publication Detail:
Type:  Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of neurology     Volume:  63     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2006 Nov 
Date Detail:
Created Date:  2006-11-14     Completed Date:  2006-12-12     Revised Date:  2007-12-03    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1632-8     Citation Subset:  AIM; IM    
Affiliation:
Department of Neurology, Division of Behavioral Neurology and Movement Disorders, Mayo Clinic, Rochester, MN 55905, USA. josephs.keith@mayo.edu
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MeSH Terms
Descriptor/Qualifier:
Aged
Aged, 80 and over
Brain Mapping
Cell Death / physiology
Dementia / complications*,  pathology*
Female
Hippocampus / pathology
Humans
Immunohistochemistry / methods
Magnetic Resonance Imaging / methods
Male
Middle Aged
Neurons / metabolism*
Pick Disease of the Brain / pathology
Ubiquitin / metabolism*
Grant Support
ID/Acronym/Agency:
AG16574/AG/NIA NIH HHS; HD49078/HD/NICHD NIH HHS; P01-AG03949/AG/NIA NIH HHS; P01-AG14449/AG/NIA NIH HHS; P01-AG17216/AG/NIA NIH HHS; P50-AG16574/AG/NIA NIH HHS; P50-NS40256/NS/NINDS NIH HHS; R01-AG11378/AG/NIA NIH HHS; U01-AG06786/AG/NIA NIH HHS
Chemical
Reg. No./Substance:
0/Ubiquitin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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