Document Detail


Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP.
MedLine Citation:
PMID:  16401843     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: To examine the relationship between early clinical features, pathologies, and biochemistry of the frontotemporal lobar degenerations (FTLDs), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). METHODS: The authors conducted pathologic reexamination with the most recent immunohistochemistry of all cases diagnosed with FTLD, PSP, and CBD between 1970 and 2004. The authors also reviewed the early clinical features for clinical diagnosis and application of published research criteria. RESULTS: Of 127 cases analyzed, 57 had a pathologic diagnosis of FTLD, 49 PSP, and 21 CBD. Of these, 38 were clinically reclassified as frontal variant frontotemporal dementia (FTD), 13 as progressive non-fluent aphasia (PNFA), 21 as CBD-like, 33 as PSP-like, and 13 with frontotemporal dementia with coexisting motor neuron disease (FTD-MND). The authors were unable to classify nine cases. All cases of FTD-MND were tau-negative and had pathologic evidence of motor neuron degeneration. All cases classified as PSP-like or CBD-like had tau-positive pathology. Of the 13 cases with PNFA, PSP and CBD accounted for almost 70% of the cases, while FTD was almost equally divided between tau-positive and tau-negative diseases. CONCLUSION: Frontotemporal lobar degeneration, corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP) have overlapping clinical features. The prediction of tau-positive pathology from a CBD or PSP-like presentation is good, while the frontotemporal dementia (FTD)-motor neuron disease syndrome almost certainly predicts motor neuron degeneration. Surprisingly, PSP and CBD accounted for most cases classified as progressive non-fluent aphasia. Frontal variant FTD is an unpredictable disease in terms of its biochemistry.
Authors:
K A Josephs; R C Petersen; D S Knopman; B F Boeve; J L Whitwell; J R Duffy; J E Parisi; D W Dickson
Related Documents :
16410743 - Central and extrapontine myelinolysis: then...and now.
440643 - The pathology of female genital tuberculosis. a 31-year study of 1436 cases.
20594383 - Testamentary capacity and delirium.
Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  66     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2006 Jan 
Date Detail:
Created Date:  2006-01-10     Completed Date:  2006-04-20     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  41-8     Citation Subset:  AIM; IM    
Affiliation:
Division of Movement Disorders, Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. josephs.keith@mayo.edu
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Aged
Aphasia, Primary Progressive / diagnosis,  physiopathology
Biological Markers / metabolism
Brain / pathology*,  physiopathology*
Dementia / classification,  diagnosis*,  physiopathology
Diagnosis, Differential
Female
Humans
Immunohistochemistry
Inclusion Bodies / metabolism,  pathology
Male
Middle Aged
Motor Neuron Disease / classification,  diagnosis,  physiopathology
Neurons / metabolism,  pathology
Predictive Value of Tests
Supranuclear Palsy, Progressive / classification,  diagnosis*,  physiopathology
Tauopathies / classification,  diagnosis*,  physiopathology
tau Proteins / metabolism
Grant Support
ID/Acronym/Agency:
P01 AG7216/AG/NIA NIH HHS; P50 AG16574/AG/NIA NIH HHS; R01 AG23195/AG/NIA NIH HHS; UO1 AG06786/AG/NIA NIH HHS
Chemical
Reg. No./Substance:
0/Biological Markers; 0/tau Proteins
Comments/Corrections
Comment In:
Neurology. 2006 Jan 10;66(1):8-9   [PMID:  16401836 ]

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


Previous Document:  Familial Alzheimer disease in Latinos: interaction between APOE, stroke, and estrogen replacement.
Next Document:  Dissociation of neuropathology from severity of dementia in late-onset Alzheimer disease.