Document Detail

Rapidly progressive neurodegenerative dementias.
MedLine Citation:
PMID:  19204156     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death.
OBJECTIVE: To characterize patients with a neurodegenerative disease and a rapidly progressive course to death.
DESIGN, SETTING, AND PATIENTS: Using a text word search for "rapid" and "dementia" in the same sentence, the Mayo Clinic Medical Records Linkage system was used to identify all patients evaluated between January 1, 2000, and September 30, 2007, with brain autopsy (N = 96) at a tertiary care medical center. Of these 96 patients, we included only those with disease duration of less than 4 years to death and with histological diagnosis of a neurodegenerative disease.
MAIN OUTCOME MEASURES: Rapidly progressive dementia with death sooner than 4 years after onset and pathological diagnosis at our institution of a neurodegenerative disease.
RESULTS: We included 22 patients (10 men). Although 8 cases (36%) had Creutzfeldt-Jakob disease (CJD), the rest had frontotemporal lobar degeneration with motor neuron degeneration (5 cases [23%]), a tauopathy (progressive supranuclear palsy or corticobasal degeneration) (4 cases [18%]), diffuse Lewy body disease (3 cases [14%]), or Alzheimer disease (2 cases [9%]). All of the patients with CJD died 12 months or sooner after onset, whereas the others had an illness duration longer than 12 months. Notably, all of the 3 patients with diffuse Lewy body disease but no others initially experienced a transient postoperative or illness-associated encephalopathy, then relative normality for 2 years, and finally a rapidly progressive dementia and decline to death in 4 to 12 months.
CONCLUSIONS: Based on this cohort, although CJD is the most likely cause of a rapidly progressive neurodegenerative dementia, frontotemporal lobar degeneration with motor neuron degeneration, diffuse Lewy body disease, tauopathies, and Alzheimer disease can also cause a rapidly progressive dementia. If illness duration is beyond 12 months, a non-CJD neurodegenerative disease may be more likely than CJD to be the diagnosis.
Keith A Josephs; J Eric Ahlskog; Joseph E Parisi; Bradley F Boeve; Brian A Crum; Caterina Giannini; Ronald C Petersen
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of neurology     Volume:  66     ISSN:  1538-3687     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-02-10     Completed Date:  2009-04-09     Revised Date:  2014-09-05    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  201-7     Citation Subset:  AIM; IM    
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MeSH Terms
Age of Onset
Aged, 80 and over
Alzheimer Disease / mortality,  pathology,  physiopathology
Brain / pathology*,  physiopathology
Cohort Studies
Creutzfeldt-Jakob Syndrome / mortality,  pathology,  physiopathology
Dementia / mortality*,  pathology*,  physiopathology
Disease Progression
Lewy Body Disease / mortality,  pathology,  physiopathology
Middle Aged
Neurodegenerative Diseases / mortality*,  pathology*,  physiopathology
Retrospective Studies
Survival Rate
Tauopathies / mortality,  pathology,  physiopathology
Grant Support
K12 HD049078/HD/NICHD NIH HHS; K12 HD049078-01/HD/NICHD NIH HHS; K12-HD49078/HD/NICHD NIH HHS; P50 AG016574/AG/NIA NIH HHS; P50 AG016574-10/AG/NIA NIH HHS; P50-AG16574/AG/NIA NIH HHS; U01 AG006786/AG/NIA NIH HHS; U01 AG006786-22/AG/NIA NIH HHS; U01-AG06786/AG/NIA NIH HHS

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