Document Detail

Clinical features differentiating patients with postmortem confirmed progressive supranuclear palsy and corticobasal degeneration.
MedLine Citation:
PMID:  10525996     Owner:  NLM     Status:  MEDLINE    
Progressive supranuclear palsy (PSP) and cortocobasal degeneration (CBD) are often clinically confused with each other because they share a rapid disease progression, parkinsonism that responds poorly or transiently to levodopa therapy, and associated signs (e.g., ocular abnormalities, pyramidal signs and cognitive involvement). To improve the accuracy in diagnosing these disorders, this study examined the clinical features of 51 patients pathologically diagnosed with PSP and CBD. Logistic regression analysis identified two sets of predictors (models) for CBD patients, one consisting of asymmetric parkinsonism, cognitive disturbances at onset and instability and falls at first clinic visit, and the other one of asymmetric parkinsonism, cognitive disturbances at symptom onset and speech disturbances. While PSP patients often had severe postural instability at onset, symmetric parkinsonism, vertical supranuclear gaze palsy, speech and frontal lobe-type features, CBD patients presented with lateralized motor (e.g., parkinsonism, dystonia or myoclonus) and cognitive signs (e.g., ideomotor apraxia, aphasia or alien limb). On the other hand, CBD patients presenting with an alternate phenotype characterized by early severe frontal dementia and bilateral parkinsonism were generally misdiagnosed. PSP patients without vertical supranuclear gaze palsy were misdiagnosed. Recognizing the features which differentiate these disorders and the less obvious disease presentations as well as developing an increased index of suspicion will improve the diagnostic accuracy of these disorders.
I Litvan; D A Grimes; A E Lang; J Jankovic; A McKee; M Verny; K Jellinger; K R Chaudhuri; R K Pearce
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Publication Detail:
Type:  Comparative Study; Journal Article; Review    
Journal Detail:
Title:  Journal of neurology     Volume:  246 Suppl 2     ISSN:  0340-5354     ISO Abbreviation:  J. Neurol.     Publication Date:  1999 Sep 
Date Detail:
Created Date:  1999-12-10     Completed Date:  1999-12-10     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  II1-5     Citation Subset:  IM    
Neuropharmacology Unit Defense & Veteran Head Injury Program, Henry M. Jackson Foundation, Federal Building, Room 714, Bethesda, Maryland 20892-9130, USA, email:
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MeSH Terms
Brain / physiopathology
Cognition Disorders / physiopathology
Parkinsonian Disorders / diagnosis,  physiopathology*
Supranuclear Palsy, Progressive / diagnosis,  physiopathology*

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