title: Clinical overlap between Jakob-Creutzfeldt disease and Lewy body disease.
abstract: OBJECTIVE
  Sporadic Jakob-Creutzfeldt disease (sCJD) and dementia with Lewy bodies (DLB) have overlapping clinical symptoms that can lead to their misdiagnosis. We delineated the clinical overlap between sCJD and DLB, and assessed the value of magnetic resonance imaging (MRI) to differentiate between them.
  
  
  METHODS
  Medical records, MRI, electroencephalogram (EEG) and cerebrospinal fluid (CSF) were reviewed from 56 sCJD and 30 DLB subjects.
  
  
  RESULTS
  46% of sCJD subjects met probable DLB criteria and 40% of DLB subjects met probable CJD criteria. A greater proportion of sCJD subjects had cerebellar signs (66% vs. 10%, p<0.001), myoclonus (64% vs. 30%, p=0.002), and visual symptoms (other than hallucinations) (61% vs. 7%, p<0.001), whereas more DLB subjects had hallucinations (70% vs. 39%, p=0.007) and fluctuations (57% vs. 23%, p=0.002). Cortical and/or basal ganglia MRI diffusion weighted imaging hyperintensities consistent with sCJD were seen in 96% of sCJD subjects but in none with DLB. Logistic regression in sCJD revealed that those meeting probable DLB criteria were more likely to have occipital lobe involvement on MRI (OR 1.4, p=0.058, model p=0.022). Parietal lobe involvement on MRI was a predictor of "Other Focal Cortical signs" (OR 1.9, p=0.021). EEG and CSF assessments lacked sensitivity for sCJD as 48% of sCJD patients had a negative EEG; 67% of the 36 sCJD patents with a CSF evaluation had a negative or inconclusive 14-3-3 result. Too few DLB patients had EEG or CSF to assess their utility.
  
  
  CONCLUSIONS
  Sporadic CJD and DLB have significant symptom overlap. MRI helps differentiate these diseases and is related to the signs/symptoms observed in sCJD.
authors.affiliation: Department of Neurology, University of California, San Francisco, California, USA.
authors.forename: Maria Carmela
authors.initials: MC
authors.lastname: Tartaglia
authors.forename: David Y
authors.initials: DY
authors.lastname: Johnson
authors.forename: Julie Ngoc
authors.initials: JN
authors.lastname: Thai
authors.forename: Tatiana
authors.initials: T
authors.lastname: Cattaruzza
authors.forename: Katherine
authors.initials: K
authors.lastname: Wong
authors.forename: Paul
authors.initials: P
authors.lastname: Garcia
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Dearmond
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Miller
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology*; D015331:Cohort Studies; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000503:physiopathology*; D004569:Electroencephalography / Q000379:methods; D005260:Female; D006801:Humans; D020961:Lewy Body Disease / Q000175:diagnosis* / Q000503:physiopathology*; D016015:Logistic Models; D008279:Magnetic Resonance Imaging; D008297:Male; D008609:Mental Status Schedule; D008875:Middle Aged; D012189:Retrospective Studies