title: Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis.
abstract: OBJECTIVE
  Abnormalities on diffusion-weighted images (DWIs) and fluid-attenuated inversion recovery (FLAIR) images are reported in Creutzfeldt-Jakob disease (CJD). To our knowledge, no large study has been conducted to determine the sensitivity and specificity of DWI and FLAIR imaging for diagnosing CJD.
  
  
  METHODS
  Two neuroradiologists, blinded to diagnosis, retrospectively evaluated DWI and FLAIR images from 40 patients with probable or definite CJD and 53 control subjects with other forms of dementia and rated the likelihood of CJD on the basis of the imaging findings.
  
  
  RESULTS
  DWI and FLAIR imaging was 91% sensitive, 95% specific, and 94% accurate for CJD. Interrater reliability was high (kappa = 0.93). Sensitivity was higher for DWI than FLAIR imaging. Abnormalities involved cortex and deep gray matter (striatum and/or thalamus) in 68% of patients with CJD, cortex alone in 24%, and deep gray matter alone in 5%. The most typical and specific patterns were corresponding hyperintensity on both FLAIR images and DWIs confined to the gray matter in the cortex, striatum, medial and/or posterior thalamus, or a combination of these areas. Narrow-window soft-copy review of artifact-free DWIs and FLAIR images and recognition of the normal variation in cortical signal intensity proved critical for successful differentiation of CJD from other dementias.
  
  
  CONCLUSIONS
  Because specific patterns of abnormality on DWI and FLAIR images are highly sensitive and specific for CJD, these sequences should be performed whenever CJD is suspected.
authors.affiliation: Department of Radiology, University of California, San Francisco, 74143, USA.
authors.forename: Geoffrey S
authors.initials: GS
authors.lastname: Young
authors.forename: Michael D
authors.initials: MD
authors.lastname: Geschwind
authors.forename: Nancy J
authors.initials: NJ
authors.lastname: Fischbein
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Martindale
authors.forename: Roland G
authors.initials: RG
authors.lastname: Henry
authors.forename: Songling
authors.initials: S
authors.lastname: Liu
authors.forename: Ying
authors.initials: Y
authors.lastname: Lu
authors.forename: Stephen
authors.initials: S
authors.lastname: Wong
authors.forename: Hong
authors.initials: H
authors.lastname: Liu
authors.forename: Bruce L
authors.initials: BL
authors.lastname: Miller
authors.forename: William P
authors.initials: WP
authors.lastname: Dillon
mesh_terms: D000328:Adult; D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012680:Sensitivity and Specificity