Correlating DWI MRI with pathologic and other features of Jakob-Creutzfeldt disease.
Diffusion-weighted (DWI) magnetic resonance imaging (MRI) is a highly sensitive and specific test for diagnosis of sporadic Jakob-Creutzfeldt disease (sCJD); however, the neuropathologic origin of DWI signal abnormalities including other clinical features has not been well defined. We describe a case of sCJD with brain MRI taken 15 days before death, which provided an opportunity to correlate clinical, electroencephalogram, MRI, and neuropathologic findings to better understand which sCJD-specific neuropathologic changes underlie the DWI abnormalities. Clinical findings correlated well with both electroencephalogram and MRI changes. Neuropathologic analysis showed that hyperintensities on DWI MRI correlated best with the vacuolation score (r=0.78, P=0.0005) and PrP(Sc) load (r=0.77; P=0.0006), followed by reactive astrocytic gliosis (r=0.63, P=0.008). This case provides further evidence that DWI abnormalities correlate well with the clinical features and with PrP(Sc) accumulation and vacuolation.
Geschwind
Michael D
MD
Departments of Neurology, University of California,San Francisco, UCSF Memory and Aging Center, Box 1207, SanFrancisco, CA 94143-1207, USA. mgeschwind@memory.ucsf
Potter
Christopher A
CA
Sattavat
Mamta
M
Garcia
Paul A
PA
Rosen
Howard J
HJ
Miller
Bruce L
BL
DeArmond
Stephen J
SJ
D000368:Aged; D001281:Atrial Fibrillation / Q000150:complications; D001921:Brain / Q000473:pathology* / Q000503:physiopathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D020521:Stroke / Q000150:complications