Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings.
OBJECTIVE
To assess if clinical features, prion protein codon 129, and molecular subtype correlate with MRI basal ganglia hyperintensity in sporadic Creutzfeldt-Jakob disease (CJD).


METHODS
The authors studied 219 patients including 153 confirmed CJD cases for their neurologic symptoms and MRI findings. The MRI was assessed by a blinded investigator for the presence of high signal intensity on T2-weighted images in the basal ganglia.


RESULTS
Patients with basal ganglia high signal on T2-weighted images were more likely to present with rapid progressive dementia in an early stage and shorter disease duration (median 6.7 months and 8.6 months). Surprisingly, among the CJD cases, patients without signal increase of the basal ganglia were shown to have a higher frequency of extrapyramidal disturbances (82% vs 70%). More striking differences were found for symptoms such as depression and sensory disturbances, which were more frequent among cases without signal increase. MRI was more likely to be diagnostic in patients with MV2 molecular subtype.


CONCLUSIONS
Selected clinical and pathologic features correlate with the presence of basal ganglia high signal on T2-weighted MRI in patients with definite or probable CJD.
Meissner
B
B
Department of Neurology, University of Göttingen, Germany.
Körtner
K
K
Bartl
M
M
Jastrow
U
U
Mollenhauer
B
B
Schröter
A
A
Finkenstaedt
M
M
Windl
O
O
Poser
S
S
Kretzschmar
H A
HA
Zerr
I
I
D000328:Adult; D000368:Aged; D000682:Amyloid / Q000235:genetics; D001479:Basal Ganglia / Q000473:pathology*; D001927:Brain Diseases / Q000473:pathology; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000175:diagnosis / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D004252:DNA Mutational Analysis; D003704:Dementia / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D018620:PrPSc Proteins / Q000235:genetics; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors / Q000235:genetics; D012189:Retrospective Studies; D016037:Single-Blind Method; D016019:Survival Analysis
D000682:Amyloid; D003062:Codon; C512656:PRNP protein, human; D018620:PrPSc Proteins; D000072002:Prion Proteins; D011328:Prions; D011498:Protein Precursors