[Unilateral Creutzfeld-Jakob disease: report of a probable case on electroclinical, MRI and biological criteria].
We report the case of a 70-year-old man who developed probable unilateral Creutzfeldt-Jakob disease. Clinically, he presented with right hemiparesis, progressive aphasia, temporospatial disorientation and cerebellar ataxia and later on, myoclonia. The MRI showed a hypersignal from the left caudate in DWI with decreased ADC. Repeated electroencephalograms showed a slow background rhythm in the left hemisphere with superimposed periodic, biphasic and triphasic sharp-wave complexes in the left temporal region. Death occurred after 5weeks. Although exceptional, unilateral Creutzfeldt-Jakob disease was retained as possible.
Service de neurologie, Clermont université, université d'Auvergne, CHU Gabriel-Montpied, place Henri-Dunant, 63000 Clermont-Ferrand, France. xavier.moisset@gmail.com
X
X
Moisset
N
N
Vitello
C
C
Cornut-Chauvinc
F
F
Taithe
E
E
Dionet
M
M
Lauxerois
N
N
Guy
P
P
Clavelou
D000368:Aged; D001037:Aphasia / Q000209:etiology; D002421:Caudate Nucleus / Q000473:pathology; D002524:Cerebellar Ataxia / Q000209:etiology; D003221:Confusion / Q000209:etiology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology; D038524:Diffusion Magnetic Resonance Imaging; D018450:Disease Progression; D004292:Dominance, Cerebral; D004569:Electroencephalography; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D010291:Paresis / Q000209:etiology