Lateralized clinical and diffusion-weighted MRI abnormalities in a probable case of sporadic Creutzfeldt-Jakob disease.
The usual clinical profile of sporadic Creutzfeldt-Jakob disease (sCJD) is subacute dementia, motor dysfunction and myoclonus. Occasionally, some patients present atypical clinical features. We report a case of probable sCJD in a 73-year-old man with a rapidly progressive lateralized neurologic dysfunction of the left hemisphere. In a few weeks the clinical picture deteriorated dramatically to akinetic mutism and myoclonus. The 14-3-3 protein was positive in the cerebrospinal fluid. Diffusion-weighted (DWI) magnetic resonance imaging (MRI) revealed increased signal in the left cortical ribbon and deep gray matter corresponding to the clinical lateralization. He died 9 weeks after onset, autopsy was not performed. This case illustrates the correlation between the lateralized clinical and DWI MRI abnormalities in sCJD.
Mourand
Isabelle
I
Service de Neurologie, Centre Hospitalier du Mans, Le Mans, France. i-mourand@chu-montpellier
Grandiere-Perez
Lucia
L
Joyes
Béatrice
B
Legout
Alain
A
D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000368:Aged; D002540:Cerebral Cortex / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000503:physiopathology*; D038524:Diffusion Magnetic Resonance Imaging / Q000379:methods*; D007839:Functional Laterality; D006801:Humans; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology
D048948:14-3-3 Proteins