[Atypical clinicoradiological course in a case panancephalic variant of Creutzfeldt-Jakob]

Creutzfeldt-Jakob disease (CJD) is the most frequent of the human transmissible spongiform encephalopathies. Pathogenic mechanisms of CJD are still unknown. Sporadic CJD, the most habitual, is clinically characterized by rapidly progressive dementia, myoclonia and ataxia. Panencephalic variant CJD, typically from Japan, is characterized by extensive involvement of the cerebral white and gray matter. International interest has grown from more than one decade ago in relation to the diagnosis of new variant (vCJD). New protocols of MR imaging have contributed to the early diagnosis of CJD with specific signs.

We report a case of panencephalic CJD, with atypical clinical presentation and unusual MR imaging findings.

Our patient developed visual and psychiatric symptoms. Brain MR imaging showed extensive white matter lesions in posterior parietal lobe and occipital regions, which disappeared after steroid treatment. The most characteristic radiological sign for sporadic CJD is the high signal intensity of the basal ganglia, for vCJD the pulvinar sign and, for panencephalic CJD the presence of periventricular white matter lesions, with tendency to the spread when the disease progress. In serial MR imaging studies of our patient, we could see how typical signs were appearing. However, the complete and unusual resolution of the original white matter lesions makes us to think about a possible inflammatory component, in some time in the evolution of white matter damage.