[Imaging diagnosis of Creutzfeldt-Jakob disease]

Creutzfeldt-Jakob disease (CJD) is one of the "prion" diseases. MRI has been reported to detect some findings in this disease, particularly high intensity in the basal ganglia on T2WI. We reviewed six cases of CJD, and found additional characteristic findings. Cerebral atrophy was detected in two of six cases, and in one of these two, it progressed markedly during 8 months. No abnormal density or abnormal signal intensity was detected on CT or T1WI of MRI. Cerebral angiography was done in one patient, and no abnormality was detected. High signal intensity on T2WI in the basal ganglia was detected in five of six cases (83%). T2WI showed high signal intensity in the cerebral cortex in four of six cases (67%), and in three cases the temporal cortex was involved. The high signal intensity in the temporal cortex appeared prior to the appearance of abnormal intensity in the basal ganglia in two cases. We conclude that the high signal intensity in the temporal cortex on T2WI is an important sign and can lead to early diagnosis of CJD. We consider that these MR findings may represent a "prion" disease, not only CJD.