MR DIAGNOSIS OF CREUTZFELDT-JAKOB-DISEASE - SIGNIFICANCE OF HIGH SIGNAL INTENSITY OF THE BASAL GANGLIA

OBJECTIVE, Creutzfeldt-Jakob disease is a rare dementing illness that usually affects older adults. Currently, neuroradiologic examinations play a minor role in the diagnosis of Creutzfeldt-Jakob disease. Several single case reports have noted a distinctive finding of hyperintense signal abnormalities in the basal ganglia on T2-weighted MR images of patients with Creutzfeldt-Jakob disease. In order to assess the diagnostic utility of this finding, we studied the imaging features of four patients with Creutzfeldt-Jakob disease in whom this MR finding was present. MATERIALS AND METHODS. Two neuroradiologists retrospectively reviewed the MR images of four patients who had pathologically proved Creutzfeldt Jakob disease and signal abnormalities in the basal ganglia on TS-weighted MR images. The patients' clinical findings were also analyzed. RESULTS. The four patients had MR examinations between 6 months and 1 year after the onset of symptoms. In all four cases, the hyperintense signal abnormalities in the basal ganglia on T2-weighted images were diffuse and bilaterally symmetric. The T1-weighted images were normal. A CT scan was obtained on a single patient and was normal. CONCLUSION. Although a lack of signal abnormality in the basal ganglia on MR imaging cannot be used to rule out a diagnosis of Creutzfeldt-Jakob disease, our experience and review of published reports suggest that in the proper clinical setting, bilaterally symmetric, diffuse hyperintense abnormalities in the basal ganglia on T2-weighted images may be a specific sign of Creutzfeldt-Jakob disease.