Clinical analysis of 30 patients with sporadic Creutzfeldt-Jakob disease / 临床神经病学杂志

ABSTRACT

Objective To investigate the features of clinical,electroencephalogram(EEG)and imaging in the patients with sporadic Creutzfeldt-Jakob disease(sCJD).Methods The clinical data of 30 patients with sCJD were analyzed retrospectively.Results 6 patients were acute onset,18 patients were subacute onset,which followed by conscious disturbance,myoclonus and decorticate rigidity in 1～3 months.6 patients were chronic onset,which followed by above described typical symptoms in 1～2 years.All the patients showed generally persistent slow waves on EEG in the early stage.18 patients showed typical triphasic wave on EEG and 8 patients presented atypical triphasic wave,which were almost in the intermediate-advandced stage.There were 10 patients in bilateral basal ganglia,and 1 patient in right lenticular nucleus showed hypersignal in MRI T2WI.17 patients carryed out MR diffusion-weighted images(DWI).All the images showed the hypersignal in frontal,temporal,occipital cortex,and 8 patients of them accompanied symmetric hypersignal in bilateral basal ganglia.Conclusions Almost of sCJDs are subacute onset.DWI show typical hypersignal lesions in occipital,frontal,temporal cortex and /or basal ganglia in their early course of disease,which are useful in early diagnosis of CJD.All the patients in the intermediate-advandced stage shows conscious disturbance,myoclonus and decorticate rigidity.The character of EEG is typical triphasic wave.