Study on clinic, pathology and image of sporadic Creutzfeldt-Jakob disease / 临床神经病学杂志

ABSTRACT

Objective To investigate the clinical, pathological and imaging features of sporadic Creutzfeldt-Jakob disease (CJD). Methods The clinical, pathological, electroencephalogram (EEG) and imaging data of 12 cases of CJD were analyzed retrospectively.Results (1) 7 cases of the enrolled patients were male and 5 were female. The average onset age of them was 49 years old. 3 cases were acute onset by visual loss and 9 cases were subacute onset by hypophrenia, mental and behavior disorder or cerebellar ataxia. All the cases showed dementia, myoclonus and extrapyramidal sign. (2) 9 cases showed typical triphasic wave on EEG and 1 case presented atypical triphasic wave. (3) 5 cases showed abnormal hyperintense lesions in basal ganglia on T 2-weighted images (T 2WI). 8 cases in occipital and/or frontal cortex showed the abnormal hyperintense lesions on diffusion-weighted images (DWI), and 5 of them accompanied symmetric diffuse hyperintense lesions in basal ganglia. (4) 1 case with postmortem and 6 cases with biopsy of brain showed classic pathological features of CJD.Conclusion On the base of typical clinic manifestations, EEG and DWI examinations are useful in early diagnosis of sporadic CJD.