Duration between initial manifestation of CJD and detection of PSD, specific findings on MRI, CSF 14-3-3 protein, or CSF high NSE

To evaluate the usefulness of clinical examinations in the diagnosis of CJD, we examined duration between initial manifestation of CJD and detection of PSD, specific findings on MRI, 14-3-3 protein, or high NSE. We analyzed data collected by Japanese CJD surveillance committee statistically. Patients consisted of 405 sporadic CJD and 52 infectious CJD. Average duration between the onset and detection of PSD, specific findings on MRI, 14-3-3 protein in the CSF, or high NSE in the CSF in the total cases was 3.6, 4.4, 4.8, or 3.6 months, respectively. There was no significant difference among 4 examinations (P=0.12). Peak of duration until positive result on MRI, 14-3-3 protein, or NSE was 1 month while that on EEG was 2 months. Similar result was obtained when we analyzed the subgroup of sporadic CJD. The sensitivity of EEG, MRI, 14-3-3 protein, or NSE in the definite cases was 72.4, 77.1, 74.2, 50.0 %, and the performed rate in the total cases was 99.1, 75.9, 36.5, 44.0 %, respectively as we showed in the previous report partially. The sensitivity and performed rate of EEG were high and duration until positive result was short while the number of detection of PSD reached its peak 2 months after the onset in contrast with 1 month as in the other examinations. Conventionality in diagnosis of CJD and inclusion in the criteria fot CJD might influence on high performed rate and short average duration. Although some researches reported very early detection of specific findings on MRI, the average duration until the detection of specific MRI findings was not significantly shorter than those on the other examinations in Japanese CJD surveillance. This might be due to lower recognition rate of usefulness of DWI in diagnosis of CJD. The sensitivity of NSE in the definite cases was lowest although the difference of the sensitivity was not significant among 4 examination (p=0.063). Low performed rate of 14-3-3 protein or NSE was remarkable. In conclusion, analysis of data from Japanese CJD surveillance revealed no significant difference of duration between the onset and detection of positive results among 4 examinations. Further spread of recognition of usefulness of DWI in the diagnosis of CJD might shorten duration between the onset and detection of positive result on MRI.