[Disease notification and self-determination in treatment decisions for patients with Creutzfeldt-Jakob disease].

We investigated disease notification and self-determination in treatment decisions of Creutzfeldt-Jakob disease (CJD). For this purpose, we retrospectively analyzed 18 patients with CJD (sporadic:familial = 14:4) who were admitted to our hospital. The durations from symptom onset to the time of diagnosis ranged from 0.3 to 48 months (median, 2 months). The Hasegawa Dementia Scale-revised (HDS-R) range was 0 to 29 (median, 5.0); however, 4 patients (22%), including 2 with familial CJD and 2 with sporadic CJD, maintained cognitive function (HDS-R score ≥ 21). Two patients (11%), who satisfied Lo's criteria for self-determination, were given a disease notification, and both of them archived self-determination in treatment decisions. In conclusion, we demonstrated that patients with CJD could be given a disease notification and archive self-determination in treatment decisions upon early diagnosis using DWI.