Abstract Details

Title Disease Notification and Self-Determination in Treatment Decisions for Creutzfeldt-Jacob Disease

Topic Infections/AIDS/Prion Disease

Presentation(s) P04 - (-)

Poster/Presentation
Number 018

Objective

Background BACKGROUND: DWI is useful in detecting CJD during the very early phase, indicating that CJD could be diagnosed even before the onset of dementia. However, it remains unclear whether CJD patients can receive disease notification and can exercise self-determination in treatment decisions.

Design/Methods DESIGN/METHODS: We retrospectively analyzed CJD patients who were admitted to our hospital. We assessed the following parameters: type of CJD, interval from symptom onset to the detection of hyperintense signals on DWI or the time of diagnosis, cognitive function at the time of diagnosis, presence or absence of disease notification to the patients, and self-determination in treatment decisions such as nutrition management. Cognitive function was measured on the basis of Hasegawa Dementia Scale-revised (HDS-R) scores, ranging from 0 (poor) to 30 (good).

Results RESULTS: We analyzed 18 patients (aged 66.3±14.3 years). Fourteen patients had sporadic CJD (MM1, 9; MM2, 3; MV1, 2) and 4 had familial CJD (M232A, 2; V180I, 1; 168-bp insertion, 1). The intervals from symptom onset to the detection of hyperintense signals on DWI or the time of diagnosis were 0.3-48 months (median: 1.5 months) and 0.3-48 months (median: 2 months), respectively. Eight patients diagnosed within less than 2 months were all MM1 patients except 1 patient, whereas 10 patients diagnosed after more than 2 months included only 1 MM1 patient. The HDS-R range was 0 to 29 (median: 5.0); however, 4 patients including 3 familial and 1 MM1 patients maintained a relatively better cognitive function (HDS-R score, ?20). Three patients received disease notification, and all of them exercised self-determination in treatment decisions.

Conclusions CONCLUSIONS: Early diagnosis using DWI enables CJD patients to receive disease notification and exercise self-determination in treatment decisions.

Authors/Disclosures
Fumihiro Yanagimura PRESENTER	No disclosure on file
Takayoshi Shimohata, MD, FAAN (Department of Neurology, Gifu University)	Dr. Shimohata has nothing to disclose.
Masatoyo Nishizawa, MD (Niigata University of Health and Welfare)	No disclosure on file
	No disclosure on file

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