Caregiver Burden in Prion Disease.

Introduction: Caregiver burden significantly impacts patient and caregiver outcomes and is an important treatment consideration in dementia. Previous research has demonstrated that like behavioral variant frontotemporal dementia, prion disease has higher levels of caregiver burden than other forms of dementia; however, limited prospective research has investigated this specifically. Here, we aimed to describe caregiver well-being and caregiver burden in prion disease and determine whether demographic features, support group attendance, or features of the disease process predicted higher caregiver burden. Methods: Thirty patients with prion disease and their caregivers were assessed longitudinally through the Teleneurology Assessment Program for Creutzfeldt-Jakob Disease. Caregivers were administered the Neuropsychiatric Inventory Questionnaire (NPI-Q), MRC Prion Disease Rating Scale, Outcome Evaluation of the National Family Caregiver Support Program, and other assessment instruments. We performed descriptive and inferential statistics to examine the progression of caregiver burden and to identify features that impacted caregiver burden severity. Results: Thirty caregiver-patient dyads were followed longitudinally. Prion disease duration averaged 7.88 months. Mean initial NPI-Q distress score was 15. Qualitatively, distress increased from the time of study enrollment until peaking on average half-way through study participation and then declined. Higher burden (4-item Zarit Burden Interview) was associated with younger age at disease onset. Burden was not predicted by disease type, duration, caregiver demographics, relationship to the patient, MRC Prion Rating Scale scores, NPI-Q, or support group attendance. Conclusion: These findings confirm significant caregiver distress in prion disease and help better describe the course of caregiver burden throughout the disease. Statistical analyses were limited by small sample size and phenotypic heterogeneity, and future research would benefit from larger sample sizes.