by Isabella Colonna
For January we selected: Wurm R, Klotz S, Erber A, et al. Mood Alterations in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease. JAMA Neurol. Published online 30 December 2024. doi:10.1001/jamaneurol.2024.4447.
Creutzfeldt-Jakob disease (CJD) is a rare and rapidly progressive neurodegenerative disease caused by misfolded prion proteins, which lead to neuronal loss and spongiform encephalopathy. Despite significant research efforts, the etiology and the pathogenesis of sporadic CJD (sCJD) remain unclear. Its short survival period, rarity, and wide-ranging symptoms pose significant challenges to identifying potential risk factors or triggers. Moreover, overlapping health conditions and atypical symptoms often hinder antemortem diagnosis.
In this study, the authors compared drug prescription prior to the clinical onset of sCJD in patients versus matched controls aiming to uncover potential risk or protective factors. Additionally, it examined whether drug exposure in the year before onset influenced survival outcomes in sCJD patients. This retrospective case-control study analysed data collected between 2013 and 2020 from the Austrian Reference Centre for Human Prion Disease and Austrian insurance claims records.
A total of 129 sCJD patients (median [IQR] age, 68.9 [62.4-75.5] years; 67 female [51.9%]) and 1350 controls (median [IQR] age, 69.0 [62.2-75.3] years; 700 female [51.9%]) were included. The authors found a significantly higher prescription rate of SSRIs in the year prior to the disease onset in sCJD patients compared to controls (18.6% versus 8.2%), suggesting a greater prevalence of mood disturbances or depressive symptoms. Interestingly, SSRI prescription rates began to increase as early as three years before symptom onset in sCJD patients, suggesting a potentially longer prodromal phase for the disease.
In conclusion, this study highlights evidence of prodromal mood changes occurring as early as three years before symptom onset in sCJD patients. While sCJD is a rare cause of mood changes, heightened awareness of neurodegenerative conditions in such cases could potentially broaden the diagnostic window.
