NEUROPSYCHIATRIC symptoms are highly prevalent in sporadic Creutzfeldt-Jakob disease (sCJD), with nearly all patients exhibiting behavioral changes early in the disease course, according to a recent study. Researchers analyzed data from 789 patients with neurodegenerative diseases, including 92 with sCJD, and found that 97% of sCJD patients had at least one neuropsychiatric symptom at their first clinical visit. This new evidence suggests that behavioral symptoms could play a critical role in distinguishing sCJD from other neurodegenerative disorders, such as Alzheimer’s disease (AD) and dementia with Lewy bodies (DLB).
Using the Neuropsychiatric Inventory (NPI), researchers assessed behavioral symptoms across sCJD and five other neurodegenerative diseases. In sCJD, the most common symptoms included appetite and eating disorders (68%), apathy (66%), nighttime disturbances (53%), aberrant motor behavior (53%), and anxiety (52%). Even less frequent symptoms, such as disinhibition, were present in nearly one-fifth of patients. Notably, sCJD patients showed significantly higher scores for nighttime behaviors and delusions compared to both DLB and behavioral variant frontotemporal dementia (bvFTD), two conditions already known for their pronounced behavioral components. Additionally, sCJD patients exhibited more severe hallucinations and depression than those with bvFTD and more pronounced agitation and aberrant motor behavior than those with DLB.
These findings highlight the distinct neuropsychiatric profile of sCJD and underscore the need to incorporate behavioral symptoms into its diagnostic criteria. Since sCJD progresses rapidly and lacks a definitive early biomarker, recognizing these symptoms sooner could improve clinical suspicion and guide earlier interventions.
Reference: Zitser J et al. Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease. Brain. 2025:awaf077. doi: 10.1093/brain/awaf077. [Online ahead of print].
Anaya Malik | AMJ
