The Hidden Struggles: Behavioral Symptoms in a Rare Disease

A rare and fatal brain disorder, sporadic Creutzfeldt-Jakob disease (sCJD), often goes unnoticed until it's too late. This disease is known for its rapid progression and devastating effects on the brain. However, there's more to sCJD than meets the eye. Beyond the well-known symptoms, there's a significant behavioral and neuropsychiatric side to this condition. It is often overlooked, but it can provide crucial insights into the disease. Behavioral symptoms are not typically part of the diagnostic criteria for sCJD. However, recent studies and clinical experiences have shown that these symptoms are quite common and often appear early in the disease's progression. This raises an important question: could these behavioral symptoms help in distinguishing sCJD from other neurodegenerative diseases? A study was conducted to assess the neuropsychiatric features of sCJD and compare them with five other neurodegenerative diseases. These included Alzheimer's disease, dementia with Lewy bodies, progressive supranuclear palsy, behavioral variant frontotemporal dementia, and semantic variant primary progressive aphasia. The Neuropsychiatric Inventory (NPI) was used to evaluate the behavioral symptoms of patients. This inventory is a tool designed to assess a range of neuropsychiatric symptoms in patients with neurodegenerative diseases. The study involved 789 patients who met the diagnostic criteria for one of the six neurodegenerative disorders. All participants had to undergo a Mini-Mental Status Evaluation and provide demographic data. The results were striking. Of the 92 sCJD patients, 97% exhibited at least one neuropsychiatric symptom by their first research visit. Half of these patients had at least six of the 12 symptom categories assessed by the NPI. The most common behavioral symptoms in sCJD patients included appetite and eating disorders, apathy, night-time behaviors, aberrant motor behavior, and anxiety. Even the least common behavior, disinhibition, was present in 19% of sCJD patients. When compared to other neurodegenerative diseases, sCJD showed significantly higher scores in several symptom categories. For instance, sCJD patients had higher scores for night-time behaviors and delusions compared to those with dementia with Lewy bodies and behavioral variant frontotemporal dementia. They also had higher scores for hallucinations, depression, appetite and eating disorders, aberrant motor behavior, and agitation compared to other diseases. The findings suggest that sCJD is a highly behavioral syndrome. While no single neuropsychiatric symptom is unique to sCJD, certain symptoms could help differentiate it from other neurodegenerative diseases. This supports the idea that behavioral symptoms should be included in the diagnostic criteria for sCJD. This could lead to earlier diagnosis and better management of the disease.