A prospective observational study involving 226 adult patients with suspected rapidly progressive dementia (RPD) has led to significant advancements in the early detection of treatment-responsive causes of the condition. The study, which followed patients for up to 2 years, aimed to characterize diseases associated with RPD as potentially treatment-responsive or non-responsive, based on clinical literature.
Key Findings:
- Patient Demographics: Among the 155 patients who met the defined RPD criteria, 86 (55.5%) had potentially treatment-responsive causes. The median age at symptom onset was 68.9 years, with a balanced distribution between men and women.
- Clinical Features: Several features at presentation were independently associated with treatment-responsive causes of RPD, including seizures, tumor (disease-associated), magnetic resonance imaging suggestive of autoimmune encephalitis, mania, movement abnormalities, and pleocytosis (≥10 cells/mm³) in cerebrospinal fluid.
- Predictive Value: The presence of ≥3 STAM3 P features, including age-at-symptom onset <50 years, captured 82 of 86 (95.3%) cases of treatment-responsive RPD, with a positive predictive value of 100%.
This research underscores the importance of recognizing specific clinical and paraclinical features early in the diagnostic evaluation to identify patients with potentially treatment-responsive RPD, thereby improving outcomes through timely and appropriate intervention.
