2025 American Academy of Neurology Abstract Website

Objective:

To report a cluster of rapidly progressive cases of sporadic Creutzfeldt-Jakob Disease (sCJD) with significantly elevated cerebrospinal fluid (CSF) biomarkers, highlighting the prognostic implications of these findings.

Background:

Previous studies have reported a correlation between elevated total tau levels and shortened survival time in patients with sCJD. We present a case series of three patients diagnosed with probable sCJD at Maimonides Medical Center (MMC) in Brooklyn, NY, within one year, each exhibiting a rapid disease course of 4 to 9 weeks and significantly elevated CSF total tau levels.

Design/Methods:

Results:

Case 1: A 77-year-old woman with a disease duration of 4 weeks until death, with total tau levels exceeding 20,000 pg/mL.

Case 2: A 69-year-old woman with a disease duration of 8 weeks until discharge to hospice, with total tau levels exceeding 20,000 pg/mL.

Case 3: A 64-year-old man with a disease duration of 9 weeks until death, with total tau levels of 18,748 pg/mL.

All patients presented with rapid-onset dementia and motor deficits. Each had a positive RT-QuIC test, along with findings suggestive of sCJD, including triphasic periodic sharp wave complexes on EEG and cortical ribboning on DWI MRI. In all three cases, the time from symptom onset to death or hospice discharge was significantly shorter than the typical progression of sCJD. Each patient exhibited exceptionally high CSF total tau levels, which correlated with the unusually rapid disease course.

Conclusions:

This case series emphasizes the inverse relationship between CSF total tau levels and survival time in patients with probable sCJD. Further investigation into patient-specific factors within this cohort may provide additional insights into determinants of survival time. The occurrence of this case cluster within one year suggests the need for heightened awareness and continued monitoring.