Abstract Details

Title Advanced Description of a Large Cohort of Patients with Creutzfeldt-Jakob Disease

Topic Aging, Dementia, and Behavioral Neurology

Presentation(s) P10 - Poster Session 10 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-012

Objective To analyze clinical data including MRI, biomarker, and EEG patterns in patients diagnosed with Creutzfeldt-Jakob Disease (CJD).

Background CJD is a rare, fatal, neurodegenerative prion-mediated disease. This study represents one of the largest single-center studies analyzing EEG and MRI data from patients diagnosed with CJD.

Design/Methods This is a retrospective study of patients diagnosed with CJD at a single-center hospital with long-term EEG monitoring data from 2006-2023. Clinical factors, MRI findings, and EEG reports were analyzed. Dipole localization mapping is in process with an epileptologist and electrodiagnostics expert.

Results 31 patients met inclusion criteria. Preliminary results from this cohort reveal mean age 69.58 years, 77% Caucasian/white. Most common comorbidities were hypertension, hyperlipidemia, and diabetes mellitus. Two most common findings on exam were cognitive deficits and gait abnormalities. Ataxia/dysmetria, abnormal tone, and myoclonus were the most common abnormal movements. Average CSF results included: WBC 3, protein 53, glucose 74. 23 patients had 14-3-3 testing with 19 (82%) having positive results. Twenty patients had CSF RT-Quic testing with mixed results (positive, n=14, 70%; negative, n=3, 15%; indeterminate, n=3, 15%). Twenty patients also had elevated T-tau protein markers. Of the 30 patients with MR imaging, 26 (87%) showed abnormal diffusion restriction (cortical, n=23; subcortical, n=18; caudate, n=9; basal ganglia, n=8; thalamus, n=4; left predominant, n=6; right predominant, n=8). 27 patients had abnormal EEGs with focal slowing, periodic or continuous discharges, or asymmetric background. Further sub-analysis of EEG data and dipole localization mapping are pending completion.

Conclusions Diagnosing CJD is challenging, as clinical factors and physical exam can be nonspecific. MRI, EEG, and biomarker testing are helpful but imperfect. Further sub-analyses of EEG data that have not been performed in prior studies will yield new information about the electrographic changes seen in CJD.

Authors/Disclosures
Ada Breitenbucher, MD PRESENTER	Dr. Breitenbucher has nothing to disclose.
Yazan Al-Hasan, MD, PhD (St. Joseph's Hospital and Medical Center)	Dr. Al-Hasan has nothing to disclose.
Justin Hoskin, MD (Barrow Neurological Institute)	Dr. Hoskin has nothing to disclose.
Stephen Foldes (Barrow Neurological Institute)	No disclosure on file
Vladimir Shvarts, MD	Dr. Shvarts has nothing to disclose.

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