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Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease
Moustafa M Elziny1, Shaimaa S Elsaid2
1Department of Academic Internal Medicine and Geriatrics, University of Illinois at Chicago, Chicago, USA.
Abstract:
Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain infection caused by a human prion. Because CJD is associated with rapidly progressive neurological degeneration, it requires high suspicion for diagnosis. We report the case of a 79-year-old patient who presented with a rapidly progressive neurological clinical picture. The patient had positive 14-3-3 proteins in cerebrospinal fluid, electroencephalography was significant for periodic discharges, and magnetic resonance imaging of the brain showed both diffusion restriction and increased fluid-attenuated inversion recovery signal in different cortical regions, consistent with probable sporadic CJD infection. The patient was enrolled under hospice and palliative care. The patient passed away two months after the onset of her symptoms. We discuss the probable sporadic CJD diagnostic criteria and possible risk factors that might have led to a faster progressive course.
Insights
Creutzfeldt-Jakob disease (CJD), a rare prion infection, presents with rapid neurological decline. This case highlights diagnostic markers and factors influencing its swift progression in an elderly patient.
Area of Science:
- Neurology
- Infectious Diseases
Background:
- Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder caused by prions.
- Rapidly progressive neurological symptoms necessitate high diagnostic suspicion for CJD.
Observation:
- A 79-year-old patient presented with rapidly progressive neurological decline.
- Diagnostic findings included positive 14-3-3 proteins in cerebrospinal fluid, characteristic electroencephalography (EEG) periodic discharges, and MRI showing diffusion restriction and FLAIR hyperintensities.
Findings:
- The clinical presentation and diagnostic results were consistent with probable sporadic CJD.
- The patient's condition progressed rapidly, leading to death within two months of symptom onset.
Implications:
- This case underscores the importance of recognizing CJD diagnostic criteria.
- Further discussion on risk factors potentially accelerating sporadic CJD progression is warranted.
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