Posters
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Other
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Neurology
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Neurology
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Poster
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Community/Public Health
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Research/Clinical
Abstract
Background: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and neurodegenerative disease caused by an accumulation of misfolded prion proteins in the Central Nervous System. It is estimated that the incidence of Creutzefeldt-Jakob disease is 1-2 cases for every 1 million people. As of 2020 the Texas Rio Grande Valley is home to 1.3 million people.
Case Presentation: Three patients from the Rio Grande Valley were included in this study, all between the ages of 58 and 66, two males and one female. The patients had different socioeconomic backgrounds and were unrelated to one another. All patients underwent multiple diagnostics studies. The first patient, a 65-year-old hispanic male had an initial presentation of dizziness, headache and confusion with quick addition of gait abnormalities, and weakness with involuntary movement of the right arm. Patient was treated with anti-epileptics that provided partial symptomatic relief. Soon after, the patient's symptoms progressed to include ataxic gait and left leg weakness. A cerebrospinal fluid (CSF) panel showed (lets add here which test was this please) a 98% probability of prion disease and a magnetic resonance imaging with and without contrast which showed persistent bilateral symmetric parietal lobe and occipital lobe cortical diffusion restriction consistent with Creutzfeldt-Jakob disease, also known as cortical ribboning.
The second patient, a 58-year-old male presented with 3 months of short-term memory loss and vertigo. He and his spouse noticed a decline in cognitive abilities at home and work. The patient had difficulty performing adequately at work for which he had to take a leave of absence. There was a rapid progression of previous symptoms with the addition of ataxic gait, hallucinations, global aphasia, agitation, spasticity and rigidity The patient's magnetic resonance imaging studies showed significant diffusion restriction within the cortex involving bilateral frontal, parietal and temporal lobes, similar to our first patient. A cerebrospinal fluid analysis was relevant for an elevated Neuron-specific enolase at 24.3ng/mL.
The third patient, a 66-year-old female first suspected of having encephalitis developed slurred speech, word searching and forgetfulness which progressed to right-sided weakness within 2 weeks. A magnetic resonance imaging showed restricted diffusion. A neuron-specific enolase test was done and found to be elevated at 20.3ng/mL.
Prominent restricted diffusion on Diffuse Weighted Imaging with T2 hyperintensities, were found in all three of our patients. The typical electroencephalogram findings for Creutzfeldt-Jakob disease were observed only in one of our patients, but all three patients had abnormalities in their electroencephalograms.
Conclusions: A prompt diagnosis of Creutzfeldt-Jakob disease plays a detrimental part in overall patient support and their families' opportunity to assimilate to the rapid progression of Creutzfeldt-Jakob disease. It is understood that an early diagnosis will not change the outcome or progression of the disease, but it does provide earlier symptomatic treatment and would decrease the number of interventions the patient has to undergo, and the overall resources utilized per patient.
Also presented as an Oral Presentation.
Recommended Citation
Salinas, Daniela I. and Cruz, Alejandro, "Creutzfeldt-Jakob Disease in a Community Hospital in South Texas: Case Series" (2025). Research Symposium. 53.
https://scholarworks.utrgv.edu/somrs/2025/posters/53
Included in
Creutzfeldt-Jakob Disease in a Community Hospital in South Texas: Case Series
Background: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and neurodegenerative disease caused by an accumulation of misfolded prion proteins in the Central Nervous System. It is estimated that the incidence of Creutzefeldt-Jakob disease is 1-2 cases for every 1 million people. As of 2020 the Texas Rio Grande Valley is home to 1.3 million people.
Case Presentation: Three patients from the Rio Grande Valley were included in this study, all between the ages of 58 and 66, two males and one female. The patients had different socioeconomic backgrounds and were unrelated to one another. All patients underwent multiple diagnostics studies. The first patient, a 65-year-old hispanic male had an initial presentation of dizziness, headache and confusion with quick addition of gait abnormalities, and weakness with involuntary movement of the right arm. Patient was treated with anti-epileptics that provided partial symptomatic relief. Soon after, the patient's symptoms progressed to include ataxic gait and left leg weakness. A cerebrospinal fluid (CSF) panel showed (lets add here which test was this please) a 98% probability of prion disease and a magnetic resonance imaging with and without contrast which showed persistent bilateral symmetric parietal lobe and occipital lobe cortical diffusion restriction consistent with Creutzfeldt-Jakob disease, also known as cortical ribboning.
The second patient, a 58-year-old male presented with 3 months of short-term memory loss and vertigo. He and his spouse noticed a decline in cognitive abilities at home and work. The patient had difficulty performing adequately at work for which he had to take a leave of absence. There was a rapid progression of previous symptoms with the addition of ataxic gait, hallucinations, global aphasia, agitation, spasticity and rigidity The patient's magnetic resonance imaging studies showed significant diffusion restriction within the cortex involving bilateral frontal, parietal and temporal lobes, similar to our first patient. A cerebrospinal fluid analysis was relevant for an elevated Neuron-specific enolase at 24.3ng/mL.
The third patient, a 66-year-old female first suspected of having encephalitis developed slurred speech, word searching and forgetfulness which progressed to right-sided weakness within 2 weeks. A magnetic resonance imaging showed restricted diffusion. A neuron-specific enolase test was done and found to be elevated at 20.3ng/mL.
Prominent restricted diffusion on Diffuse Weighted Imaging with T2 hyperintensities, were found in all three of our patients. The typical electroencephalogram findings for Creutzfeldt-Jakob disease were observed only in one of our patients, but all three patients had abnormalities in their electroencephalograms.
Conclusions: A prompt diagnosis of Creutzfeldt-Jakob disease plays a detrimental part in overall patient support and their families' opportunity to assimilate to the rapid progression of Creutzfeldt-Jakob disease. It is understood that an early diagnosis will not change the outcome or progression of the disease, but it does provide earlier symptomatic treatment and would decrease the number of interventions the patient has to undergo, and the overall resources utilized per patient.
Also presented as an Oral Presentation.
