Structural signature of sporadic Creutzfeldt–Jakob disease
Copyright policy )Structural signature of sporadic Creutzfeldt–Jakob disease
Background and purposeSporadic Creutzfeldt–Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease caused by an abnormal isoform of the human prion protein. Structural magnetic resonance imaging in patients with pathologically confirmed sCJD was compared with cognitively normal individuals to identify a cortical thickness signature of sCJD.MethodsThis retrospective cross‐sectional study compared patients with autopsy‐confirmed sCJD with dementia (n = 11) with age‐ and sex‐matched cognitively normal individuals (n = 22). We identified regions of interest (ROIs) in which cortical thickness was most affected by sCJD. Within patients with sCJD, the relationship between ROI cortical thickness and clinical measures (disease duration, cerebrospinal fluid tau and diffusion‐weighted imaging abnormalities) was evaluated.ResultsCompared with cognitively normal individuals, patients with sCJD had significantly reduced cortical thickness in multiple ROIs, including the fusiform gyrus, precentral gyrus, precuneus and superior temporal gyrus bilaterally; the caudal middle frontal gyrus, superior frontal gyrus, postcentral gyrus, inferior temporal gyrus and transverse temporal gyrus in the left hemisphere; and the superior parietal lobule in the right hemisphere. Only one patient with sCJD had co‐pathology consistent with Alzheimer's disease. Reduced cortical thickness did not correlate with disease duration, presence of diffusion restriction or elevated cerebrospinal fluid tau.ConclusionCortical signature changes in sCJD may reflect brain changes not captured by standard clinical measures. This information may be used with clinical measures to inform the progression of sCJD and patterns of prion protein spread throughout the brain. These results may have implications for prediction of symptomatic progression and plausibly for development of therapeutic strategies.
- University of Mary United States
- University of California, San Francisco United States
- Washington University in St. Louis United States
- Hope Center for Neurological Disorders United States
Male, Aging, Acquired Cognitive Impairment (rcdc), Neurodegenerative, Alzheimer's Disease, rapidly progressive dementia, Creutzfeldt-Jakob Syndrome, Clinical Research (rcdc), Aging (rcdc), Biomedical Imaging (rcdc), Transmissible Spongiform Encephalopathy (TSE) (rcdc), cortical signature, Brain Disorders (rcdc), 1103 Clinical Sciences (for), Dementia (rcdc), magnetic resonance imaging, 2.1 Biological and endogenous factors, Neurology & Neurosurgery (science-metrix), Aetiology, 32 Biomedical and Clinical Sciences (for-2020), Male (mesh), Neurosciences (rcdc), Neurodegenerative (rcdc), Humans (mesh), Alzheimer's Disease (rcdc), Brain, Middle Aged, 3209 Neurosciences (for-2020), Infectious Diseases, 4.2 Evaluation of markers and technologies (hrcs-rac), neurodegenerative disorders, Neurological, biomarker, Biomedical Imaging, Female, 4.2 Evaluation of markers and technologies, Diffusion Magnetic Resonance Imaging (mesh), Retrospective Studies (mesh), Clinical Sciences, 610, tau Proteins, Emerging Infectious Diseases (rcdc), Creutzfeldt-Jakob Syndrome (mesh), Cross-Sectional Studies (mesh), Rare Diseases (rcdc), Brain (mesh), Rare Diseases, Clinical Research, Middle Aged (mesh), Acquired Cognitive Impairment, Humans, 3202 Clinical Sciences (for-2020), Aged, Retrospective Studies, Neurology & Neurosurgery, Biomedical and Clinical Sciences, Neurological (hrcs-hc), Aged (mesh), Neurosciences, Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD), Transmissible Spongiform Encephalopathy (TSE), 2.1 Biological and endogenous factors (hrcs-rac), 3202 Clinical sciences (for-2020), cortical thickness, 1109 Neurosciences (for), Creutzfeldt-Jakob disease, prion diseases, Brain Disorders, Emerging Infectious Diseases, Cross-Sectional Studies, Diffusion Magnetic Resonance Imaging, Infectious Diseases (rcdc), Female (mesh), Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) (rcdc), Dementia, tau Proteins (mesh)
Male, Aging, Acquired Cognitive Impairment (rcdc), Neurodegenerative, Alzheimer's Disease, rapidly progressive dementia, Creutzfeldt-Jakob Syndrome, Clinical Research (rcdc), Aging (rcdc), Biomedical Imaging (rcdc), Transmissible Spongiform Encephalopathy (TSE) (rcdc), cortical signature, Brain Disorders (rcdc), 1103 Clinical Sciences (for), Dementia (rcdc), magnetic resonance imaging, 2.1 Biological and endogenous factors, Neurology & Neurosurgery (science-metrix), Aetiology, 32 Biomedical and Clinical Sciences (for-2020), Male (mesh), Neurosciences (rcdc), Neurodegenerative (rcdc), Humans (mesh), Alzheimer's Disease (rcdc), Brain, Middle Aged, 3209 Neurosciences (for-2020), Infectious Diseases, 4.2 Evaluation of markers and technologies (hrcs-rac), neurodegenerative disorders, Neurological, biomarker, Biomedical Imaging, Female, 4.2 Evaluation of markers and technologies, Diffusion Magnetic Resonance Imaging (mesh), Retrospective Studies (mesh), Clinical Sciences, 610, tau Proteins, Emerging Infectious Diseases (rcdc), Creutzfeldt-Jakob Syndrome (mesh), Cross-Sectional Studies (mesh), Rare Diseases (rcdc), Brain (mesh), Rare Diseases, Clinical Research, Middle Aged (mesh), Acquired Cognitive Impairment, Humans, 3202 Clinical Sciences (for-2020), Aged, Retrospective Studies, Neurology & Neurosurgery, Biomedical and Clinical Sciences, Neurological (hrcs-hc), Aged (mesh), Neurosciences, Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD), Transmissible Spongiform Encephalopathy (TSE), 2.1 Biological and endogenous factors (hrcs-rac), 3202 Clinical sciences (for-2020), cortical thickness, 1109 Neurosciences (for), Creutzfeldt-Jakob disease, prion diseases, Brain Disorders, Emerging Infectious Diseases, Cross-Sectional Studies, Diffusion Magnetic Resonance Imaging, Infectious Diseases (rcdc), Female (mesh), Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) (rcdc), Dementia, tau Proteins (mesh)
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