FDG‐PET patterns associate with survival in patients with prion disease
Abstract Objective Prion disease classically presents with rapidly progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising key questions surrounding the...
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| Format: | Article |
| Language: | English |
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Wiley
2024-12-01
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| Series: | Annals of Clinical and Translational Neurology |
| Online Access: | https://doi.org/10.1002/acn3.52230 |
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| author | Nick Corriveau‐Lecavalier Yoav D. Piura Brian S. Appleby Dror Shir Leland R. Barnard Venkatsampath Gogineni David T. Jones Gregory S. Day |
| author_facet | Nick Corriveau‐Lecavalier Yoav D. Piura Brian S. Appleby Dror Shir Leland R. Barnard Venkatsampath Gogineni David T. Jones Gregory S. Day |
| author_sort | Nick Corriveau‐Lecavalier |
| collection | DOAJ |
| description | Abstract Objective Prion disease classically presents with rapidly progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising key questions surrounding the relationship between patterns of neurodegeneration and survival. We assessed the contribution of fluorodeoxyglucose (FDG‐PET) imaging for this purpose. Methods FDG‐PET were performed in 40 clinic patients with prion disease. FDG‐PET images were projected onto latent factors generated in an external dataset to yield patient‐specific eigenvalues. Eigenvalues were input into a clustering algorithm to generate data‐driven clusters, which were compared by survival time. Results Median age at FDG‐PET was 65.3 years (range 23–85). Median time from FDG‐PET to death was 3.7 months (range 0.3–19.0). Four data‐driven clusters were generated, termed “Neocortical” (n = 7), “Transitional” (n = 12), “Temporo‐parietal” (n = 13), and “Deep nuclei” (n = 6). Deep nuclei and transitional clusters had a shorter survival time than the neocortical cluster. Subsequent analyses suggested that this difference was driven by greater hypometabolism of deep nuclei relative to neocortical areas. FDG‐PET‐patterns were not associated with demographic (age and sex) or clinical (CSF total‐tau, 14‐3‐3) variables. Interpretation Greater hypometabolism within deep nuclei relative to neocortical areas associated with more rapid decline in patients with prion disease and vice versa. FDG‐PET informs large‐scale network physiology and may inform the relationship between spreading pathology and survival in patients with prion disease. Future studies should consider whether FDG‐PET may enrich multimodal prion disease prognostication models. |
| format | Article |
| id | doaj-art-4b40233b3b0b474dbbeea6c8abca1dd9 |
| institution | OA Journals |
| issn | 2328-9503 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Wiley |
| record_format | Article |
| series | Annals of Clinical and Translational Neurology |
| spelling | doaj-art-4b40233b3b0b474dbbeea6c8abca1dd92025-08-20T01:58:23ZengWileyAnnals of Clinical and Translational Neurology2328-95032024-12-0111123227323710.1002/acn3.52230FDG‐PET patterns associate with survival in patients with prion diseaseNick Corriveau‐Lecavalier0Yoav D. Piura1Brian S. Appleby2Dror Shir3Leland R. Barnard4Venkatsampath Gogineni5David T. Jones6Gregory S. Day7Department of Neurology Mayo Clinic Rochester Minnesota USADepartment of Neurology Mayo Clinic in Florida Jacksonville Florida USANational Prion Disease Pathology Surveillance Center, Case Western Reserve Cleveland Ohio USADepartment of Neurology Mayo Clinic Rochester Minnesota USADepartment of Neurology Mayo Clinic Rochester Minnesota USADepartment of Neurology Mayo Clinic Rochester Minnesota USADepartment of Neurology Mayo Clinic Rochester Minnesota USADepartment of Neurology Mayo Clinic in Florida Jacksonville Florida USAAbstract Objective Prion disease classically presents with rapidly progressive dementia, leading to death within months of diagnosis. Advances in diagnostic testing have improved recognition of patients with atypical presentations and protracted disease courses, raising key questions surrounding the relationship between patterns of neurodegeneration and survival. We assessed the contribution of fluorodeoxyglucose (FDG‐PET) imaging for this purpose. Methods FDG‐PET were performed in 40 clinic patients with prion disease. FDG‐PET images were projected onto latent factors generated in an external dataset to yield patient‐specific eigenvalues. Eigenvalues were input into a clustering algorithm to generate data‐driven clusters, which were compared by survival time. Results Median age at FDG‐PET was 65.3 years (range 23–85). Median time from FDG‐PET to death was 3.7 months (range 0.3–19.0). Four data‐driven clusters were generated, termed “Neocortical” (n = 7), “Transitional” (n = 12), “Temporo‐parietal” (n = 13), and “Deep nuclei” (n = 6). Deep nuclei and transitional clusters had a shorter survival time than the neocortical cluster. Subsequent analyses suggested that this difference was driven by greater hypometabolism of deep nuclei relative to neocortical areas. FDG‐PET‐patterns were not associated with demographic (age and sex) or clinical (CSF total‐tau, 14‐3‐3) variables. Interpretation Greater hypometabolism within deep nuclei relative to neocortical areas associated with more rapid decline in patients with prion disease and vice versa. FDG‐PET informs large‐scale network physiology and may inform the relationship between spreading pathology and survival in patients with prion disease. Future studies should consider whether FDG‐PET may enrich multimodal prion disease prognostication models.https://doi.org/10.1002/acn3.52230 |
| spellingShingle | Nick Corriveau‐Lecavalier Yoav D. Piura Brian S. Appleby Dror Shir Leland R. Barnard Venkatsampath Gogineni David T. Jones Gregory S. Day FDG‐PET patterns associate with survival in patients with prion disease Annals of Clinical and Translational Neurology |
| title | FDG‐PET patterns associate with survival in patients with prion disease |
| title_full | FDG‐PET patterns associate with survival in patients with prion disease |
| title_fullStr | FDG‐PET patterns associate with survival in patients with prion disease |
| title_full_unstemmed | FDG‐PET patterns associate with survival in patients with prion disease |
| title_short | FDG‐PET patterns associate with survival in patients with prion disease |
| title_sort | fdg pet patterns associate with survival in patients with prion disease |
| url | https://doi.org/10.1002/acn3.52230 |
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