| Autor: |
Simuni, Tanya, Chahine, Lana, Poston, Kathleen, Brumm, Michael, Buracchio, Theresa, Campbell, Michelle, Chowdhury, Sohini, Coffey, Christopher, Concha-Marambio, Luis, Dam, Tien, DiBiaso, Peter, Foroud, Tatiana, Frasier, Mark, Gochanour, Caroline, Jennings, Danna, Kieburtz, Karl, Kopil, Catherine M., Merchant, Kalpana, Mollenhauer, Brit, Montine, Thomas, Nudelman, Kelly, Pagano, Gennaro, Seibyl, John, Sherer, Todd, Singleton, Andrew, Stephenson, Diane, Stern, Matthew, Soto, Claudio, Tanner, Caroline M., Tolosa, Eduardo, Weintraub, Daniel, Xiao, Yuge, Siderowf, Andrew, Dunn, Billy, Marek, Kenneth |
| Přispěvatelé: |
Poewe, Werner, Handler, Alison, Mathur, Soania, Siu, Carroll, Asis, Angelica, Campbell, Clyde, Dexter, David, Fargo, Keith, Lee, Karen, Matthews, Helen, Naito, Anna, Taylor, Angela, Multiple Collaborators at The Critical Path Institute, Parkinson Canada, Shake It Up Australia Foundation, Parkinson's UK, Lewy Body Dementia Association, Cure Parkinson's |
| Jazyk: |
angličtina |
| Rok vydání: |
2023 |
| Předmět: |
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| DOI: |
10.5281/zenodo.8178862 |
| Popis: |
Parkinson’s disease and dementia with Lewy Bodies share the same underlying neurobiology (Lewy pathology with neuronal aggregates of pathologic alpha-synuclein) yet are currently defined clinically. We propose a biological definition for “Neuronal alpha-Synuclein Disease (NSD)” including all clinicopathological entities associated with neuronal-predominant pathologic alpha-synuclein (n-asyn) aggregation. NSD is defined by presence of pathologic n-asyn species detected in-vivo (S) independent of any specific clinical syndrome. We further propose that individuals with n-asyn are at risk for dopaminergic neuronal dysfunction (D), the second biologic anchor for NSD. The Neuronal Synuclein Disease Integrated Staging System (NSD-ISS) integrates these biological anchors (S and D) and degree of functional impairment caused by signs/symptoms. Stages 0-1 are without signs/symptoms and defined by presence of pathogenic variants in SNCA gene (Stage 0), S alone (Stage 1A) or S and D (Stage 1B). Presence of clinical manifestations marks transition to Stage 2 and beyond. Stage 2 is characterized by subtle signs/symptoms but no functional impairment. Stages 2B-6 require both S and D and the stage-specific increases in functional impairment. NSD definition and NSD-ISS, which will evolve as additional biomarkers emerge, provide a framework essential to advancing biologically-targetedtherapeutics and enablinginterventional trials at early disease stages. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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