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Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia
- Bergeron, David;
- Gorno‐Tempini, Maria L;
- Rabinovici, Gil D;
- Santos‐Santos, Miguel A;
- Seeley, William;
- Miller, Bruce L;
- Pijnenburg, Yolande;
- Keulen, M Antoinette;
- Groot, Colin;
- van Berckel, Bart NM;
- van der Flier, Wiesje M;
- Scheltens, Philip;
- Rohrer, Jonathan D;
- Warren, Jason D;
- Schott, Jonathan M;
- Fox, Nick C;
- Sanchez‐Valle, Raquel;
- Grau‐Rivera, Oriol;
- Gelpi, Ellen;
- Seelaar, Harro;
- Papma, Janne M;
- van Swieten, John C;
- Hodges, John R;
- Leyton, Cristian E;
- Piguet, Olivier;
- Rogalski, Emily J;
- Mesulam, Marsel M;
- Koric, Lejla;
- Kristensen, Nora;
- Pariente, Jeéreémie;
- Dickerson, Bradford;
- Mackenzie, Ian R;
- Hsiung, Ging‐Yuek R;
- Belliard, Serge;
- Irwin, David J;
- Wolk, David A;
- Grossman, Murray;
- Jones, Matthew;
- Harris, Jennifer;
- Mann, David;
- Snowden, Julie S;
- Chrem‐Mendez, Patricio;
- Calandri, Ismael L;
- Amengual, Alejandra A;
- Miguet‐Alfonsi, Carole;
- Magnin, Eloi;
- Magnani, Giuseppe;
- Santangelo, Roberto;
- Deramecourt, Vincent;
- Pasquier, Florence;
- Mattsson, Niklas;
- Nilsson, Christer;
- Hansson, Oskar;
- Keith, Julia;
- Masellis, Mario;
- Black, Sandra E;
- Matías‐Guiu, Jordi A;
- Cabrera‐Martin, María‐Nieves;
- Paquet, Claire;
- Dumurgier, Julien;
- Teichmann, Marc;
- Sarazin, Marie;
- Bottlaender, Michel;
- Dubois, Bruno;
- Rowe, Christopher C;
- Villemagne, Victor L;
- Vandenberghe, Rik;
- Granadillo, Elias;
- Teng, Edmond;
- Mendez, Mario;
- Meyer, Philipp T;
- Frings, Lars;
- Lleó, Alberto;
- Blesa, Rafael;
- Fortea, Juan;
- Seo, Sang Won;
- Diehl‐Schmid, Janine;
- Grimmer, Timo;
- Frederiksen, Kristian Steen;
- Sánchez‐Juan, Pascual;
- Chételat, Gaël;
- Jansen, Willemijn;
- Bouchard, Rémi W;
- Laforce, Robert Jr;
- Visser, Pieter Jelle;
- Ossenkoppele, Rik
- et al.
Published Web Location
https://doi.org/10.1002/ana.25333Abstract
Objective
To estimate the prevalence of amyloid positivity, defined by positron emission tomography (PET)/cerebrospinal fluid (CSF) biomarkers and/or neuropathological examination, in primary progressive aphasia (PPA) variants.Methods
We conducted a meta-analysis with individual participant data from 1,251 patients diagnosed with PPA (including logopenic [lvPPA, n = 443], nonfluent [nfvPPA, n = 333], semantic [svPPA, n = 401], and mixed/unclassifiable [n = 74] variants of PPA) from 36 centers, with a measure of amyloid-β pathology (CSF [n = 600], PET [n = 366], and/or autopsy [n = 378]) available. The estimated prevalence of amyloid positivity according to PPA variant, age, and apolipoprotein E (ApoE) ε4 status was determined using generalized estimating equation models.Results
Amyloid-β positivity was more prevalent in lvPPA (86%) than in nfvPPA (20%) or svPPA (16%; p < 0.001). Prevalence of amyloid-β positivity increased with age in nfvPPA (from 10% at age 50 years to 27% at age 80 years, p < 0.01) and svPPA (from 6% at age 50 years to 32% at age 80 years, p < 0.001), but not in lvPPA (p = 0.94). Across PPA variants, ApoE ε4 carriers were more often amyloid-β positive (58.0%) than noncarriers (35.0%, p < 0.001). Autopsy data revealed Alzheimer disease pathology as the most common pathologic diagnosis in lvPPA (76%), frontotemporal lobar degeneration-TDP-43 in svPPA (80%), and frontotemporal lobar degeneration-TDP-43/tau in nfvPPA (64%).Interpretation
This study shows that the current PPA classification system helps to predict underlying pathology across different cohorts and clinical settings, and suggests that age and ApoE genotype should be considered when interpreting amyloid-β biomarkers in PPA patients. Ann Neurol 2018;84:737-748.Many UC-authored scholarly publications are freely available on this site because of the UC's open access policies. Let us know how this access is important for you.
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