title: Primary Progressive Aphasia in the Network of French Alzheimer Plan Memory Centers.
abstract: Few demographical data about primary progressive aphasia (PPA) are available, and most knowledge regarding PPA is based on tertiary centers' results.
  
  
  
  Our aims were to describe demographical characteristics of the PPA population in a large sample of PPA patients from the network of French Alzheimer plan memory centers (Sample 1), and to describe the stratification of cerebrospinal fluid (CSF) biomarkers in two different samples of PPA patients (Samples 2 and 3).
  
  
  
  All registered PPA patients in the French Alzheimer's disease (AD) databank (Sample 1: n = 2,035) and a subsample (Sample 2: n = 65) derived from a multicentric prospective cohort with CSF biomarker analysis were analyzed. A multicentric retrospective cohort from language expert tertiary centers (Sample 3: n = 97) with CSF biomarker analysis was added. Sample 3 was added to replicate the CSF results of the Sample 2 and to evaluate repartition of AD pathology in the three variant of PPA according to the latest classification.
  
  
  
  Non-Fluent/Agrammatic, Logopenic, and Unclassifiable PPA patients (NF/A-Logo-Unclass PPA) were older and more frequent than Semantic PPA patients (2.2 versus 0.8/100,000 inhabitants; p < 0.00001). Male predominance occurred after the age of 80 (p < 0.00001). A higher level of education was observed in the PPA population compared to a typical amnesic AD group. No demographical significant difference between PPA due to AD and not due to AD was observed. The Logopenic variant was most frequent with 85% of AD CSF biomarker profiles (35% in NF/A PPA; 20% in Semantic PPA).
  
  
  
  PPA occurs also in an elderly population, especially in male patients over 80. CSF biomarkers are useful to stratify PPA. The epidemiology of PPA should be further investigated to confirm gender and cognitive reserve role in PPA to better understand the factors and mechanisms leading to this language-predominant deficit during neurodegenerative diseases.
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Hôpital Universitaire de Besançon, Besançon, France.
authors: Eloi
authors: E
authors: Magnin
authors: Centre Leenaards de la Mémoire, CHUV, Lausanne, Suisse.
authors: Jean-François
authors: JF
authors: Démonet
authors: Service de Neurologie, Hôpital universitaire de Rouen, et INSERM UMR1079, Université de Rouen, Rouen, France.
authors: David
authors: D
authors: Wallon
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Paris Nord Ile de France, APHP, Paris, France.|INSERM U942, Paris, France.
authors: Julien
authors: J
authors: Dumurgier
authors: Inserm 1171, Univ. Lille, CHU Lille, Distalz, CMRR, CNR-MAJ, F-59000 Lille, France.
authors: Anne-Cécile
authors: AC
authors: Troussière
authors: Centre de Neurologie, 6 place Luxembourg, Thionville, France.
authors: Alain
authors: A
authors: Jager
authors: Centre Mémoire de Ressources et de Recherche Paris Sud, Hôpital Broca APHP, Université Paris V, Paris, France.
authors: Emmanuelle
authors: E
authors: Duron
authors: Centre Mémoire de Ressources et de Recherche (CMRR) de Montpellier, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire Montpellier et INSERM U1040, hôpital Saint Eloi, Montpellier, France.
authors: Audrey
authors: A
authors: Gabelle
authors: Centre Mémoire de Ressources (CMRR) de Caen, Hôpital Universitaire de Caen, Caen, France.
authors: Vincent
authors: V
authors: de la Sayette
authors: Consultation Mémoire, Groupe Hôpitalier Saint-Joseph, Paris, France.
authors: Lisette
authors: L
authors: Volpe-Gillot
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Hôpital Universitaire de Besançon, Besançon, France.
authors: Gregory
authors: G
authors: Tio
authors: Centre mémoire de ressources et de recherche, Service de Neurologie, CHU Nantes, Nantes, France.
authors: Sarah
authors: S
authors: Evain
authors: Centre mémoire de ressources et de recherche, Service de Neurologie, CHU Nantes, Nantes, France.
authors: Claire
authors: C
authors: Boutoleau-Bretonnière
authors: Inserm 1171, Univ. Lille, CHU Lille, Distalz, CMRR, CNR-MAJ, F-59000 Lille, France.
authors: Adeline
authors: A
authors: Enderle
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Paris Nord Ile de France, APHP, Paris, France.|INSERM U942, Paris, France.|Unité d'Histologie et de Biologie du Vieillissement, APHP, Paris, France.
authors: François
authors: F
authors: Mouton-Liger
authors: Centre Mémoire de Ressources et de Recherche/EA COBTEK, CHU, Université de Nice, Nice, France.
authors: Philippe
authors: P
authors: Robert
authors: Service de Neurologie, Hôpital universitaire de Rouen, et INSERM UMR1079, Université de Rouen, Rouen, France.
authors: Didier
authors: D
authors: Hannequin
authors: Inserm 1171, Univ. Lille, CHU Lille, Distalz, CMRR, CNR-MAJ, F-59000 Lille, France.
authors: Florence
authors: F
authors: Pasquier
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Paris Nord Ile de France, APHP, Paris, France.|INSERM U942, Paris, France.|Unité d'Histologie et de Biologie du Vieillissement, APHP, Paris, France.
authors: Jacques
authors: J
authors: Hugon
authors: Centre Mémoire de Ressources et de Recherche (CMRR), Paris Nord Ile de France, APHP, Paris, France.|INSERM U942, Paris, France.|Unité d'Histologie et de Biologie du Vieillissement, APHP, Paris, France.
authors: Claire
authors: C
authors: Paquet
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology*; D018888:Aphasia, Primary Progressive / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology*; D015331:Cohort Studies; D016208:Databases, Factual / Q000639:trends; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012189:Retrospective Studies; D063728:Secondary Care Centers / Q000639:trends; D062606:Tertiary Care Centers / Q000639:trends
keywords: Alzheimer’s disease; cerebrospinal fluid biomarkers; epidemiology; frontotemporal dementia; gender; primary progressive aphasia