title: The phenotypical core of Alzheimer's disease-related and nonrelated variants of the corticobasal syndrome: A systematic clinical, neuropsychological, imaging, and biomarker study.
abstract: The corticobasal syndrome (CBS) constitutes a neurodegenerative disease spectrum with substantial phenotypical or biological heterogeneity, requiring large or multimodal studies to identify its clinico-biological signature while disentangling Alzheimer's disease (AD)-related from non-AD-related CBS.
  
  
  
  We analyzed a large (N = 45) monocenter expert-clinic CBS cohort, recruited in motor and/or cognitive units to avoid recruitment biases, assessed with standardized motor and/or cognitive-language tests, brain perfusion imaging, and cerebrospinal fluid biomarkers.
  
  
  
  CBS mainly manifests as a motor and/or language disorder incorporating a "mixed progressive aphasia" phenotype, consistent with left-lateralized damage to frontal-parietal-temporal cortices. Biomarker expression indicates in 18% underlying AD causing predominant parietal-temporal damage and Gerstmann syndrome (sensitivity 75%; specificity 75%), whereas non-AD-CBS presented with predominant prefrontal and lexical-semantic impairment.
  
  
  
  CBS is primarily a "motor-plus-aphasia" disease unfolding into AD-related and non-AD-related variants with distinctive cognitive-anatomic patterns. CBS, and notably its "Gerstmann variant", should be included in the new AD "lexicon" and categorized in the evolving diagnostic spectrum of "atypical AD"d.
authors.affiliation: Department of Neurology, Institut de la mémoire et de la maladie d'Alzheimer, Centre de Référence 'Démences Rares', Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France; Department of Neurology, University of Cagliari, Cagliari, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Di Stefano
authors.affiliation: Service de Médecine Nucléaire, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France; Université Pierre et Marie Curie-Paris 6, INSERM, UMR-S 678, Paris, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Kas
authors.affiliation: Service de Médecine Nucléaire, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France; Université Pierre et Marie Curie-Paris 6, INSERM, UMR-S 678, Paris, France.
authors.forename: Marie-Odile
authors.initials: MO
authors.lastname: Habert
authors.affiliation: Service de Médecine Nucléaire, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Decazes
authors.affiliation: Department of Metabolic Biochemistry, Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France.
authors.forename: Foudil
authors.initials: F
authors.lastname: Lamari
authors.affiliation: AXA Research Fund & UPMC Chair, Sorbonne Universités, Université Pierre et Marie Curie, Paris, France; Institut du Cerveau et de la Moelle Epinière (ICM), ICM-INSERM 1127, FrontLab, Paris, France.
authors.forename: Simone
authors.initials: S
authors.lastname: Lista
authors.affiliation: Department of Neurology, Institut de la mémoire et de la maladie d'Alzheimer, Centre de Référence 'Démences Rares', Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France; AXA Research Fund & UPMC Chair, Sorbonne Universités, Université Pierre et Marie Curie, Paris, France; Institut du Cerveau et de la Moelle Epinière (ICM), ICM-INSERM 1127, FrontLab, Paris, France.
authors.forename: Harald
authors.initials: H
authors.lastname: Hampel
authors.affiliation: Department of Neurology, Institut de la mémoire et de la maladie d'Alzheimer, Centre de Référence 'Démences Rares', Hôpital de la Pitié-Salpêtrière, AP-HP, Paris, France; Institut du Cerveau et de la Moelle Epinière (ICM), ICM-INSERM 1127, FrontLab, Paris, France. Electronic address: marc.teichmann@psl.aphp.fr.
authors.forename: Marc
authors.initials: M
authors.lastname: Teichmann
mesh_terms: D000544:Alzheimer Disease / Q000145:classification / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D015415:Biomarkers / Q000134:cerebrospinal fluid*; D006801:Humans; D007807:Language Tests / Q000706:statistics & numerical data; D019636:Neurodegenerative Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data; D010641:Phenotype
chemical_list: D015415:Biomarkers
keywords: Alzheimer's disease; Aphasia; CSF biomarkers; Corticobasal syndrome; Neuroimaging