From: deputydog23x@gmail.com   
      
   Sensitivity of Revised Diagnostic Criteria for the Behavioural Variant of   
   Frontotemporal Dementia   
      
      
   Katya Rascovsky; John R. Hodges; David Knopman; Mario F. Mendez; Joel H.   
   Kramer; John Neuhaus; John C. van Swieten; Harro Seelaar; Elise G. P. Dopper;   
   Chiadi U. Onyike; Argye E. Hillis; Keith A. Josephs; Bradley F. Boeve; Andrew   
   Kertesz; William W.    
   Seeley; Katherine P. Rankin; Julene K. Johnson; Maria-Luisa Gorno-Tempini;   
   Howard Rosen; Caroline E. Prioleau-Latham; Albert Lee; Christopher M. Kipps;   
   Patricia Lillo; Olivier Piguet; Jonathan D. Rohrer; Martin N. Rossor; Jason D.   
   Warren; Nick C. Fox;    
   Douglas Galasko; David P. Salmon; Sandra E. Black; Marsel Mesulam; Sandra   
   Weintraub; Brad C. Dickerson; Janine Diehl-Schmid; Florence Pasquier; Vincent   
   Deramecourt; Florence Lebert; Yolande Pijnenburg; Tiffany W. Chow; Facundo   
   Manes; Jordan Grafman;    
   Stefano F. Cappa; Morris Freedman; Murray Grossman; Bruce L. Miller|Disclosures   
   Brain. 2011;134(9):2456-2477.    
       
   ABSTRACT AND INTRODUCTION   
   Abstract   
      
   Based on the recent literature and collective experience, an international   
   consortium developed revised guidelines for the diagnosis of behavioural   
   variant frontotemporal dementia. The validation process retrospectively   
   reviewed clinical records and    
   compared the sensitivity of proposed and earlier criteria in a multi-site   
   sample of patients with pathologically verified frontotemporal lobar   
   degeneration. According to the revised criteria, 'possible' behavioural   
   variant frontotemporal dementia    
   requires three of six clinically discriminating features (disinhibition,   
   apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours,   
   hyperorality and dysexecutive neuropsychological profile). 'Probable'   
   behavioural variant    
   frontotemporal dementia adds functional disability and characteristic   
   neuroimaging, while behavioural variant frontotemporal dementia 'with definite   
   frontotemporal lobar degeneration' requires histopathological confirmation or   
   a pathogenic mutation.    
   Sixteen brain banks contributed cases meeting histopathological criteria for   
   frontotemporal lobar degeneration and a clinical diagnosis of behavioural   
   variant frontotemporal dementia, Alzheimer's disease, dementia with Lewy   
   bodies or vascular dementia at    
   presentation. Cases with predominant primary progressive aphasia or   
   extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an   
   experienced neurologist or psychiatrist ascertained clinical features   
   necessary for making a diagnosis    
   according to previous and proposed criteria at presentation. Of 137 cases   
   where features were available for both proposed and previously established   
   criteria, 118 (86%) met 'possible' criteria, and 104 (76%) met criteria for   
   'probable' behavioural    
   variant frontotemporal dementia. In contrast, 72 cases (53%) met previously   
   established criteria for the syndrome (P < 0.001 for comparison with   
   'possible' and 'probable' criteria). Patients who failed to meet revised   
   criteria were significantly older    
   and most had atypical presentations with marked memory impairment. In   
   conclusion, the revised criteria for behavioural variant frontotemporal   
   dementia improve diagnostic accuracy compared with previously established   
   criteria in a sample with known    
   frontotemporal lobar degeneration. Greater sensitivity of the proposed   
   criteria may reflect the optimized diagnostic features, less restrictive   
   exclusion features and a flexible structure that accommodates different   
   initial clinical presentations. Future    
   studies will be needed to establish the reliability and specificity of these   
   revised diagnostic guidelines.   
      
   Introduction   
      
   The behavioural variant of frontotemporal dementia (bvFTD) is a clinical   
   syndrome characterized by a progressive deterioration of personality, social   
   comportment and cognition. These changes result from frontotemporal lobar   
   degeneration (FTLD) associated    
   with a range of heterogeneous pathologies (Mackenzie et al., 2009, 2010).   
   Despite recent advances in the characterization of bvFTD, the diagnosis of the   
   syndrome remains challenging; while some patients are dismissed as 'normal'   
   others may be    
   misdiagnosed as suffering from psychiatric disorders or Alzheimer's disease   
   (Mendez et al., 1993, 2007; Varma et al., 1999). Early and accurate   
   differential diagnosis of bvFTD is critical, as it has implications for   
   heritability (Hutton et al., 1998;    
   Poorkaj et al., 1998; Spillantini et al., 1998; Watts et al., 2004; Skibinski   
   et al., 2005; Baker et al., 2006; Cruts et al., 2006; Kumar-Singh and Van   
   Broeckhoven, 2007), prognosis (Rascovsky et al., 2005; Roberson et al., 2005;   
   Chow et al., 2006),    
   therapeutics (Swartz et al., 1997; Moretti et al., 2003; Pasquier et al.,   
   2003; Lebert et al., 2004; Huey et al., 2006; Boxer and Boeve, 2007; Mendez,   
   2009) and environmental management of patients (Perry and Miller, 2001;   
   Robinson, 2001; Talerico and    
   Evans, 2001; Merrilees and Miller, 2003; Merrilees, 2007; Boutol   
   au-Bretonniere et al., 2008).   
      
   In the absence of definitive biomarkers, the diagnosis of bvFTD is dependent   
   on clinical diagnostic criteria; in other words, the identification of the   
   syndrome's core or necessary symptoms. The publication of consensus criteria   
   by Neary and colleagues (   
   1998) was a major development in the field. These criteria are widely used in   
   research and practice, but some limitations have become apparent. Among these   
   are the ambiguity of behavioural descriptors and inflexibility in the   
   application of criteria (i.e.   
    the requirement that all five core features be manifest). Most importantly, a   
   number of studies have established the relative insensitivity of these   
   criteria in the early stages of bvFTD when disease-modifying treatments are   
   likely to be most effective (   
   Mendez and Perryman, 2002; Mendez et al., 2007; Rascovsky et al., 2007a;   
   Piguet et al., 2009).   
      
      
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