Journal ArticleDOI
Frontotemporal lobar degeneration A consensus on clinical diagnostic criteria
David Neary,Julie S. Snowden,L. Gustafson,U. Passant,Donald T. Stuss,Sandra E. Black,Morris Freedman,Andrew Kertesz,Philippe Robert,Marilyn S. Albert,Kyle B. Boone,Bruce L. Miller,Jeffrey L. Cummings,D. F. Benson +13 more
TLDR
Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotem temporal lobar degeneration and ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders.Abstract:
Objective: To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration. Methods: Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotemporal lobar degeneration. These criteria build on earlier published clinical diagnostic guidelines for frontotemporal dementia produced by some of the workshop members. Results: The consensus criteria specify core and supportive features for each of the three prototypic clinical syndromes and provide broad inclusion and exclusion criteria for the generic entity of frontotemporal lobar degeneration. The criteria are presented in lists, and operational definitions for features are provided in the text. Conclusions: The criteria ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders that account for a substantial proportion of cases of primary degenerative dementia occurring before the age of 65 years.read more
Citations
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The diagnosis of dementia due to Alzheimer’s disease: Recommendations from the National Institute on Aging-Alzheimer’s Association workgroups on diagnostic guidelines for Alzheimer's disease
Guy M. McKhann,Guy M. McKhann,David S. Knopman,Howard Chertkow,Bradley T. Hyman,Clifford R. Jack,Claudia H. Kawas,William E. Klunk,Walter J. Koroshetz,Jennifer J. Manly,Richard Mayeux,Richard C. Mohs,John C. Morris,Martin N. Rossor,Philip Scheltens,Maria C. Carrillo,Bill Thies,Sandra Weintraub,Creighton H. Phelps +18 more
TL;DR: The workgroup sought to ensure that the revised criteria would be flexible enough to be used by both general healthcare providers without access to neuropsychological testing, advanced imaging, and cerebrospinal fluid measures, and specialized investigators involved in research or in clinical trial studies who would have these tools available.
Journal ArticleDOI
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Manuela Neumann,Deepak M. Sampathu,Linda K. Kwong,Adam C. Truax,Matthew Micsenyi,Thomas T. Chou,Jennifer Bruce,Theresa Schuck,Murray Grossman,Christopher M. Clark,Leo McCluskey,Bruce L. Miller,Eliezer Masliah,Ian R. A. Mackenzie,Howard Feldman,Wolfgang Feiden,Hans A. Kretzschmar,John Q. Trojanowski,Virginia M.-Y. Lee +18 more
TL;DR: It is shown that TDP-43 is the major disease protein in both frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis.
Journal ArticleDOI
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS
Mariely DeJesus-Hernandez,Ian R. A. Mackenzie,Bradley F. Boeve,Adam L. Boxer,Matt Baker,Nicola J. Rutherford,Alexandra M. Nicholson,Ni Cole A. Finch,Heather C. Flynn,Jennifer Adamson,Naomi Kouri,Aleksandra Wojtas,Pheth Sengdy,Ging-Yuek Robin Hsiung,Anna Karydas,William W. Seeley,Keith A. Josephs,Giovanni Coppola,Daniel H. Geschwind,Zbigniew K. Wszolek,Howard Feldman,Howard Feldman,David S. Knopman,Ronald C. Petersen,Bruce L. Miller,Dennis W. Dickson,Kevin B. Boylan,Neill R. Graff-Radford,Rosa Rademakers +28 more
TL;DR: It is found that repeat expansion in C9ORF72 is a major cause of both FTD and ALS, suggesting multiple disease mechanisms.
Journal ArticleDOI
Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.
Katya Rascovsky,John R. Hodges,David S. 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 J. Rosen,Caroline E. Prioleau-Latham,Albert Lee,Christopher M. Kipps,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,M.-Marsel Mesulam,Sandra Weintraub,Brad C. Dickerson,Janine Diehl-Schmid,Florence Pasquier,Vincent Deramecourt,Florence Lebert,Yolande A.L. Pijnenburg,Tiffany W. Chow,Facundo Manes,Jordan Grafman,Stefano F. Cappa,Morris Freedman,Murray Grossman,Bruce L. Miller +46 more
TL;DR: The revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotmporal lobar degeneration and reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations.
Journal ArticleDOI
Classification of primary progressive aphasia and its variants
Maria Luisa Gorno-Tempini,Maria Luisa Gorno-Tempini,Argye E. Hillis,Sandra Weintraub,Andrew Kertesz,Mario F. Mendez,Stefano F. Cappa,J. M. Ogar,Jonathan D. Rohrer,Sandra E. Black,Bradley F. Boeve,Facundo Manes,Nina F. Dronkers,Rik Vandenberghe,Katya Rascovsky,Karalyn Patterson,Bruce L. Miller,D. S. Knopman,John R. Hodges,M.-Marsel Mesulam,Murray Grossman +20 more
TL;DR: This article provides a classification of primary progressive aphasia (PPA) and its 3 main variants to improve the uniformity of case reporting and the reliability of research results.
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Tau is a candidate gene for chromosome 17 frontotemporal dementia.
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Slowly progressive aphasia without generalized dementia
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Semantic dementia: A form of circumscribed cerebral atrophy
TL;DR: In this article, a 67-yr-old man and 2 women (aged 60 and 66 yrs) with primary cerebral atrophy in whom progressive breakdown in language and visual perception are attributed to loss of semantic information.
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