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Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration.
Olney, Nicholas T; Ong, Elise; Goh, Sheng-Yang M; Bajorek, Lynn; Dever, Reilly; Staffaroni, Adam M; Cobigo, Yann; Bock, Meredith; Chiang, Kevin; Ljubenkov, Peter; Kornak, John; Heuer, Hilary W; Wang, Ping; Rascovsky, Katya; Wolf, Amelia; Appleby, Brian; Bove, Jessica; Bordelon, Yvette; Brannelly, Patrick; Brushaber, Danielle; Caso, Christine; Coppola, Giovanni; Dickerson, Bradford C; Dickinson, Susan; Domoto-Reilly, Kimiko; Faber, Kelly; Ferrall, Jessica; Fields, Julie; Fishman, Ann; Fong, Jamie; Foroud, Tatiana; Forsberg, Leah K; Gearhart, Debra J; Ghazanfari, Behnaz; Ghoshal, Nupur; Goldman, Jill; Graff-Radford, Jonathan; Graff-Radford, Neill R; Grant, Ian; Grossman, Murray; Haley, Dana; Hsiung, Gingyuek; Huey, Edward D; Irwin, David J; Jones, David T; Kantarci, Kejal; Karydas, Anna M; Kaufer, Daniel; Kerwin, Diana; Knopman, David S.
Afiliación
  • Olney NT; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Ong E; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Goh SM; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Bajorek L; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Dever R; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Staffaroni AM; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Cobigo Y; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Bock M; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Chiang K; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Ljubenkov P; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Kornak J; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Heuer HW; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Wang P; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Rascovsky K; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
  • Wolf A; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Appleby B; Department of Neurology, Case Western Reserve University, Cleveland, OH, USA.
  • Bove J; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
  • Bordelon Y; Department of Neurology, University of California, Los Angeles, Los Angeles, CA, USA.
  • Brannelly P; Tau Consortium, Rainwater Charitable Foundation, Fort Worth, TX, USA.
  • Brushaber D; Department of Health Sciences Research, Mayo Clinic, Rochester, MN, USA.
  • Caso C; Department of Neurology, University of Washington, Seattle, WA, USA.
  • Coppola G; Department of Neurology, University of California, Los Angeles, Los Angeles, CA, USA.
  • Dickerson BC; Department of Neurology, Frontotemporal Disorders Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
  • Dickinson S; Association for Frontotemporal Degeneration, Radnor, PA, USA.
  • Domoto-Reilly K; Department of Neurology, University of Washington, Seattle, WA, USA.
  • Faber K; National Centralized Repository for Alzheimer's Disease and Related Disorders (NCRAD), Indiana University, Indianapolis, IN, USA.
  • Ferrall J; Department of Neurology, University of North Carolina, Chapel Hill, NC, USA.
  • Fields J; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Fishman A; Department of Psychiatry and Behavioral Sciences, School of Medicine, Johns Hopkins University, Baltimore, MD, USA.
  • Fong J; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
  • Foroud T; National Centralized Repository for Alzheimer's Disease and Related Disorders (NCRAD), Indiana University, Indianapolis, IN, USA.
  • Forsberg LK; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Gearhart DJ; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Ghazanfari B; Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
  • Ghoshal N; Department of Psychiatry, Washington University, St. Louis, MO, USA.
  • Goldman J; Department of Neurology, Washington University, St. Louis, MO, USA.
  • Graff-Radford J; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, NY, USA.
  • Graff-Radford NR; Department of Neurology, Columbia University, New York, NY, USA.
  • Grant I; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Grossman M; Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.
  • Haley D; Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
  • Hsiung G; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
  • Huey ED; Department of Neurology, Mayo Clinic, Jacksonville, FL, USA.
  • Irwin DJ; Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.
  • Jones DT; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, NY, USA.
  • Kantarci K; Department of Neurology, Columbia University, New York, NY, USA.
  • Karydas AM; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
  • Kaufer D; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Kerwin D; Department of Neurology, Mayo Clinic, Rochester, MN, USA.
  • Knopman DS; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
Alzheimers Dement ; 16(1): 49-59, 2020 01.
Article en En | MEDLINE | ID: mdl-31784375
INTRODUCTION: The Advancing Research and Treatment in Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects longitudinal studies were designed to describe the natural history of familial-frontotemporal lobar degeneration due to autosomal dominant mutations. METHODS: We examined cognitive performance, behavioral ratings, and brain volumes from the first time point in 320 MAPT, GRN, and C9orf72 family members, including 102 non-mutation carriers, 103 asymptomatic carriers, 43 mildly/questionably symptomatic carriers, and 72 carriers with dementia. RESULTS: Asymptomatic carriers showed similar scores on all clinical measures compared with noncarriers but reduced frontal and temporal volumes. Those with mild/questionable impairment showed decreased verbal recall, fluency, and Trail Making Test performance and impaired mood and self-monitoring. Dementia was associated with impairment in all measures. All MAPT carriers with dementia showed temporal atrophy, but otherwise, there was no single cognitive test or brain region that was abnormal in all subjects. DISCUSSION: Imaging changes appear to precede clinical changes in familial-frontotemporal lobar degeneration, but specific early clinical and imaging changes vary across individuals.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atrofia / Procesamiento de Imagen Asistido por Computador / Predisposición Genética a la Enfermedad / Degeneración Lobar Frontotemporal / Pruebas Neuropsicológicas Tipo de estudio: Observational_studies / Risk_factors_studies Límite: Female / Humans / Male / Middle aged Idioma: En Revista: Alzheimers Dement Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Atrofia / Procesamiento de Imagen Asistido por Computador / Predisposición Genética a la Enfermedad / Degeneración Lobar Frontotemporal / Pruebas Neuropsicológicas Tipo de estudio: Observational_studies / Risk_factors_studies Límite: Female / Humans / Male / Middle aged Idioma: En Revista: Alzheimers Dement Año: 2020 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos