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Individualized atrophy scores predict dementia onset in familial frontotemporal lobar degeneration.
Staffaroni, Adam M; Cobigo, Yann; Goh, Sheng-Yang M; Kornak, John; Bajorek, Lynn; Chiang, Kevin; Appleby, Brian; Bove, Jessica; Bordelon, Yvette; Brannelly, Patrick; Brushaber, Danielle; Caso, Christina; Coppola, Giovanni; Dever, Reilly; Dheel, Christina; Dickerson, Bradford C; Dickinson, Susan; Dominguez, Sophia; Domoto-Reilly, Kimiko; Faber, Kelly; Ferrall, Jessica; Fields, Julie A; Fishman, Ann; Fong, Jamie; Foroud, Tatiana; Forsberg, Leah K; Gavrilova, Ralitza; Gearhart, Debra; Ghazanfari, Behnaz; Ghoshal, Nupur; Goldman, Jill; Graff-Radford, Jonathan; Graff-Radford, Neill; Grant, Ian; Grossman, Murray; Haley, Dana; Heuer, Hilary W; Hsiung, Ging-Yuek; Huey, Edward D; Irwin, David J; Jones, David T; Jones, Lynne; Kantarci, Kejal; Karydas, Anna; Kaufer, Daniel I; Kerwin, Diana R; Knopman, David S; Kraft, Ruth; Kramer, Joel H; Kremers, Walter K.
Afiliação
  • Staffaroni AM; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA 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 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 University of California, San Francisco, San Francisco CA, USA.
  • Kornak J; Department of Epidemiology and Biostatistics, University of California, San Francisco, San Francisco CA, USA Department of Neurology, Memory and Aging Center.
  • Bajorek L; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA 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 University of California, San Francisco, San Francisco CA, USA.
  • Appleby B; Department of Neurology, Case Western Reserve University, Cleveland, OH, USA University of California, San Francisco, San Francisco CA, USA.
  • Bove J; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA Department of Epidemiology and Biostatistics.
  • Bordelon Y; Department of Neurology, University of California, Los Angeles, Los Angeles, CA, USA Case Western Reserve University, Cleveland, OH, USA.
  • Brannelly P; Tau Consortium, Rainwater Charitable Foundation, Fort Worth, TX, USA Department of Neurology.
  • Brushaber D; Department of Health Sciences Research, Mayo Clinic, Rochester, MN, USA University of Pennsylvania, Philadelphia, PA, USA.
  • Caso C; Department of Neurology, University of Washington, Seattle, WA, USA Perelman School of Medicine, Department of Neurology.
  • Coppola G; Department of Neurology, University of California, Los Angeles, Los Angeles, CA, USA Case Western Reserve University, Cleveland, OH, USA.
  • Dever R; Department of Psychiatry and Biobehavioral Sciences, University of California, Los Angeles, Los Angeles, CA, USA University of California, Los Angeles, Los Angeles, CA, USA.
  • Dheel C; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA University of California, San Francisco, San Francisco CA, USA.
  • Dickerson BC; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Dickinson S; Department of Neurology, Frontotemporal Disorders Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Tau Consortium, Rainwater Charitable Foundation, Fort Worth, TX, USA.
  • Dominguez S; Association for Frontotemporal Degeneration, Radnor, PA, USA Mayo Clinic, Rochester, MN, USA.
  • Domoto-Reilly K; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA Department of Epidemiology and Biostatistics.
  • Faber K; Department of Neurology, University of Washington, Seattle, WA, USA Perelman School of Medicine, Department of Neurology.
  • Ferrall J; National Cell Repository for Alzheimer's Disease (NCRAD), Indiana University, Indianapolis, IN, USA National Cell Repository for Alzheimer's Disease (NCRAD), Indiana University, Indianapolis, IN, USA.
  • Fields JA; Department of Neurology, University of North Carolina, Chapel Hill, NC, USA University of Washington, Seattle, WA, USA.
  • Fishman A; Department of Psychiatry and Psychology, Mayo Clinic, Rochester, MN, USA Department of Psychiatry and Psychology, Mayo Clinic, Rochester, MN, USA.
  • Fong J; Department of Psychiatry, School of Medicine, Johns Hopkins University, Baltimore, MD, USA School of Medicine, Department of Psychiatry.
  • Foroud T; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA University of California, San Francisco, San Francisco CA, USA.
  • Forsberg LK; National Cell Repository for Alzheimer's Disease (NCRAD), Indiana University, Indianapolis, IN, USA National Cell Repository for Alzheimer's Disease (NCRAD), Indiana University, Indianapolis, IN, USA.
  • Gavrilova R; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Gearhart D; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Ghazanfari B; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Ghoshal N; Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Toronto, Ontario, Canada University of California, Los Angeles, Los Angeles, CA, USA.
  • Goldman J; Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
  • Graff-Radford J; Departments of Neurology and Psychiatry, Washington University School of Medicine, Washington University, St. Louis, MO, USA Mayo Clinic, Rochester, MN, USA.
  • Graff-Radford N; Department of Neurology, Columbia University, New York, NY, USA Department of Neurology.
  • Grant I; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, NY, USA Department of Neurology, Frontotemporal Disorders Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
  • Grossman M; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Haley D; Department of Neurology, Mayo Clinic, Jacksonville, FL, USA Association for Frontotemporal Degeneration, Radnor, PA, USA.
  • Heuer HW; Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA National Cell Repository for Alzheimer's Disease (NCRAD), Indiana University, Indianapolis, IN, USA.
  • Hsiung GY; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA Department of Epidemiology and Biostatistics.
  • Huey ED; Department of Neurology, Mayo Clinic, Jacksonville, FL, USA Association for Frontotemporal Degeneration, Radnor, PA, USA.
  • Irwin DJ; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA University of California, San Francisco, San Francisco CA, USA.
  • Jones DT; Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada University of North Carolina, Chapel Hill, NC, USA.
  • Jones L; Department of Neurology, Columbia University, New York, NY, USA Department of Neurology.
  • Kantarci K; Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, NY, USA Department of Neurology, Frontotemporal Disorders Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
  • Karydas A; Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA Department of Epidemiology and Biostatistics.
  • Kaufer DI; Department of Neurology, Mayo Clinic, Rochester, MN, USA Department of Neurology.
  • Kerwin DR; Department of Radiology, Washington University School of Medicine, Washington University, St. Louis, MO, USA Johns Hopkins University, Baltimore, MD, USA.
  • Knopman DS; Department of Radiology, Mayo Clinic, Rochester, MN, USA Department of Radiology.
  • Kraft R; Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco CA, USA University of California, San Francisco, San Francisco CA, USA.
  • Kramer JH; Department of Neurology, University of North Carolina, Chapel Hill, NC, USA University of Washington, Seattle, WA, USA.
  • Kremers WK; Department of Neurology and Neurotherapeutics, Center for Alzheimer's and Neurodegenerative Diseases, The University of Texas, Southwestern Medical Center at Dallas, Dallas, TX, USA Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto.
Alzheimers Dement ; 16(1): 37-48, 2020 01.
Article em En | MEDLINE | ID: mdl-31272932
ABSTRACT

INTRODUCTION:

Some models of therapy for neurodegenerative diseases envision starting treatment before symptoms develop. Demonstrating that such treatments are effective requires accurate knowledge of when symptoms would have started without treatment. Familial frontotemporal lobar degeneration offers a unique opportunity to develop predictors of symptom onset.

METHODS:

We created dementia risk scores in 268 familial frontotemporal lobar degeneration family members by entering covariate-adjusted standardized estimates of brain atrophy into a logistic regression to classify asymptomatic versus demented participants. The score's predictive value was tested in a separate group who were followed up longitudinally (stable vs. converted to dementia) using Cox proportional regressions with dementia risk score as the predictor.

RESULTS:

Cross-validated logistic regression achieved good separation of asymptomatic versus demented (accuracy = 90%, SE = 0.06). Atrophy scores predicted conversion from asymptomatic or mildly/questionably symptomatic to dementia (HR = 1.51, 95% CI [1.16,1.98]).

DISCUSSION:

Individualized quantification of baseline brain atrophy is a promising predictor of progression in asymptomatic familial frontotemporal lobar degeneration mutation carriers.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Atrofia / Predisposição Genética para Doença / Demência Frontotemporal / Mutação / Testes Neuropsicológicos Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2020 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Atrofia / Predisposição Genética para Doença / Demência Frontotemporal / Mutação / Testes Neuropsicológicos Tipo de estudo: Prognostic_studies / Risk_factors_studies Limite: Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2020 Tipo de documento: Article