Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study.
Argyrophilic grain disease (AGD) is a frequent late-onset, 4-repeat tauopathy reported in Caucasians with high educational attainment. Little is known about AGD in non-Caucasians or in those with low educational attainment. We describe AGD demographics, clinical, and neuropathological features in a multiethnic cohort of 983 subjects ≥50 years of age from São Paulo, Brazil. Clinical data were collected through semistructured interviews with an informant and included in the Informant Questionnaire on Cognitive Decline in the Elderly, the Clinical Dementia Rating, and the Neuropsychiatric Inventory. Neuropathologic assessment relied on internationally accepted criteria. AGD was frequent (15.2%) and was the only neuropathological diagnosis in 8.9% of all cases (mean, 78.9 ± 9.4 years); it rarely occurred as an isolated neuropathological finding. AGD was associated with older age, lower socioeconomic status (SES), and appetite disorders. This is the first study of demographic, clinical, and neuropathological aspects of AGD in different ethnicities and subjects from all socioeconomic strata. The results suggest that prospective studies of AGD patients include levels of hormones related to appetite control as possible antemortem markers. Moreover, understanding the mechanisms behind higher susceptibility to AGD of low SES subjects may disclose novel environmental risk factors for AGD and other neurodegenerative diseases.
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Roberta Diehl
RD
Rodriguez
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Claudia Kimie
CK
Suemoto
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Mariana
M
Molina
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Camila Fernandes
CF
Nascimento
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Renata Elaine Paraizo
RE
Leite
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Renata Eloah
RE
de Lucena Ferretti-Rebustini
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
José Marcelo
JM
Farfel
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Helmut
H
Heinsen
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Ricardo
R
Nitrini
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Kenji
K
Ueda
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Carlos Augusto
CA
Pasqualucci
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Wilson
W
Jacob-Filho
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY).
Kristine
K
Yaffe
From the Discipline of Pathophysiology (RDR, MM, CFN), Behavioral and Cognitive Neurology Unit, Department of Neurology (RDR, RN), Brazilian Brain Bank of the Aging Brain Study Group, LIM-22 (CKS, REPL, REdLF-R, JMF, HH, RN, CAP, WJ-F, LTG), Discipline of Geriatrics, University of São Paulo, São Paulo, Brazil (CKS, REPL, JMF, WJ-F); Medical-Surgical Nursing Department, University of São Paulo School of Nursing, São Paulo, Brazil (REdLF-R); Department of Pathology, University of São Paulo, São Paulo, Brazil (HH, CAP, LTG); Department of Psychiatry, Morphological Brain Research Unit, University of Würzburg, Würzburg, Germany (HH); Department of Neurochemistry, Tokyo Institute of Psychiatry, Setagaya-ku/Tokyo, Japan (KU); Memory and Aging Center, Department of Neurology and Pathology (KY, LTG); and Department of Psychiatry, University of California, San Francisco, California (KY). lea.grinberg@ucsf.edu.
Lea Tenenholz
LT
Grinberg
D000368:Aged; D000369:Aged, 80 and over; D001344:Autopsy; D001938:Brazil / Q000453:epidemiology; D003710:Demography / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000453:epidemiology / Q000473:pathology / Q000523:psychology; D024801:Tauopathies / Q000453:epidemiology* / Q000473:pathology* / Q000523:psychology
Dementia; Neurodegeneration; Neuropathology; Postmortem; Tauopathy