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Argyrophilic grain disease

Grant number: 12/07526-5
Support type:Scholarships in Brazil - Doctorate (Direct)
Effective date (Start): August 01, 2012
Effective date (End): April 30, 2015
Field of knowledge:Health Sciences - Medicine - Pathological Anatomy and Clinical Pathology
Principal researcher:Lea Tenenholz Grinberg
Grantee:Roberta Diehl Rodriguez
Home Institution: Faculdade de Medicina (FM). Universidade de São Paulo (USP). São Paulo , SP, Brazil

Abstract

Argyrophilic grain disease (AGD) is a highly frequent degenerative sporadic tauopathy, but little diagnosed. It is morphologically characterized by the formation of hyperphosphorylated tau protein in specific and oligodendrocytic neurons. There is not a pathognomonic clinical presentation, with a slow evolution, mild cognitive impairment being the most common clinical manifestation. The only risk factor known to date is advanced age. The specific diagnosis for AGD is only made through post-mortem analysis. AGD follows a stereotyped pattern with early involvement of limbic system and later neo-cortex system. It is believed that the involvement of entorhinal cortex, hippocampus, temporal gyrus, and amygdala might be the anatomical substrate for cognitive alteration in AGD. Structures characterizing AGD neuropathologically are silver-positive grain-shaped structures known as argyrophilic grains (AGs) through the deposition of hyperphosphorylated tau in neurons in a pre-wired form, and in oligodendrocytes in a corpuscular form in embryos. Other findings that can be found are ballooned neurons and bush-like astrocytes. Neuropathological criteria for AGD were described, but none of them was formally accepted. AGD is often associated to other neurodegenerative diseases, with most of them presenting pathological Alzheimer-like alterations typically mild to moderate with corresponding distribution to Braak I through III stages. Despite the prevalence of AGs little is really known about risk factors, as potential trigger factors. The most accepted hypothesis is that AGD is an independent pathology. Few studies carried out with AGD assess cognitively normal donors, without previous history of cognitive alteration. Therefore, the casuistry of the Bank of Human Encephala at University of São Paulo will be highly important to perform this study, since it has a significant number of cases with history of previous cognitive alteration. Thus, this study aims at estimating the prevalence of AGD in donors of encephalon from the Bank of human encephala, determining their epidemiological profile while comparing cases versus controls, staging cases with positive grains according to scales recently proposed, assessing APoe genotype, and determining the relationship between AGD and cognition. (AU)

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Scientific publications (4)
(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)
RODRIGUEZ, ROBERTA DIEHL; SUEMOTO, CLAUDIA KIMIE; MOLINA, MARIANA; NASCIMENTO, CAMILA FERNANDES; LEITE, RENATA ELAINE PARAIZO; DE LUCENA FERRETTI-REBUSTINI, RENATA ELOAH; FARFEL, JOSE MARCELO; HEINSEN, HELMUT; NITRINI, RICARDO; UEDA, KENJI; et al. d Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, v. 75, n. 7, p. 628-635, . (12/07526-5, 11/19833-7, 10/06521-4)
ROBERTA DIEHL RODRIGUEZ; LEA TENENHOLZ GRINBERG. Doença com grãos argirofílicos: uma taupatia subestimada. Dement. Neuropsychol., v. 9, n. 1, p. 2-8, . (12/07526-5)
SUEMOTO, CLAUDIA K.; FERRETTI-REBUSTINI, RENATA E. L.; RODRIGUEZ, ROBERTA D.; LEITE, RENATA E. P.; SOTERIO, LUCIANA; BRUCKI, SONIA M. D.; SPERA, RAPHAEL R.; CIPPICIANI, TARCILA M.; FARFEL, JOSE M.; CHIAVEGATTO FILHO, ALEXANDRE; et al. Neuropathological diagnoses and clinical correlates in older adults in Brazil: A cross-sectional study. PLOS MEDICINE, v. 14, n. 3, . (12/07526-5, 09/09134-4)
NASCIMENTO, CAMILA; SUEMOTO, CLAUDIA K.; RODRIGUEZ, ROBERTA D.; DI LORENZO ALHO, ANA TEREZA; LEITE, RENATA P.; FARFEL, JOSE MARCELO; GONCALVES PASQUALUCCI, CARLOS AUGUSTO; JACOB-FILHO, WILSON; GRINBERG, LEA T.. Higher Prevalence of TDP-43 Proteinopathy in Cognitively Normal Asians: A Clinicopathological Study on a Multiethnic Sample. Brain Pathology, v. 26, n. 2, p. 177-185, . (12/07526-5, 11/19833-7, 10/06521-4)
Academic Publications
(References retrieved automatically from State of São Paulo Research Institutions)
RODRIGUEZ, Roberta Diehl. Argyrophilic grain disease. 2015. Doctoral Thesis - Universidade de São Paulo (USP). Faculdade de Medicina (FM/SBD) São Paulo.

Please report errors in scientific publications list by writing to: cdi@fapesp.br.