Argyrophilic grain disease: An underestimated tauopathy

Argyrophilic grain disease (AGD) is an under-recognized, distinct, highly frequent sporadic tauopathy, with a prevalence reaching 31.3% in centenarians. The most common AGD manifestation is slowly progressive amnestic mild cognitive impairment, accompanied by a high prevalence of neuropsychiatric symptoms. AGD diagnosis can only be achieved postmortem based on the finding of its three main pathologic features: argyrophilic grains, oligodendrocytic coiled bodies and neuronal pretangles. AGD is frequently seen together with Alzheimer's disease-type pathology or in association with other neurodegenerative diseases. Recent studies suggest that AGD may be a defense mechanism against the spread of other neuropathological entities, particularly Alzheimer's disease. This review aims to provide an in-depth overview of the current understanding on AGD.

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Molecular evolution and genetics of the Saitohin gene and tau haplotype in Alzheimer's disease and argyrophilic grain disease

Journal of Neurochemistry ◽

10.1046/j.1471-4159.2004.02320.x ◽

2004 ◽

Vol 89 (1) ◽

pp. 179-188 ◽

Cited By ~ 38

Author(s):

Chris Conrad ◽

Cintia Vianna ◽

Christian Schultz ◽

Dietmar R. Thal ◽

Estifanos Ghebremedhin ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Molecular Evolution ◽

Argyrophilic Grain Disease ◽

Argyrophilic Grain

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P3-186 Concurrent Alzheimer's disease-related pathology lowers the threshold for developing dementia in argyrophilic grain disease

Neurobiology of Aging ◽

10.1016/s0197-4580(04)81338-1 ◽

2004 ◽

Vol 25 ◽

pp. S408-S409

Author(s):

Dietmar R. Thal ◽

Christian Schultz ◽

Giovannina Botez ◽

Mrak E. Robert ◽

Sue T. Griffin ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Argyrophilic Grain Disease ◽

Argyrophilic Grain

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Neuronal Hemoglobin is Reduced in Alzheimer's Disease, Argyrophilic Grain Disease, Parkinson's Disease, and Dementia with Lewy Bodies

Journal of Alzheimer s Disease ◽

10.3233/jad-2010-101485 ◽

2011 ◽

Vol 23 (3) ◽

pp. 537-550 ◽

Cited By ~ 47

Author(s):

Isidro Ferrer ◽

Ana Gómez ◽

Margarita Carmona ◽

Gema Huesa ◽

Silvia Porta ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Parkinson’S Disease ◽

Alzheimer's Disease ◽

Parkinson's Disease ◽

Dementia With Lewy Bodies ◽

Lewy Bodies ◽

Argyrophilic Grain Disease ◽

Argyrophilic Grain

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Argyrophilic grain disease: An update on a frequent cause of dementia

Dementia & Neuropsychologia ◽

10.1590/s1980-57642009dn30100002 ◽

2009 ◽

Vol 3 (1) ◽

pp. 2-7 ◽

Cited By ~ 13

Author(s):

Lea T. Grinberg ◽

Helmut Heinsen

Keyword(s):

Cognitive Decline ◽

Late Onset ◽

Case Series ◽

Clinical Syndrome ◽

Argyrophilic Grain Disease ◽

Coiled Bodies ◽

Behavioral Abnormalities ◽

Argyrophilic Grain ◽

Neurodegenerative Dementias ◽

Argyrophilic Grains

Abstract Argyrophilic grain disease (AGD) is a sporadic, very late-onset tauopathy, accounting for approximately 4-13% of neurodegenerative dementias. AGD may manifest with a range of symptoms such as cognitive decline and behavioral abnormalities. To date, no study has been able to demonstrate a distinct clinical syndrome associated with AGD. The diagnosis is exclusively based on postmortem findings, the significance of which remains controversial because up to 30% of AGD cases are diagnosed in subjects without any cognitive impairment, while AGD findings often overlap with those of other neurodegenerative processes. Nevertheless, the presence of AGD is likely to have a significant effect on cognitive decline. The neuropathological hallmarks of AGD are argyrophilic grains, pre-neurofibrillary tangles in neurons and coiled bodies in oligodendrocytes found mainly in the entorhinal cortex and hippocampus. This review aims to provide an up-to-date overview of AGD, emphasizing pathological aspects. Additionally, the findings of a Brazilian case series are described.

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