argyrophilic grain disease

argyrophilic grain disease

A common (5% of all dementias) sporadic neurodegenerative disease (tauopathy) of old age which is characterised by argyrophilic grains (AGs—dendritic-derived appendages) seen by Golgi stains, and pre-tangle neurons in the limbic system, which contain hyperphosphorylated 4R tau. Hyperphosphorylated tau also accumulates in oligodendroglia and limbic astrocytes.
Clinical findings Largely depend on the extent of AGs and other associated tauopathies, e.g., Alzheimer's disease, progressive supranuclear palsy, corticobasal degeneration, and synucleinopathies.
References in classic literature ?
It is not the least among the strange things bred by the intense artificialness of sea-usages, that while in the open air of the deck some officers will, upon provocation, bear themselves boldly and defyingly enough towards their commander; yet, ten to one, let those very officers the next moment go down to their customary dinner in that same commander's cabin, and straightway their inoffensive, not to say deprecatory and humble air towards him, as he sits at the head of the table; this is marvellous, sometimes most comical.
Several also would have shocked a delicate instinct by an appearance of artificialness indicating that there had been such commixture, and, as it were, adultery, of various vegetable species, that the production was no longer of God's making, but the monstrous offspring of man's depraved fancy, glowing with only an evil mockery of beauty.
The following findings do not exclude the application of CARTS terminology for reasons that are further clarified below: the absence of morphologic HS (although frequently present), the absence of severe cerebral arteriolosclerosis (although frequently present), and/or the presence of other degenerative brain diseases, including [alpha]-synucleinopathies, and non-AD tauopathies, such as PART, progressive supranuclear palsy, and argyrophilic grain disease.
No hereditary form of argyrophilic grain disease (AGD) has been described to date.