Dementia with Grains (Argyrophilic Grain Disease)

  title={Dementia with Grains (Argyrophilic Grain Disease)},
  author={Kurt A. Jellinger},
  journal={Brain Pathology},
Dementia with grains, also referred to as argyrophilic grain disease, is a morphological condition in elderly individuals histologically characterised by the widespread occurrence of minute, spindle or comma‐shaped argyrophilic, tau‐immunoreactive structures distinct from neuropil threads that are predominantly located in the hippocampus and related limbic areas including the amygdala. They are suggested to arise mainly in dendrites of neurons showing accumulation of hyperphosphorylated tau… 
Argyrophilic grain disease: A late‐onset dementia with distinctive features among tauopathies
  • M. Tolnay, F. Clavaguera
  • Biology
    Neuropathology : official journal of the Japanese Society of Neuropathology
  • 2004
Argyrophilic grain disease seems to be clinically distinct from PSP and CBD and shares rather features of (mild) AD or other forms of ‘limbic’ dementias, among them senile dementia with tangles and the localized form of AD.
Severe Involvement of Ambient Gyrus in Dementia with Grains
It is suggested that selective severe involvement of the ambient gyrus may explain the clinical manifestations of a limbic-type dementia in DG, in contrast to Alzheimer disease.
Argyrophilic grain disease.
Argyrophilic grain disease (AGD) is a common sporadic neurodegenerative disease of old age characterized by the presence of argyrophilic grains (AGs)--dendritic-derived appendages as revealed with
Argyrophilic grain disease: an update about a frequent cause of dementia
This review aims to provide an up-to-date overview of AGD, emphasizing pathological aspects and the findings of a Brazilian case series, as well as to demonstrate a distinct clinical syndrome associated with AGD.
Argyrophilic grain disease: a clinicopathological review of an overlooked tauopathy.
It is believed that patients above the age of 60 years should undergo post-mortem screening for AGD to avoid missed opportunity for diagnosis and enable future clinicopathological studies.
Severe involvement of the ambient gyrus in a case of dementia with argyrophilic grain disease
Argyrophilic grain disease and Alzheimer's disease are distinguished by their different distribution of tau protein isoforms
It is demonstrated that AgD is characterized by a major tau doublet that is distinct from AD and PiD and in contrast to earlier immunohistochemical studies, it is shown biochemically that Ser262 indeed is phosphorylated in the PTP of AgD.
Staging of Argyrophilic Grains: An Age‐Associated Tauopathy
The study confirms that dementia with grains is an age-associated tauopathy with relatively uniform distribution and may independently contribute to cognitive decline in the elderly and propose the following staging paradigm.
Argyrophilic Grain Disease Is a Sporadic 4‐Repeat Tauopathy
The results suggest that AGD, PSP and CBD are 4R tauopathies that share common pathologic, biochemical, and genetic characteristics.
Diffuse form of argyrophilic grain disease: a new variant of four-repeat tauopathy different from limbic argyrophilic grain disease
A study on two AGD patients, belonging to a series of demented patients affected by several tauopathies, prospectively followed until death, suggests that “diffuse” AGD might be a subgroup of AGD, the specific profile of which is different from that of “limbic’ AGD.


Late onset dementia with argyrophilic grains and subcortical tangles or atypical progressive supranuclear palsy?
It is suggested that the late onset dementia with argyrophilic grains syndrome is also characterized by the presence of tangles and threads with the topographical distribution of progressive supranuclear palsy.
Argyrophilic grain disease: frequency of occurrence in different age categories and neuropathological diagnostic criteria
The fact that the same material contained 146 cases of fully developed Alzheimer's disease (6%) supports the view that argyrophilic grain disease is not a rare disorder.
Argyrophilic grain disease: distribution of grains in patients with and without dementia
The results show that the intellectual status of patients with AgD was related to the extension of ArG in the limbic area, suggesting that AgD is a progressive neurodegenerative disorder with early subclincial lesions in the anterior part of the hippocampal formation.
Argyrophilic grain disease: widespread hyperphosphorylation of tau protein in limbic neurons
Unlike in AD, tau hyperphosphorylation in the somatodendritic domain of neurons in AgD does not appear to be followed by neurofibrillary tangle formation, even in the presence of widespread ArG in the neuropil.
Argyrophilic grains: characteristic pathology of cerebral cortex in cases of adult onset dementia without Alzheimer changes
Prevalence and Disease Associations of Argyrophilic Grains of Braak
It is concluded that rather than defining a single disease, BAG constitute lesions that accompany several degenerative diseases, but also occur in normal elderly subjects, and rarely in demented subjects without other major histological findings.
A case of adult‐onset dementia with argyrophilic grains
Although the case was clinically indistinguishable from Alzheimer's disease, plaque and tangle pathological findings were absent and cortical changes of the type described by Braak and Braak are confirmed and additional data on subcortical changes in this case is provided.
Some Immunohistochemical Features of Argyrophilic Grain Dementia With Normal Cortical Choline Acetyltransferase Levels but Extensive Subcortical Pathology and Markedly Reduced Dopamine
Detailed immunohistochemical and biochemical studies are reported on two cases of progressive dementia showing no Alzheimer-type pathology but extensive argyrophilic grains, which may be consistent with the criteria for progressive subcortical gliosis.
Senile Dementia with Tangles (Tangle Predominant Form of Senile Dementia)
Senile dementia with tangles is suggested to be a variant of Alzheimer disease occurring in the oldest‐old, but its nosological position within aging disorders of the brain is still controversy.
Subcortical neurofibrillary tangles, neuropil threads, and argentophilic glial inclusions in corticobasal degeneration.
The results of the present study indicate that the cytopathology of the subcortical gray matter and brainstem in CBD patients resembles that of progressive supranuclear palsy.