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argyrophilic grain disease (AgD)
Epidemiology:
1) disorder of the elderly, incidence increases with age
2) approximately 5% of all cases of dementia
Pathology:
1) abundant spindle-shaped argyrophilic grains in neuronal processes
2) coiled bodies in oligodendrocytes
3) hyperphosphorylated microtubule-associated protein tau largely in limbic regions
a) hippocampus
b) entorhinal cortex
c) transentorhinal cortex
d) amygdala
4) four-repeat (4R) tauopathy, similar to
a) progressive supranuclear palsy (PSP)
b) corticobasal degeneration (CBD) distinct from Alzheimer's disease (AD) & Pick's disease
Genetics:
- common genetic background regarding the tau gene haplotype has been suggested for AgD, PSP & CBD
Clinical manifestations:
1) clinically distinct from PSP & CBD
2) shares features of mild AD
General
neurodegenerative disease
References
- Probst A, Tolnay M.
[Argyrophilic grain disease (AgD), a frequent and largely
underestimated cause of dementia in old patients]
Rev Neurol (Paris). 2002 Feb;158(2):155-65. Review. French.
PMID: 11965171
- Tolnay M, Clavaguera F.
Argyrophilic grain disease: a late-onset dementia with
distinctive features among tauopathies.
Neuropathology. 2004 Dec;24(4):269-83. Review.
PMID: 15641585