Background and purpose Argyrophilic grain disease (AGD) is a limbic-predominant 4R-tauopathy.
AGD is thought to be an age-related disorder and is frequently detected as a concomitant
pathology with other neurodegenerative conditions. There is a paucity of data on the
clinical phenotype of pure AGD. In elderly patients, however, AGD pathology frequently
associates with cognitive decline, personality changes, urine incontinence and cachexia.
In this study, clinicopathological findings were analysed in individuals younger than
75. Methods Patients were identified retrospectively based on neuropathological examinations
during 2006-2017 and selected when AGD was the primary and dominant pathological finding.
Clinical data were obtained retrospectively through medical records. Results In all,
55 patients (2% of all examinations performed during that period) with AGD were identified.
In seven cases (13%) AGD was the primary neuropathological diagnosis without significant
concomitant pathologies. Two patients were female, median age at the time of death
was 64 years (range 51-74) and the median duration of disease was 3 months (range
0.5-36). The most frequent symptoms were progressive cognitive decline, urinary incontinence,
seizures and psychiatric symptoms. Brain magnetic resonance imaging revealed mild
temporal atrophy. Conclusions Argyrophilic grain disease is a rarely recognized limbic
tauopathy in younger individuals. Widening the clinicopathological spectrum of tauopathies
may allow identification of further patients who could benefit from tau-based therapeutic
strategies.
