Argyrophilic grain disease presenting as behavioral frontotemporal dementia

2019 ◽  
Vol 38 (01) ◽  
pp. 8-13 ◽  
Author(s):  
María José Gil ◽  
Soledad Serrano ◽  
María Sagrario Manzano ◽  
María Luz Cuadrado ◽  
Elena Góméz ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Argyrophilic Grain Disease ◽  
Argyrophilic Grain

scholarly journals Early vs late age at onset frontotemporal dementia and frontotemporal lobar degeneration

Neurology ◽  
2018 ◽  
Vol 90 (12) ◽  
pp. e1047-e1056 ◽  
Author(s):  
Sang Won Seo ◽  
Marie-Pierre Thibodeau ◽  
David C. Perry ◽  
Alice Hua ◽  
Manu Sidhu ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Symptom Onset ◽  
Frontotemporal Lobar Degeneration ◽  
Age At Onset ◽  
Dna Binding Protein ◽  
Clinical Syndrome ◽  
Early Age ◽  
Consecutive Series ◽  
Argyrophilic Grain Disease ◽  
Argyrophilic Grain

ObjectiveTo examine clinicopathologic correlations in early vs late age at onset frontotemporal dementia (FTD) and frontotemporal lobar degeneration (FTLD).MethodsAll patients were clinically evaluated and prospectively diagnosed at the UCSF Memory and Aging Center. Two consecutive series were included: (1) patients with a clinically diagnosed FTD syndrome who underwent autopsy (cohort 1) and (2) patients with a primary pathologic diagnosis of FTLD, regardless of the clinical syndrome (cohort 2). These series were divided by age at symptom onset (cutoff 65 years).ResultsIn cohort 1, 48 (25.3%) were 65 years or older at symptom onset. Pathologic causes of behavioral variant FTD (bvFTD) were similar in the early age at onset (EO) and late age at onset (LO) bvFTD groups. In corticobasal syndrome (CBS), however, the most common pathologic substrate differed according to age at onset: progressive supranuclear palsy (42.9%) in LO-CBS and Alzheimer disease (AD; 40.7%) in EO-CBS. In cohort 2, 57 (28.4%) were classified as LO-FTLD. Regarding FTLD major molecular classes, FTLD with transactive response DNA-binding protein of 43 kDa was most common in EO-FTLD (44.4%), whereas FTLD-tau (58.3%) was most common in LO-FTLD. Antemortem diagnosis of a non-FTD syndrome, usually AD-type dementia, was more frequent in LO-FTLD than EO-FTLD (19.3% vs 7.7%, p = 0.017). LO-FTLD was also associated with more prevalent comorbid pathologic changes. Of these, moderate to severe AD neuropathologic change and argyrophilic grain disease were overrepresented among patients who received an antemortem diagnosis of AD-type dementia.ConclusionPatients with FTD and FTLD often develop symptoms after age 65, and age at onset represents an important consideration when making antemortem neuropathologic predictions.

Argyrophilic grain disease presenting with frontotemporal dementia: A neuropsychological and pathological study of an autopsied case with presenile onset

2005 ◽  
Vol 25 (2) ◽  
pp. 165-170 ◽  
Author(s):  
Kenji Ishihara ◽  
Shigeo Araki ◽  
Nami Ihori ◽  
Jun-ichi Shiota ◽  
Mitsuru Kawamura ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Pathological Study ◽  
Argyrophilic Grain Disease ◽  
Argyrophilic Grain

scholarly journals Argyrophilic grain disease: An underestimated tauopathy

2015 ◽  
Vol 9 (1) ◽  
pp. 2-8 ◽  
Author(s):  
Roberta Diehl Rodriguez ◽  
Lea Tenenholz Grinberg
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Defense Mechanism ◽  
Neuropsychiatric Symptoms ◽  
Argyrophilic Grain Disease ◽  
Coiled Bodies ◽  
Pathologic Features ◽  
High Prevalence ◽  
Argyrophilic Grain ◽  
Argyrophilic Grains

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.

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

2004 ◽  
Vol 89 (1) ◽  
pp. 179-188 ◽  
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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