In-vivo signatures of non-fluent/agrammatic primary progressive aphasia caused by FTLD pathology
| Autor: | Maya L. Henry, John Q. Trojanowski, Bruce L. Miller, Eric J. Huang, Maria Luisa Mandelli, William W. Seeley, Brianne M. Bettcher, Benno Gesierich, Jennifer M. Ogar, Massimo Filippi, Manu Sidhu, Nina F. Dronkers, Giancarlo Comi, Francesca Caso, Giuseppe Magnani, Maria Luisa Gorno-Tempini, Lea T. Grinberg |
|---|---|
| Přispěvatelé: | Caso, F, Mandelli, Ml, Henry, M, Gesierich, B, Bettcher, Bm, Ogar, J, Filippi, Massimo, Comi, Giancarlo, Magnani, G, Sidhu, M, Trojanowski, Jq, Huang, Ej, Grinberg, Lt, Miller, Bl, Dronkers, N, Seeley, Ww, Gorno Tempini, Ml |
| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Male
Pathology Aging Audiology Neurodegenerative Apraxia Primary progressive aphasia Dysarthria 80 and over 2.1 Biological and endogenous factors Prospective Studies Aetiology Alzheimer's Disease Related Dementias (ADRD) Aged 80 and over Rehabilitation Frontotemporal lobar degeneration Middle Aged Frontal Lobe DNA-Binding Proteins Frontotemporal Dementia (FTD) medicine.anatomical_structure Frontal lobe Neurological Female Cognitive Sciences medicine.symptom Psychology medicine.medical_specialty Apraxias Clinical Sciences tau Proteins Article White matter Atrophy Rare Diseases Progressive nonfluent aphasia Clinical Research mental disorders medicine Aphasia Acquired Cognitive Impairment Humans Primary Progressive Nonfluent Aphasia Aged Aphasia Broca Neurology & Neurosurgery Neurosciences Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD) medicine.disease nervous system diseases Brain Disorders Broca Dementia Neurology (clinical) Frontotemporal Lobar Degeneration |
| Zdroj: | Neurology, vol 82, iss 3 |
| Popis: | Objective: To identify early cognitive and neuroimaging features of sporadic nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) caused by frontotemporal lobar degeneration (FTLD) subtypes. Methods: We prospectively collected clinical, neuroimaging, and neuropathologic data in 11 patients with sporadic nfvPPA with FTLD-tau (nfvPPA-tau, n = 9) or FTLD–transactive response DNA binding protein pathology of 43 kD type A (nfvPPA-TDP, n = 2). We analyzed patterns of cognitive and gray matter (GM) and white matter (WM) atrophy at presentation in the whole group and in each pathologic subtype separately. We also considered longitudinal clinical data. Results: At first evaluation, regardless of pathologic FTLD subtype, apraxia of speech (AOS) was the most common cognitive feature and atrophy involved the left posterior frontal lobe. Each pathologic subtype showed few distinctive features. At presentation, patients with nfvPPA-tau presented with mild to moderate AOS, mixed dysarthria with prominent hypokinetic features, clear agrammatism, and atrophy in the GM of the left posterior frontal regions and in left frontal WM. While speech and language deficits were prominent early, within 3 years of symptom onset, all patients with nfvPPA-tau developed significant extrapyramidal motor signs. At presentation, patients with nfvPPA-TDP had severe AOS, dysarthria with spastic features, mild agrammatism, and atrophy in left posterior frontal GM only. Selective mutism occurred early, when general neurologic examination only showed mild decrease in finger dexterity in the right hand. Conclusions: Clinical features in sporadic nfvPPA caused by FTLD subtypes relate to neurodegeneration of GM and WM in frontal motor speech and language networks. We propose that early WM atrophy in nfvPPA is suggestive of FTLD-tau pathology while early selective GM loss might be indicative of FTLD-TDP. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|