Ulugut, H;
Stek, S;
Wagemans, LEE;
Jutten, RJ;
Keulen, MA;
Bouwman, FH;
Prins, ND;
... Pijnenburg, YAL; + view all
(2021)
The natural history of primary progressive aphasia: beyond aphasia.
Journal of Neurology
10.1007/s00415-021-10689-1.
(In press).
Preview |
Text
Ulugut2021_Article_TheNaturalHistoryOfPrimaryProg.pdf - Published Version Download (2MB) | Preview |
Abstract
Introduction: Primary progressive aphasia (PPA) is divided into three prototypical subtypes that are all characterized by their single core symptom of aphasia. Although later in their course, other cognitive, behavioral, and motor domains may become involved, little is known about the progression profile of each subtype relative to the other subtypes. Methods: In this longitudinal retrospective cohort study, based on the recent biomarker-supported diagnostic criteria, 24 subjects diagnosed with semantic variant (svPPA), 22 with non-fluent variant (nfvPPA), and 18 with logopenic variant (lvPPA) were collected and followed up for 1–6 years. Symptom distribution, cognitive test and neuropsychiatric inventory scores, and progression into another syndrome were assessed. Results: Over time, lvPPA progressed with broader language problems (PPA-extended) and nfvPPA progressed to mutism, whereas semantic impairment remained the major problem in svPPA. Apart from linguistic problems, svPPA developed pronounced behavioral disturbances, whereas lvPPA exhibited a greater cognitive decline. By contrast, in nfvPPA motor deficits were more common. Furthermore, within 5 years (IQR = 2.5) after clinical onset, 65.6% of the patients additionally fulfilled the clinical criteria for another neurodegenerative syndrome (PPA-plus). Fourteen out of 24 (58%) svPPA patients additionally met the diagnostic criteria of behavioral variant frontotemporal dementia (5.1 years, IQR = 1.1), whereas the clinical features of 15/18 (83%) lvPPA patients were consistent with Alzheimer disease dementia (4.5 years IQR = 3.4). Furthermore, 12/22 (54%) of the subjects with the nfvPPA progressed to meet the diagnostic criteria of corticobasal syndrome, progressive supranuclear palsy, or motor neuron disease (5.1 years IQR = 3.4). Discussion: Despite aphasia being the initial and unique hallmark of the syndrome, our longitudinal results showed that PPA is not a language limited disorder and progression differs widely for each subtype, both with respect to the nature of symptoms and disease duration.
| Type: | Article |
|---|---|
| Title: | The natural history of primary progressive aphasia: beyond aphasia |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1007/s00415-021-10689-1 |
| Publisher version: | https://doi.org/10.1007/s00415-021-10689-1 |
| Language: | English |
| Additional information: | This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences & Neurology, Dementia, Frontotemporal lobar degeneration, Frontotemporal dementia, Aphasia, Primary progressive aphasia, Mortality, Survival analysis, Natural history, CORTICOBASAL DEGENERATION, SUPRANUCLEAR PALSY, LOGOPENIC VARIANT, BEHAVIORAL VARIANT, NONFLUENT APHASIA, DIAGNOSIS, DEMENTIA, CRITERIA, SUBTYPES, SYMPTOMS |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Brain Repair and Rehabilitation |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10131911 |
Archive Staff Only
 |
View Item |
