A new clinical staging system for primary progressive aphasia
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1
University College London, United Kingdom
The primary progressive aphasias are a group of devastating language-led neurodegenerative diseases, comprising three major subtypes: semantic dementia (SD), progressive nonfluent aphasia (PNFA) and logopenic aphasia (LPA). To date, no staging system specific to PPA exists that allows clinicians, patients, or carers to ascertain and track progression of any of these diseases. We searched the UCL PPA Research Database for thirty people with a canonical form of SD (n=10), PNFA (n=10) and LPA (n=10). By reviewing clinical notes for these patients, we devised initial stages for each of the three major PPA syndromes, creating four stages for each (Stage 1 = Prodromal; Stage 2 = Mild; Stage 3 = Moderate; Stage 4 = Severe). We then sought feedback on these initial stages from carers of people living with PPA via an online survey that ran from October 2018 until January 2019 with members of the UK national PPA Support Group (www.raredementia.org/ppa), inviting people to give free-text responses on the stages relevant to them, and collecting basic demographic information about each patient included in the survey. 118 responses were received. Forty-eight of these only contained answers to the demographic questions; the remaining 70 contained both quantitative and qualitative responses. The qualitative responses were then analysed to assess the frequency of those symptoms included in the initial stages or those volunteered separately by survey respondents. The resulting stages were then scrutinised by caregiver focus groups and their feedback was used to further refine the formulation and ordering of the symptoms. This feedback indicated that our initial ‘four-stage’ system for each disease was not sensitive or discriminative enough to capture the range of symptom severity, and an expanded seven-stage approach was adopted instead for each syndrome, in line with Reisberg et al. (1982): Stage 1 = No impairment; Stage 2 = Very mild cognitive decline; Stage 3 = Mild cognitive decline; Stage 4 = Moderate cognitive decline; Stage 5 = Moderately severe cognitive decline; Stage 6 = Severe cognitive decline; Stage 7 = Very severe cognitive decline. Finally, the stages were validated retrospectively and prospectively in a separate validation cohort that did not participate in the initial characterisation of the stages. To our knowledge, this is the first clinical, symptom-led staging system that covers the three major PPA subtypes, and one of the few staging systems in neurodegenerative disease to be directly informed by the experience of people living with the illness. We hope that our proposed staging classification will be of use not only to clinicians who diagnose and research PPA, but also to those living with or supporting someone with PPA to track and anticipate changes in symptoms, and help guide counselling and interventions.
Acknowledgements
This abstract is part of the symposium “Aphasia in neurodegenerative conditions.”
References
Reisberg, B., Ferris, S. H., de Leon, M. J., & Crook, T. (1982). The Global Deterioration Scale for assessment of primary degenerative dementia. The American journal of psychiatry.
Keywords:
Semantic Dementia,
Progressive nonfluent aphasia,
Logopenic aphasia,
primary progressive aphasia,
Frontotemporal Dementia
Conference:
Academy of Aphasia 57th Annual Meeting, Macau, Macao, SAR China, 27 Oct - 29 Oct, 2019.
Presentation Type:
Symposium
Topic:
Not eligible for student award
Citation:
Hardy
CJ,
Suarez-Gonzalez
A,
Stott
J,
Camic
PM,
Rohrer
J,
Crutch
S and
Warren
J
(2019). A new clinical staging system for primary progressive aphasia.
Front. Hum. Neurosci.
Conference Abstract:
Academy of Aphasia 57th Annual Meeting.
doi: 10.3389/conf.fnhum.2019.01.00052
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Received:
06 May 2019;
Published Online:
09 Oct 2019.
*
Correspondence:
Dr. Chris J Hardy, University College London, London, United Kingdom, c.hardy.12@ucl.ac.uk