Search

Background: Patients with Primary Progressive Aphasias (PPAs) almost systematically inquire about the longitudinal evolution of their disease in clinics but very little research exists on the issue. Method: We studied 82 PPA patients from the Research Chair on PPA – Fondation de la Famille Lemaire Cohort over a 10‐year span (42 logopenic, 21 non‐fluent/agrammatic and 19 semantic PPAs) and collected data from 5 domains (language, cognition, motor, psychiatric, functional) at 5 time points from onset to death. Logistical regression analyses and repeated measures ANOVAs were conducted to delineate the longitudinal profile of each variant PPA. Result: All patients presented anomia and executive impairments over time. Language deficits tended to be more significant for lvPPA, particularly after 3 years of evolution and this group showed broader cognitive impairments. Psychiatric symptoms were more frequent in svPPA and nfvPPA, particularly after 5 years of evolution. Motor features predominantly affected patients with nfvPPA after 2 years of evolution. Overall functional abilities remained preserved the longest in svPPA (up to 5 years). Conclusion: To our knowledge, this naturalistic study on all major PPA symptoms over a 10‐year span from onset to death is the largest to date. Data from this study can help clinicians better inform and prepare their patients for future challenges as well as design more focused interventions. [ABSTRACT FROM AUTHOR]

Background: For over 25 years, cholinesterase inhibitors (ChEIs) have been the main symptomatic treatment for Alzheimer's disease (AD). Several meta‐analyses have supported their effectiveness in various neurocognitive aspects of amnestic AD. Over 86% of cases of the logopenic variant of primary progressive aphasia (lvPPA) are caused by a similar pathologic process than AD, yet no study has examined the efficacy of ChEIs in this AD variant. We aimed to explore the efficacy of ChEIs in the treatment of lvPPA by comparing their longitudinal MMSEs to that of treated amnestic AD patients. Method: A retrospective chart review was performed on 48 patients with lvPPA and 52 patients with amnestic AD. Both groups were similar with respect to age, level of education, and onset of symptoms. Drug history and MMSE scores were collected on several time points before and after the introduction of ChEIs. Results: Patients with lvPPA showed a similar trajectory of decline than amnestic AD patients on serial MMSEs (both WORLD and 100‐7) up to twelve months after the introduction of ChEIs (Figure 1 and 2). Conclusion: This study provides preliminary evidence for efficacy of ChEIs in patients with lvPPA and suggests similar benefits to those seen in amnestic AD patients. [ABSTRACT FROM AUTHOR]

Background: Initial language impairment in lvPPA causes naming and repetition difficulties along with phonological errors (Gorno‐Tempini et al., 2011). Little is known about the evolution of other language capacities with disease progression. This study aimed at exploring the full range of language impairments throughout the course of the disease, from onset to death. Method: We performed a retrospective chart review on a large cohort of deceased lvPPA patients (n = 38; 18 M, 19 F; Mean age at onset = 69 years old). Presence or absence of impairment in various language abilities was determined via consensus by three experienced raters at 5 time points from onset to death. Result: All 38 patients developed anomia, repetition impairment and writing difficulties throughout disease course (Figure 1). The overall prevalence of reading difficulties was also high (Figure 1), with impairment appearing more than two years post‐onset in most cases (Figure 2). Comprehension deficits were highly prevalent, appearing mostly in the first two years post onset (Figure 2). More than 75% of patients showed grammar difficulties (Figure 1), mainly in later stages of the disease (Figure 2). Semantic deficits also appeared later (Figure 2). Finally, apraxia of speech was quite infrequent (Figure 1). Conclusion: In addition to known language deficits in lvPPA (i.e., naming, repetition, reading and writing, see Brambati et al., 2009; Faria et al., 2013; Gorno‐Tempini et al., 2011; Sepelyak et al., 2011), our results suggest early impairments in comprehension as well as late grammar difficulties. To our knowledge, this is the first study which addresses the full range of language impairments in lvPPA over the entire course of the disease. We believe this is highly relevant for patients/families to better prepare for the future, as well as therapists to design more focused interventions. [ABSTRACT FROM AUTHOR]

Background: Very limited web‐based information is available for patients and families affected by Primary Progressive Aphasia (PPA). When accessible, it is often incomplete or worded in complex terminology. One of the objectives of the Research Chair on PPA – Fondation de la famille Lemaire (app‐ffl.ulaval.ca) is to educate the general population and healthcare professionals on PPA to promote earlier diagnosis and better care. We present the development of the PPA Platform, an innovative knowledge transfer tool. Method: We carried out a focus group with patients and their relatives to understand their needs. We developed templates for 'Patients/Relatives' and 'Health Professionals' and adapted content and terminology for both sections. Content was then validated with experts in the field (neurologists, SLP). Videos were selected to illustrate the symptoms (eg, naming, repetition, etc.) and we included testimonials to further capture caregivers' experience (eg, first symptoms, challenges, etc.). The French version of the platform was launched in 2020 and the English one in 2022. Result: Since its inauguration, the PPA Platform has been consulted by more than 27 000 individuals in over 85 countries. Moreover, its content has been presented to approximately 500 healthcare professionals over training sessions either in person or by videoconference. Content is regularly updated and translation in other languages (Mandarin, Hindi, Spanish, Arabic and German) is ongoing. Conclusion: The PPA Platform is an outstanding knowledge translation tool for patients, families and healthcare professionals. In a short amount of time, it positioned itself worldwide as a key reference on PPA. [ABSTRACT FROM AUTHOR]

Background: The logopenic variant of primary progressive aphasia (lvPPA) is known to be associated with behavioral and psychological symptoms of dementia (BPSD) such as depression and apathy. However, few data are available regarding the evolution of BPSD, particularly in advanced stages and end of life, which is often of great concern for patients and families. Method: We performed a retrospective chart review in a cohort including 42 deceased patients with a diagnosis of lvPPA. Symptoms of BPSD were assessed from disease onset to death by three experienced raters and, when possible, interviews were performed with proxies of patients to complete the missing data. Result: Depression and anxiety were mostly prevalent at the early stages but decreased over time. In particular, at one‐year after first visit, 36% of the patients were affected by anxiety symptoms and 19% were suffering from depressive symptoms. Apathy increased with the follow‐up with a peak at 5 years from the first clinical visit (46% of the patients). Other BPSD such as rituals, hyperorality, or agitation were less prevalent throughout the disease. Nevertheless, patients frequently presented with dishinibition during the follow‐up (between 20‐36% of the patients during the 5 years following the first visit). Antidepressants were used in around 20‐28% of the patients from first visit to later stages, followed by anxiolytics (3‐22%). Antipsychotics were mostly used in the advanced stages. At the first visit, 42% of the patients were treated with acetylcholinesterase inhibitors. During the follow up, this rate fluctuated between 40‐64%. Conclusion: Affective symptoms were most prevalent in prodromal and early stages, whereas in the advanced stages, lvPPA was mostly characterized by apathy. Dishinibition was also frequent during the follow‐up. These findings could support a psychiatric assessment in the early stages, since depression and anxiety are known to respond well to psychotropic treatment. [ABSTRACT FROM AUTHOR]

Background: Language impairment is an important marker of neurocognitive disorders. Despite this, there is no universal system of terminology used to describe speech impairment and large inter‐rater variability can exist between clinicians assessing speech. The role of automated speech analysis is emerging as a novel and potentially more objective method to assess speech in individuals with mild cognitive impairment (MCI) and Alzheimer's dementia (AD). No studies have analyzed whether variables extracted through automated speech analysis can also be correlated to speech characteristics identified by a clinician. We sought to: (1) investigate whether clinician‐identified speech characteristics could be correlated with acoustic and linguistic variables identified through automated speech analysis, and (2) through automated speech analysis, identify novel acoustic and linguistic variables that may be associated with MCI or AD. Method: Using the DementiaBank speech corpus (Cookie Theft picture description task), audio recordings from patients with possible/probable AD (n=10), MCI (n=10), and controls (n=10) were rated by clinicians. Four characteristics of speech were rated by clinicians, including: word‐finding difficulty, coherence, perseveration, and speech errors. Clinicians were blinded to each other's scoring. Where scores differed, a consensus rating was established. The speech recordings were then transcribed, and linguistic and acoustic variables were extracted through automated speech analysis. The correlation between clinician‐identified speech characteristics and the acoustic and linguistic variables were then compared. Result: A significant correlation (p < 0.05) was found between clinician‐identified speech characteristics and variables extracted through automated analysis. Average word length was correlated with word‐finding difficulty (ρ = 0.74); changes in syntactic construction (use of past tense, third person singular verbs, and subordinate clauses) were correlated with coherence (ρ = 0.51) and errors in speech (ρ = 0.58); similarity between consecutive utterances was correlated to perseveration (ρ = 0.68). Conclusion: In this exploratory study, variables extracted through automated acoustic and linguistic analysis of MCI and AD speech were strongly correlated to clinician‐identified speech characteristics. Our results suggest further investigation for using an automated, data‐driven approach to define and monitor subjective clinical speech descriptors in neurocognitive disorders. [ABSTRACT FROM AUTHOR]

Background: Primary progressive aphasias (PPAs) are a group of neurodegenerative disorders characterized by an insidious and progressive language impairment. Clinical expression and underlying pathology remain highly heterogeneous. To date, there is a lack of knowledge on the natural history of each of the three main variants, particularly mortality. Moreover, advanced stages and end of life issues are rarely discussed with caregivers and families at diagnosis. In turn, this can cause additional psychological distress.We studied the natural history of the three main variants of PPA. More specifically, we focused on mortality and end of life issues in an attempt to better prepare patients and their families. Method: We retrospectively studied all deceased patients with a diagnosis of PPA that had been followed at our tertiary memory clinic over the past twenty years (n=83). Clinical data included age of onset of symptoms, duration of illness, age at death and specific cause of death. Result: Age at death was similar across all three PPA variants but there was less variability in the non‐fluent variant (nfvPPA) group (M=76.6, SD=6.2) than in the semantic variant (svPPA; M=74.1, SD=8.5) or the logopenic variant (lvPPA; M=76.1, SD=9.1) groups. Age at onset of symptoms was earlier for svPPA participants (M=64.5, SD=8.3) then for the two other variants (lvPPA: M=69.3, SD= 10.7; nfvPPA: M=70.4, SD=6.3). Consequently, duration of the disease (number of years between symptoms' onset and death) was longer for svPPA participants (M=9.6, SD=3.0) than for nfvPPA and lvPPA participants (respectively M=6.2, SD=2.1 and M=7.0, SD=2.9). Surprisingly, cause of death was unknown for most of the participants. When available (n=15), it included sepsis (n=6), respiratory failure (n=4), neoplasia (n=3) and stroke (n=2), and. There were no differences in causes of death among PPA variants. Conclusion: Despite similar age of death across PPA variants, disease duration was longer in the svPPA. Causes of death per se were similar among PPAs. Future studies should include more participants with better emphasis on causes of death. Nonetheless, our study provides useful information about the natural history of the common variants of PPA. [ABSTRACT FROM AUTHOR]