Rhythm and speech rate as a diagnostic aid in neurodegenerative syndromes of the front-temporal lobes

Temporal variables have proven to be an effective tool to characterize speech in neurodegenerative diseases and primary progressive aphasia variants, but the evidence available so far is essentially limited to English. This article analyses, by means of a small sample of seven patient groups and a control group (total n = 34; control group = 6; progressive primary aphasia, en three variants = 15; fronto-temporal dementia in two variants = 9; supranuclear progressive palsy = 3; cortico-basal syndrome = 2), the temporal parameters of speech rate, vowel duration, consonant duration, and nine rhythmic metrics (%V, %C, ΔV, ΔC, VarcoV, VarcoC, rPVI-V, rPVI-C, nPVI-V). The results indicate that speech rate is slower in patients with primary progressive non-fluent aphasia (APPnf), amyotrophic lateral sclerosis (ALS), or corticobasal syndrome (CBS) than in controls. These three clinical groups, as well as those suffering from progressive supranuclear palsy (PSP), have longer segmental durations, especially in consonants. Among the rhythm metrics, %C, ΔV and rPVI-C also allow to differentiate between groups, specifically, between control and APPlog, ELA-DFT y SCB.

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Primary progressive aphasia patients evaluated using diffusion tensor imaging and voxel based volumetry-preliminary results

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2011000400007 ◽

2011 ◽

Vol 69 (3) ◽

pp. 446-451 ◽

Cited By ~ 6

Author(s):

Fábio Pascotto de Oliveira ◽

Jaderson Costa da Costa ◽

Sabine Possa Marroni ◽

Ana Maria Marques da Silva ◽

Sthefanie Hansen Barreiro ◽

...

Keyword(s):

Diffusion Tensor Imaging ◽

Cognitive Deficits ◽

Diffusion Tensor ◽

Primary Progressive Aphasia ◽

Control Group ◽

Progressive Aphasia ◽

Primary Progressive ◽

Language Deficit ◽

Volume Method ◽

Diffusion Tensor Imaging Dti

There are individuals who have a progressive language deficit without presenting cognitive deficits in other areas. One of the diseases related to this presentation is primary progressive aphasia (PPA). OBJECTIVE: Identify by means of diffusion tensor imaging (DTI) and measurements of cortical volume, brain areas that lead to dysphasia when presenting signs of impaired connectivity or reduced volume. METHOD: Four patients with PPA were evaluated using DTI, and measurements of cortical volumes in temporal areas. These patients were compared with two normal volunteers. RESULTS: There is a trend to a difference in the number and volume of related fibers between control group and patients with PPA. Comparing cortical volumes in temporal areas between groups yielded a trend to a smaller volume in PPA patients. CONCLUSION: Patients with PPA have a trend to impairment in cortical and subcortical levels regarding relevant areas.

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Slowing of Event-Related Potentials in Primary Progressive Aphasia. A case report

The Scientific World JOURNAL ◽

10.1100/tsw.2009.67 ◽

2009 ◽

Vol 9 ◽

pp. 633-638 ◽

Cited By ~ 2

Author(s):

Salvatore Giaquinto ◽

Francesca Ranghi

Keyword(s):

Word Recognition ◽

Cognitive Abilities ◽

Single Case ◽

Electrical Potential ◽

Primary Progressive Aphasia ◽

Event Related Potentials ◽

Control Group ◽

Progressive Aphasia ◽

Primary Progressive ◽

Related Potentials

Primary Progressive Aphasia (PPA) is a rare and insidious language impairment that worsens over time. It belongs to the group of fronto–temporal dementias. This study was aimed at assessing the role of speed of cognitive abilities, such as word recognition, in PPA. The design is a single-case, longitudinal study. A male patient suffering from PPA was enrolled and 15 healthy older adults were the control group. An event-related electrical potential connected with word recognition, namely the N400, was delayed by 200 msec at baseline compared to healthy controls and progressively deteriorated. One year later, the delay was greater and two years later the potential had disappeared. Reduced speed of processing is an early pathological factor that negatively affecting higher cognitive functions in APP. Event–related electrical potentials are recommended in the field of aphasia and cognitive decline. They permit observation of a speed decline in higher cognitive abilities, when pathological changes at a central level begin and language comprehension seems to be unaffected.

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Cognitive and Affective Empathy Disruption in Non-Fluent Primary Progressive Aphasia Syndromes

Brain Impairment ◽

10.1017/brimp.2016.21 ◽

2016 ◽

Vol 18 (1) ◽

pp. 117-129 ◽

Cited By ~ 9

Author(s):

Jessica L. Hazelton ◽

Muireann Irish ◽

John R. Hodges ◽

Olivier Piguet ◽

Fiona Kumfor

Keyword(s):

Language Impairment ◽

Disease Onset ◽

Primary Progressive Aphasia ◽

Cognitive Empathy ◽

Reactivity Index ◽

Affective Empathy ◽

Progressive Aphasia ◽

Primary Progressive ◽

Patient Groups ◽

Social Cognitive Deficits

Empathy involves being able to understand and respond to others’ emotional experiences. Whilst deficits in empathy have been observed in frontotemporal dementia, the extent to which empathy is disrupted in dementia syndromes with predominant language impairment remains unclear. The current study investigated cognitive and affective empathy in the two non-fluent primary progressive aphasia syndromes: progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA). Informants of 23 PNFA and 16 LPA patients completed the Interpersonal Reactivity Index (IRI), regarding patients’ capacity for empathy pre- and post-disease onset. Twenty-four healthy control participants completed the self-rated IRI for comparison of post-disease empathy capabilities. Within-group analyses revealed reduced cognitive empathy and increased personal distress in both patient groups. In addition, lowered affective empathy was reported in PNFA, with a similar trend observed in LPA. Interestingly, reduced affective empathy was associated with greater carer burden in LPA. Between-group analyses revealed reduced cognitive empathy in both patient groups relative to controls. The current study is the first to document empathy changes in PNFA and LPA, offering insight into the social cognitive deficits experienced in these syndromes. Future neuroimaging studies are needed to identify the underlying neural correlates and mechanisms driving empathy deficits in PNFA and LPA.

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The Nonverbal Processing of Actions Is an Area of Relative Strength in the Semantic Variant of Primary Progressive Aphasia

Journal of Speech Language and Hearing Research ◽

10.1044/2019_jslhr-19-00271 ◽

2020 ◽

Vol 63 (2) ◽

pp. 569-584 ◽

Cited By ~ 1

Author(s):

Noémie Auclair-Ouellet ◽

Marion Fossard ◽

Joël Macoir ◽

Robert Laforce

Keyword(s):

Primary Progressive Aphasia ◽

Relative Strength ◽

Control Group ◽

Progressive Aphasia ◽

Assessment Task ◽

Primary Progressive ◽

Action Naming ◽

Significant Difference ◽

Semantic Variant ◽

Better Than

Purpose Better performance for actions compared to objects has been reported in the semantic variant of primary progressive aphasia (svPPA). This study investigated the influence of the assessment task (naming, semantic picture matching) over the dissociation between objects and actions. Method Ten individuals with svPPA and 17 matched controls completed object and action naming tests, and object and action semantic picture matching tests. Performance was compared between the svPPA and control groups, within the svPPA group, and for each participant with svPPA versus the control group individually. Results Compared to controls, participants with svPPA were impaired on object and action naming, and object and action semantic picture matching. As a group, participants with svPPA had an advantage for actions over objects and for semantic picture matching tests over naming tests. Eight participants had a better performance for actions compared to objects in naming, with three showing a significant difference. Nine participants had a better performance for actions compared to objects in semantic picture matching, with six showing a significant difference. For objects, semantic picture matching was better than naming in nine participants, with five showing a significant difference. For actions, semantic picture matching was better than naming in all 10 participants, with nine showing a significant difference. Conclusion The nonverbal processing of actions, as assessed with a semantic picture matching test, is an area of relative strength in svPPA. Clinical implications for assessment planning and interpretation and theoretical implications for current models of semantic cognition are discussed.

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Motor Speech Phenotypes of Frontotemporal Dementia, Primary Progressive Aphasia, and Progressive Apraxia of Speech

Journal of Speech Language and Hearing Research ◽

10.1044/2016_jslhr-s-16-0140 ◽

2017 ◽

Vol 60 (4) ◽

pp. 897-911 ◽

Cited By ~ 10

Author(s):

Matthew L. Poole ◽

Amy Brodtmann ◽

David Darby ◽

Adam P. Vogel

Keyword(s):

Frontotemporal Dementia ◽

Meta Analysis ◽

Primary Progressive Aphasia ◽

Control Group ◽

Apraxia Of Speech ◽

Speech Impairment ◽

Motor Speech ◽

Progressive Aphasia ◽

Primary Progressive ◽

Neuroimaging Data

Purpose Our purpose was to create a comprehensive review of speech impairment in frontotemporal dementia (FTD), primary progressive aphasia (PPA), and progressive apraxia of speech in order to identify the most effective measures for diagnosis and monitoring, and to elucidate associations between speech and neuroimaging. Method Speech and neuroimaging data described in studies of FTD and PPA were systematically reviewed. A meta-analysis was conducted for speech measures that were used consistently in multiple studies. Results The methods and nomenclature used to describe speech in these disorders varied between studies. Our meta-analysis identified 3 speech measures which differentiate variants or healthy control-group participants (e.g., nonfluent and logopenic variants of PPA from all other groups, behavioral-variant FTD from a control group). Deficits within the frontal-lobe speech networks are linked to motor speech profiles of the nonfluent variant of PPA and progressive apraxia of speech. Motor speech impairment is rarely reported in semantic and logopenic variants of PPA. Limited data are available on motor speech impairment in the behavioral variant of FTD. Conclusions Our review identified several measures of speech which may assist with diagnosis and classification, and consolidated the brain–behavior associations relating to speech in FTD, PPA, and progressive apraxia of speech.

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Multidimensional Apathy in Behavioral Variant Frontotemporal Dementia, Primary Progressive Aphasia, and Alzheimer Disease

Journal of Geriatric Psychiatry and Neurology ◽

10.1177/0891988720924716 ◽

2020 ◽

pp. 089198872092471

Author(s):

Ratko Radakovic ◽

Shuna Colville ◽

Denise Cranley ◽

John M. Starr ◽

Suvankar Pal ◽

...

Keyword(s):

Alzheimer Disease ◽

Frontotemporal Dementia ◽

Primary Progressive Aphasia ◽

Future Research ◽

Self Awareness ◽

Behavioral Variant Frontotemporal Dementia ◽

Progressive Aphasia ◽

Primary Progressive ◽

Patient Groups ◽

Rating Discrepancy

Apathy is prevalent in dementia, such as behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD). As a multidimensional construct, it can be assessed and subsumed under a Dimensional Apathy Framework. A consistent apathy profile in bvFTD and PPA has yet to be established. The aim was to explore apathy profiles and awareness in bvFTD, PPA, and AD. A total of 12 patients with bvFTD, 12 patients with PPA, 28 patients with AD, and 20 matched controls, as well as their informants/carers, were recruited. All participants completed the Dimensional Apathy Scale (DAS), assessing executive, emotional, and initiation apathy subtypes, a 1-dimensional apathy measure, depression measure, and functional and cognitive screens. Apathy subtype awareness was determined through DAS informant/carer and self-rating discrepancy. Apathy profile comparison showed patients with bvFTD had significantly higher emotional apathy than patients with AD ( P < .01) and significantly higher apathy over all subtypes than patients with PPA ( Ps < .05). Additionally, patients with bvFTD had significantly lower awareness for emotional apathy ( P < .01) when compared to patients with AD and PPA. All patient groups had significant global apathy over all subtypes compared to controls. The emergent apathy profile for bvFTD seems to be emotional apathy (indifference or emotional/affective neutrality), with lower self-awareness in this subtype. Further, lower self-awareness for executive apathy (lack of motivation for planning, organization, or attention) differentiates bvFTD from PPA. Future research should investigate the cognitive and neural correlates as well as the practical impact of apathy subtypes.

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Describing Phonological Paraphasias in Three Variants of Primary Progressive Aphasia

American Journal of Speech-Language Pathology ◽

10.1044/2017_ajslp-16-0210 ◽

2018 ◽

Vol 27 (1S) ◽

pp. 336-349 ◽

Cited By ~ 9

Author(s):

Sarah Grace Hudspeth Dalton ◽

Christine Shultz ◽

Maya L. Henry ◽

Argye E. Hillis ◽

Jessica D. Richardson

Keyword(s):

Target Word ◽

Phonological Processing ◽

Speech Rate ◽

Primary Progressive Aphasia ◽

Future Research ◽

Word Target ◽

Progressive Aphasia ◽

Primary Progressive ◽

Sequencing Errors ◽

Part Of Speech

Purpose The purpose of this study was to describe the linguistic environment of phonological paraphasias in 3 variants of primary progressive aphasia (semantic, logopenic, and nonfluent) and to describe the profiles of paraphasia production for each of these variants. Method Discourse samples of 26 individuals diagnosed with primary progressive aphasia were investigated for phonological paraphasias using the criteria established for the Philadelphia Naming Test (Moss Rehabilitation Research Institute, 2013). Phonological paraphasias were coded for paraphasia type, part of speech of the target word, target word frequency, type of segment in error, word position of consonant errors, type of error, and degree of change in consonant errors. Results Eighteen individuals across the 3 variants produced phonological paraphasias. Most paraphasias were nonword, followed by formal, and then mixed, with errors primarily occurring on nouns and verbs, with relatively few on function words. Most errors were substitutions, followed by addition and deletion errors, and few sequencing errors. Errors were evenly distributed across vowels, consonant singletons, and clusters, with more errors occurring in initial and medial positions of words than in the final position of words. Most consonant errors consisted of only a single-feature change, with few 2- or 3-feature changes. Importantly, paraphasia productions by variant differed from these aggregate results, with unique production patterns for each variant. Conclusions These results suggest that a system where paraphasias are coded as present versus absent may be insufficient to adequately distinguish between the 3 subtypes of PPA. The 3 variants demonstrate patterns that may be used to improve phenotyping and diagnostic sensitivity. These results should be integrated with recent findings on phonological processing and speech rate. Future research should attempt to replicate these results in a larger sample of participants with longer speech samples and varied elicitation tasks. Supplemental Materials https://doi.org/10.23641/asha.5558107

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The left inferior frontal gyrus is causally involved in selective semantic retrieval: Evidence from tDCS in primary progressive aphasia

10.1101/2020.07.20.20151043 ◽

2020 ◽

Author(s):

Zeyi Wang ◽

Bronte N Ficek ◽

Kimberly T Webster ◽

Chiadi U Onyike ◽

John E Desmond ◽

...

Keyword(s):

Functional Connectivity ◽

Inferior Frontal Gyrus ◽

Primary Progressive Aphasia ◽

Superior Temporal Gyrus ◽

Semantic Fluency ◽

Semantic Retrieval ◽

Left Inferior Frontal Gyrus ◽

Progressive Aphasia ◽

Temporal Lobes ◽

Primary Progressive

Lesion and imaging studies have shown that the left inferior frontal gyrus (IFG) is involved in selective semantic retrieval of information from the temporal lobes. However, causal, i.e., interventional, evidence is sparse. In the present study we addressed this question by testing whether transcranial direct current stimulation (tDCS) over the left IFG in a group of individuals with primary progressive aphasia may improve semantic fluency, a task that relies to selective semantic retrieval. Semantic fluency improved significantly more in the tDCS vs. sham condition immediately post-treatment and improvement lasted up to 2 months. We further addressed the question of who will benefit most from such an intervention by testing possible demographic, clinical and functional connectivity variables that may predict the behavioral tDCS effect. We found that patients with stronger baseline functional connectivity between the subareas of the left IFG opercularis and triangularis, and between the middle temporal pole and superior temporal gyrus. were the most likely to benefit from tDCS over the left IFG. We thus provided causal evidence that the left IFG is the neural substrate of selective semantic retrieval and tDCS over the left IFG may improve semantic fluency in individuals with stronger baseline functional connectivity.

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The Electrophysiological Correlates of Phoneme Perception in Primary Progressive Aphasia: A Preliminary Case Series

Frontiers in Human Neuroscience ◽

10.3389/fnhum.2021.618549 ◽

2021 ◽

Vol 15 ◽

Author(s):

Jara Stalpaert ◽

Marijke Miatton ◽

Anne Sieben ◽

Tim Van Langenhove ◽

Pieter van Mierlo ◽

...

Keyword(s):

Primary Progressive Aphasia ◽

Mean Amplitude ◽

Diagnostic Process ◽

Control Group ◽

Onset Latency ◽

Progressive Aphasia ◽

Primary Progressive ◽

Phoneme Discrimination ◽

Clinical Variants ◽

Phoneme Perception

Aims: This study aimed to investigate phoneme perception in patients with primary progressive aphasia (PPA) by using the event-related potential (ERP) technique. These ERP components might contribute to the diagnostic process of PPA and its clinical variants (NFV: nonfluent variant, SV: semantic variant, LV: logopenic variant) and reveal insights about phoneme perception processes in these patients.Method: Phoneme discrimination and categorization processes were investigated by the mismatch negativity (MMN) and P300 in eight persons with early- and late-stage PPA (3 NFV, 2 LV, 2 SV, and 1 PPA-NOS; not otherwise specified) and 30 age-matched healthy adults. The mean amplitude, the onset latency, and the topographic distribution of both components in each patient were compared to the results of the control group.Results: The MMN was absent or the onset latency of the MMN was delayed in the patients with the NFV, LV, and PPA-NOS in comparison to the control group. In contrast, no differences in mean amplitudes and onset latencies of the MMN were found between the patients with the SV and the control group. Concerning the P300, variable results were found in the patients with the NFV, SV, and PPA-NOS, but the P300 of both patients with the LV was delayed and prolonged with increased mean amplitude in comparison to the control group.Conclusion: In this preliminary study, phoneme discrimination deficits were found in the patients with the NFV and LV, and variable deficits in phoneme categorization processes were found in all patients with PPA. In clinical practice, the MMN might be valuable to differentiate the SV from the NFV and the LV and the P300 to differentiate the LV from the NFV and the SV. Further research in larger and independent patient groups is required to investigate the applicability of these components in the diagnostic process and to determine the nature of these speech perception deficits in the clinical variants of PPA.

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Neuroimaging in Frontotemporal Dementia: Heterogeneity and Relationships with Underlying Neuropathology

Neurotherapeutics ◽

10.1007/s13311-021-01101-x ◽

2021 ◽

Author(s):

Bradley T. Peet ◽

Salvatore Spina ◽

Nidhi Mundada ◽

Renaud La Joie

Keyword(s):

Frontotemporal Dementia ◽

Primary Progressive Aphasia ◽

Resonance Imaging ◽

Structural Magnetic Resonance Imaging ◽

Progressive Aphasia ◽

Temporal Lobes ◽

Primary Progressive ◽

Positron Emission ◽

Clinical Syndromes ◽

Pathologic Features

AbstractFrontotemporal dementia encompasses a group of clinical syndromes defined pathologically by degeneration of the frontal and temporal lobes. Historically, these syndromes have been challenging to diagnose, with an average of about three years between the time of symptom onset and the initial evaluation and diagnosis. Research in the field of neuroimaging has revealed numerous biomarkers of the various frontotemporal dementia syndromes, which has provided clinicians with a method of narrowing the differential diagnosis and improving diagnostic accuracy. As such, neuroimaging is considered a core investigative tool in the evaluation of neurodegenerative disorders. Furthermore, patterns of neurodegeneration correlate with the underlying neuropathological substrates of the frontotemporal dementia syndromes, which can aid clinicians in determining the underlying etiology and improve prognostication. This review explores the advancements in neuroimaging and discusses the phenotypic and pathologic features of behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia, as seen on structural magnetic resonance imaging and positron emission tomography.

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