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Journal articles on the topic 'Semantic variant primary progressive aphasia'

Author: Grafiati

Published: 4 June 2021

Last updated: 8 February 2022

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1

Henry, Maya L., Stephen M. Wilson, Miranda C. Babiak, Maria Luisa Mandelli, Pelagie M. Beeson, Zachary A. Miller, and Maria Luisa Gorno-Tempini. "Phonological Processing in Primary Progressive Aphasia." Journal of Cognitive Neuroscience 28, no. 2 (February 2016): 210–22. http://dx.doi.org/10.1162/jocn_a_00901.

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Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory–phonological) and ventral (lexical–semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective damage to dorsal pathway structures, is considered to be a distinctive feature of the logopenic variant of PPA. By contrast, phonological abilities are considered to be relatively spared in the semantic variant and are largely unexplored in the nonfluent/agrammatic variant. Comprehensive assessment of phonological ability in the three variants of PPA has not been undertaken. We investigated phonological processing skills in a group of participants with PPA as well as healthy controls, with the goal of identifying whether patterns of performance support the dorsal versus ventral functional–anatomical framework and to discern whether phonological ability differs among PPA subtypes. We also explored the neural bases of phonological performance using voxel-based morphometry. Phonological performance was impaired in patients with damage to dorsal pathway structures (nonfluent/agrammatic and logopenic variants), with logopenic participants demonstrating particular difficulty on tasks involving nonwords. Binary logistic regression revealed that select phonological tasks predicted diagnostic group membership in the less fluent variants of PPA with a high degree of accuracy, particularly in conjunction with a motor speech measure. Brain–behavior correlations indicated a significant association between the integrity of gray matter in frontal and temporoparietal regions of the left hemisphere and phonological skill. Findings confirm the critical role of dorsal stream structures in phonological processing and demonstrate unique patterns of impaired phonological processing in logopenic and nonfluent/agrammatic variants of PPA.

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2

Ogar, Jennifer M. "Primary Progressive Aphasia and Its Three Variants." Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders 20, no. 1 (April 2010): 5–12. http://dx.doi.org/10.1044/nnsld20.1.5.

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Abstract Speech-language pathologists are increasingly treating patients with progressive disorders, including primary progressive aphasia (PPA). For many years, two variants of PPA were recognized: a nonfluent type—progressive nonfluent aphasia (PNFA)—and a fluent form—semantic dementia (SD). In 2004, a third variant—logopenic progressive aphasia (LPA)—was described. This article will review clinical symptoms, neuroimaging correlates, and the neuropathologies that are typically associated with PNFA, SD, and LPA. Case studies are included to further illustrate the characteristics of each of these three PPA variants.

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Caffarra, Paolo, Simona Gardini, Stefano Cappa, Francesca Dieci, Letizia Concari, Federica Barocco, Caterina Ghetti, Livia Ruffini, and Guido Dalla Rosa Prati. "Degenerative Jargon Aphasia: Unusual Progression of Logopenic/Phonological Progressive Aphasia?" Behavioural Neurology 26, no. 1-2 (2013): 89–93. http://dx.doi.org/10.1155/2013/965782.

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Primary progressive aphasia (PPA) corresponds to the gradual degeneration of language which can occur as nonfluent/agrammatic PPA, semantic variant PPA or logopenic variant PPA. We describe the clinical evolution of a patient with PPA presenting jargon aphasia as a late feature. At the onset of the disease (ten years ago) the patient showed anomia and executive deficits, followed later on by phonemic paraphasias and neologisms, deficits in verbal short-term memory, naming, verbal and semantic fluency. At recent follow-up the patient developed an unintelligible jargon with both semantic and neologistic errors, as well as with severe deficit of comprehension which precluded any further neuropsychological assessment. Compared to healthy controls, FDG-PET showed a hypometabolism in the left angular and middle temporal gyri, precuneus, caudate, posterior cingulate, middle frontal gyrus, and bilaterally in the superior temporal and inferior frontal gyri. The clinical and neuroimaging profile seems to support the hypothesis that the patient developed a late feature of logopenic variant PPA characterized by jargonaphasia and associated with superior temporal and parietal dysfunction.

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Wu, Teresa Q., Zachary A. Miller, Babu Adhimoolam, Diana D. Zackey, Baber K. Khan, Robin Ketelle, Katherine P. Rankin, and Bruce L. Miller. "Verbal creativity in semantic variant primary progressive aphasia." Neurocase 21, no. 1 (December 12, 2013): 73–78. http://dx.doi.org/10.1080/13554794.2013.860179.

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5

Tippett, Donna C. "Classification of primary progressive aphasia: challenges and complexities." F1000Research 9 (January 30, 2020): 64. http://dx.doi.org/10.12688/f1000research.21184.1.

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Primary progressive aphasia (PPA) is classified into three variants, logopenic variant PPA (lvPPA), nonfluent agrammatic PPA (nfaPPA), and semantic variant PPA (svPPA), based on clinical (syndromic) characteristics with support from neuroimaging and/or underlying neuropathology. Classification of PPA variants provides information valuable to disease management. International consensus criteria are widely employed to identify PPA subtypes; however, classification is complex, and some individuals do not fit neatly into the subtyping scheme. In this review, diagnostic challenges and their implications are discussed, possible explanations for these challenges are explored, and approaches to address PPA classification are considered.

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6

Pascual, Belen, Quentin Funk, Paolo Zanotti-Fregonara, Matthew D. Cykowski, Mattia Veronese, Elijah Rockers, Kathleen Bradbury, et al. "Neuroinflammation is highest in areas of disease progression in semantic dementia." Brain 144, no. 5 (April 6, 2021): 1565–75. http://dx.doi.org/10.1093/brain/awab057.

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Abstract Despite epidemiological and genetic data linking semantic dementia to inflammation, the topography of neuroinflammation in semantic dementia, also known as the semantic variant of primary progressive aphasia, remains unclear. The pathology starts at the tip of the left temporal lobe where, in addition to cortical atrophy, a strong signal appears with the tau PET tracer 18F-flortaucipir, even though the disease is not typically associated with tau but with TDP-43 protein aggregates. Here, we characterized the topography of inflammation in semantic variant primary progressive aphasia using high-resolution PET and the tracer 11C-PBR28 as a marker of microglial activation. We also tested the hypothesis that inflammation, by providing non-specific binding targets, could explain the 18F-flortaucipir signal in semantic variant primary progressive aphasia. Eight amyloid-PET-negative patients with semantic variant primary progressive aphasia underwent 11C-PBR28 and 18F-flortaucipir PET. Healthy controls underwent 11C-PBR28 PET (n = 12) or 18F-flortaucipir PET (n = 12). Inflammation in PET with 11C-PBR28 was analysed using Logan graphical analysis with a metabolite-corrected arterial input function. 18F-flortaucipir standardized uptake value ratios were calculated using the cerebellum as the reference region. Since monoamine oxidase B receptors are expressed by astrocytes in affected tissue, selegiline was administered to one patient with semantic variant primary progressive aphasia before repeating 18F-flortaucipir scanning to test whether monoamine oxidase B inhibition blocked flortaucipir binding, which it did not. While 11C-PBR28 uptake was mostly cortical, 18F-flortaucipir uptake was greatest in the white matter. The uptake of both tracers was increased in the left temporal lobe and in the right temporal pole, as well as in regions adjoining the left temporal pole such as insula and orbitofrontal cortex. However, peak uptake of 18F-flortaucipir localized to the left temporal pole, the epicentre of pathology, while the peak of inflammation 11C-PBR28 uptake localized to a more posterior, mid-temporal region and left insula and orbitofrontal cortex, in the periphery of the damage core. Neuroinflammation, greatest in the areas of progression of the pathological process in semantic variant primary progressive aphasia, should be further studied as a possible therapeutic target to slow disease progression.

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7

Bettcher, Brianne M., and Virginia E. Sturm. "Neuropsychological Assessment of Primary Progressive Aphasia (PPA)." Perspectives on Neurophysiology and Neurogenic Speech and Language Disorders 24, no. 4 (October 2014): 128–36. http://dx.doi.org/10.1044/nnsld24.4.128.

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The goal of this article is to outline the utility of both language and non-language testing in making a diagnosis of logopenic, nonfluent/agrammatic, and semantic variant primary progressive aphasias PPA as well as delineate important behavioral and speech features that can be detected via clinical observation. We review speech/language presentations, non-language cognitive domains, and behavioral manifestations associated with each disorder. Patients with logopenic variant PPA evidence non-language cognitive impairments that include acalculia, phonological working memory deficits, and mild/variable difficulties with memory and visuospatial functions. In contrast, patients with nonfluent/agrammatic variant PPA display non-language impairments in executive functions, and show relative preservation of memory and visuospatial functions. Finally, semantic variant patients display behavioral changes in social comportment as well as non-language difficulties with category fluency and arithmetic facts; they display relative preservation, if not enhancement, of visuospatial functions. In summary, broad neural networks that support both language and non-language functions are affected in PPA syndromes, thus a comprehensive assessment of additional neuropsychological domains may aid in solidifying and subtyping PPA diagnoses.

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8

Awad, Stephanie M., and Amer M. Awad. "A Middle-Aged Woman with Logopenic Progressive Aphasia as a Precursor of Alzheimer's Disease: Case Report and Review of the Literature." Case Reports in Neurological Medicine 2011 (2011): 1–3. http://dx.doi.org/10.1155/2011/450301.

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Primary progressive aphasia is a neurodegenerative disorder that was recently classified into three types: fluent (semantic), nonfluent, and logopenic. The logopenic variant is the least common one and is closely related to Alzheimer's disease in comparison to the other two variants that are closely related to frontotemporal dementia. We report the case of a middle-aged woman who presented to our center with progressive aphasia that was undiagnosed for two years. The patient's neurological evaluation including positron emission tomography is consistent with a logopenic variant of primary progressive aphasia.

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9

Watson, Christa L., Katherine Possin, I. Elaine Allen, H. Isabel Hubbard, Marita Meyer, Ariane E. Welch, Gil D. Rabinovici, et al. "Visuospatial Functioning in the Primary Progressive Aphasias." Journal of the International Neuropsychological Society 24, no. 3 (October 17, 2017): 259–68. http://dx.doi.org/10.1017/s1355617717000984.

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AbstractObjectives: The aim of this study was to identify whether the three main primary progressive aphasia (PPA) variants would show differential profiles on measures of visuospatial cognition. We hypothesized that the logopenic variant would have the most difficulty across tasks requiring visuospatial and visual memory abilities. Methods: PPA patients (n=156), diagnosed using current criteria, and controls were tested on a battery of tests tapping different aspects of visuospatial cognition. We compared the groups on an overall visuospatial factor; construction, immediate recall, delayed recall, and executive functioning composites; and on individual tests. Cross-sectional and longitudinal comparisons were made, adjusted for disease severity, age, and education. Results: The logopenic variant had significantly lower scores on the visuospatial factor and the most impaired scores on all composites. The nonfluent variant had significant difficulty on all visuospatial composites except the delayed recall, which differentiated them from the logopenic variant. In contrast, the semantic variants performed poorly only on delayed recall of visual information. The logopenic and nonfluent variants showed decline in figure copying performance over time, whereas in the semantic variant, this skill was remarkably preserved. Conclusions: This extensive examination of performance on visuospatial tasks in the PPA variants solidifies some previous findings, for example, delayed recall of visual stimuli adds value in differential diagnosis between logopenic variant PPA and nonfluent variant PPA variants, and illuminates the possibility of common mechanisms that underlie both linguistic and non-linguistic deficits in the variants. Furthermore, this is the first study that has investigated visuospatial functioning over time in the PPA variants. (JINS, 2018, 24, 259–268)

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10

Jerkić, Lana, Dragan Pavlović, Mile Vuković, Jelena Todorović, and Mirna Zelić. "Profile of linguistic and cognitive deficits in persons with a semantic variant of a primary progressive aphasia." Medicinski casopis 54, no. 3 (2020): 113–19. http://dx.doi.org/10.5937/mckg54-27796.

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Primary progressive aphasia (PPA) includes a group of neurodegenerative disorders that are characterized by progressive deterioration of language functions, while other cognitive functions, at least at the onset of the disease, are relatively spared. There are three basic subtypes of PPA: the nonfluent progressive aphasia (nvPPA), the semantic variant of a primary progressive aphasia (svPPA), and the logopenic progressive aphasia (lvPPA). The semantic variant of a PPA can also be found in the literature under the term of semantic dementia. It is clinically manifested by progressive deterioration of semantic knowledge, fluent aphasia, impaired naming and comprehension, prosopagnosia and surface dyslexia and dysgraphia (in languages with irregular orthography). As the disease progresses, other cognitive changes can be observed. The main cause of the disorder is progressive bilateral atrophy of the anterior temporal lobes, which is more manifested in the left hemisphere. The literature is modest in terms of the use of specific treatment methods in the rehabilitation of these patients. Since speech and language disorders are the most conspicuous symptom, at least at the beginning of the disease, the role of speech therapists in the assessment and restitution of speechlanguage and communication skills is also indisputable.

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11

Lee, Jin San, Hak Young Rhee, and Key-Chung Park. "Amyloid-positive late-onset semantic variant primary progressive aphasia." Neurological Sciences 39, no. 10 (July 10, 2018): 1811–12. http://dx.doi.org/10.1007/s10072-018-3488-x.

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12

Barker, Megan S., Hannah E. Silverman, Rachel Fremont, Masood Manoochehri, Stephanie Cosentino, and Edward D. Huey. "“Everything hurts!” Distress in semantic variant primary progressive aphasia." Cortex 127 (June 2020): 396–98. http://dx.doi.org/10.1016/j.cortex.2020.03.002.

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13

Joo, Jae Young, Hyug-Gi Kim, Kyung Mi Lee, Seok Hoon Ko, Hak Young Rhee, Key-Chung Park, and Jin San Lee. "Parosmia in Right-lateralized Semantic Variant Primary Progressive Aphasia." Alzheimer Disease & Associated Disorders 35, no. 2 (January 11, 2021): 160–63. http://dx.doi.org/10.1097/wad.0000000000000429.

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14

Macoir, Joël, Monica Lavoie, Robert Laforce, Simona M. Brambati, and Maximiliano A. Wilson. "Dysexecutive Symptoms in Primary Progressive Aphasia: Beyond Diagnostic Criteria." Journal of Geriatric Psychiatry and Neurology 30, no. 3 (March 30, 2017): 151–61. http://dx.doi.org/10.1177/0891988717700507.

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Primary progressive aphasia (PPA) is a heterogeneous neurodegenerative condition in which the most prominent clinical feature is language difficulties. Other cognitive domains have been described to remain unaffected at the early stages of the disease and, therefore, excluded from diagnostic criteria. However, we show in this article that executive function (EF) disorders may be present in the 3 variants (nonfluent/agrammatic, logopenic, and semantic) of PPA. We also illustrate changes in language and EF by means of a 3-year behavioral and neuroimaging longitudinal study of a patient suffering from the semantic variant of PPA. This review provides an update on current knowledge of PPA, suggesting that dysexecutive symptoms may be encountered in the 3 PPA variants, in their early phases and/or in more advanced stages, when atrophy extends to adjacent brain areas.

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Montagut, Núria, Sergi Borrego-Écija, Magdalena Castellví, Immaculada Rico, Ramón Reñé, Mircea Balasa, Albert Lladó, and Raquel Sánchez-Valle. "Errorless Learning Therapy in Semantic Variant of Primary Progressive Aphasia." Journal of Alzheimer's Disease 79, no. 1 (January 5, 2021): 415–22. http://dx.doi.org/10.3233/jad-200904.

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Background: The semantic variant of primary progressive aphasia (svPPA) is characterized by a progressive loss of semantic knowledge impairing the ability to name and to recognize the meaning of words. Objective: We aimed to evaluate the immediate and short-term effect of errorless learning speech therapy on the naming and recognition of commonly used words in patients with svPPA. Methods: Eight participants diagnosed with svPPA received 16 sessions of intensive errorless learning speech therapy. Naming and word comprehension tasks were evaluated at baseline, immediately postintervention, and at follow-up after 1, 3, and 6 months. These evaluations were performed using two item sets (a trained list and an untrained list). Results: In the naming tasks, patients showed a significant improvement in trained items immediately after the intervention, but that improvement decayed progressively when therapy ended. No improvements were found either in trained comprehension or in untrained tasks. Conclusion: Errorless learning therapy could improve naming ability in patients with svPPA. This effect may be due to the relative preservation of episodic memory, but the benefit is not maintained over time, presumably because there is no consolidation.

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Saracino, Dario, Sophie Ferrieux, Marie Noguès-Lassiaille, Marion Houot, Aurélie Funkiewiez, Leila Sellami, Vincent Deramecourt, et al. "Primary Progressive Aphasia Associated With GRN Mutations." Neurology 97, no. 1 (May 12, 2021): e88-e102. http://dx.doi.org/10.1212/wnl.0000000000012174.

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ObjectiveTo determine relative frequencies and linguistic profiles of primary progressive aphasia (PPA) variants associated with GRN (progranulin) mutations and to study their neuroanatomic correlates.MethodsPatients with PPA carrying GRN mutations (PPA-GRN) were selected among a national prospective research cohort of 1,696 patients with frontotemporal dementia, including 235 patients with PPA. All patients with amyloid-positive CSF biomarkers were excluded. In this cross-sectional study, speech/language and cognitive profiles were characterized with standardized evaluations, and gray matter (GM) atrophy patterns using voxel-based morphometry. Comparisons were performed with controls and patients with sporadic PPA.ResultsAmong the 235 patients with PPA, 45 (19%) carried GRN mutations, and we studied 32 of these. We showed that logopenic PPA (lvPPA) was the most frequent linguistic variant (n = 13, 41%), followed by nonfluent/agrammatic (nfvPPA; n = 9, 28%) and mixed forms (n = 8, 25%). Semantic variant was rather rare (n = 2, 6%). Patients with lvPPA, qualified as nonamyloid lvPPA, presented canonical logopenic deficit. Seven of 13 had a pure form; 6 showed subtle additional linguistic deficits not fitting criteria for mixed PPA and hence were labeled as logopenic-spectrum variant. GM atrophy involved primarily left posterior temporal gyrus, mirroring neuroanatomic changes of amyloid-positive-lvPPA. Patients with nfvPPA presented agrammatism (89%) rather than apraxia of speech (11%).ConclusionsThis study shows that the most frequent PPA variant associated with GRN mutations is nonamyloid lvPPA, preceding nfvPPA and mixed forms, and illustrates that the language network may be affected at different levels. GRN testing is indicated for patients with PPA, whether familial or sporadic. This finding is important for upcoming GRN gene–specific therapies.

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Cousins, Katheryn A. Q., Sharon Ash, Christopher A. Olm, and Murray Grossman. "Longitudinal Changes in Semantic Concreteness in Semantic Variant Primary Progressive Aphasia (svPPA)." eneuro 5, no. 6 (November 2018): ENEURO.0197–18.2018. http://dx.doi.org/10.1523/eneuro.0197-18.2018.

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18

Agosta, Federica, Sebastiano Galantucci, Paola Valsasina, Elisa Canu, Alessandro Meani, Alessandra Marcone, Giuseppe Magnani, Andrea Falini, Giancarlo Comi, and Massimo Filippi. "Disrupted brain connectome in semantic variant of primary progressive aphasia." Neurobiology of Aging 35, no. 11 (November 2014): 2646–55. http://dx.doi.org/10.1016/j.neurobiolaging.2014.05.017.

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19

Makaretz, Sara J., Megan Quimby, Jessica Collins, Nikos Makris, Scott McGinnis, Aaron Schultz, Neil Vasdev, Keith A. Johnson, and Bradford C. Dickerson. "Flortaucipir tau PET imaging in semantic variant primary progressive aphasia." Journal of Neurology, Neurosurgery & Psychiatry 89, no. 10 (October 6, 2017): 1024–31. http://dx.doi.org/10.1136/jnnp-2017-316409.

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ObjectiveThe semantic variant of primary progressive aphasia (svPPA) is typically associated with frontotemporal lobar degeneration (FTLD) with longTAR DNA-binding protein (TDP)-43-positive neuropil threads and dystrophic neurites (type C), and is only rarely due to a primary tauopathy or Alzheimer’s disease. We undertook this study to investigate the localisation and magnitude of the presumed tau Positron Emission Tomography (PET) tracer [18F]Flortaucipir (FTP; also known as T807 or AV1451) in patients with svPPA, hypothesising that most patients would not show tracer uptake different from controls.MethodsFTP and [11C]Pittsburgh compound B PET imaging as well as MRI were performed in seven patients with svPPA and in 20 controls. FTP signal was analysed by visual inspection and by quantitative comparison to controls, with and without partial volume correction.ResultsAll seven patients showed elevated FTP uptake in the anterior temporal lobe with a leftward asymmetry that was not observed in healthy controls. This elevated FTP signal, largely co-localised with atrophy, was evident on both visual inspection and quantitative cortical surface-based analysis. Five patients were amyloid negative, one was amyloid positive and one has an unknown amyloid status.ConclusionsIn this series of patients with clinical profiles, structural MRI and amyloid PET imaging typical for svPPA, FTP signal was unexpectedly elevated with a spatial pattern localised to areas of atrophy. This raises questions about the possible off-target binding of this tracer to non-tau molecules associated with neurodegeneration. Further investigation with autopsy analysis will help illuminate the binding target(s) of FTP in cases of suspected FTLD-TDP neuropathology.

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Ting, Simon Kang Seng, Stephanie Pei Shi Chia, and Shahul Hameed. "Deep Dyslexia in Chinese Primary Progressive Aphasia of Semantic Variant." Journal of Neuropsychiatry and Clinical Neurosciences 28, no. 2 (April 2016): e25-e26. http://dx.doi.org/10.1176/appi.neuropsych.15110385.

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Ji, Min-Jin, Sungwon Jung, Ha-Eun Seo, Sang-Young Kim, Woo-Ram Kim, Sora Kim, Jin Sook Lee, and Young Noh. "Heterozygous TREM2 Mutation in Semantic Variant of Primary Progressive Aphasia." Journal of Clinical Neurology 16, no. 2 (2020): 352. http://dx.doi.org/10.3988/jcn.2020.16.2.352.

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22

Graff-Radford, Jonathan, Keith A. Josephs, Joseph E. Parisi, Dennis W. Dickson, Caterina Giannini, and Bradley F. Boeve. "Globular Glial Tauopathy Presenting as Semantic Variant Primary Progressive Aphasia." JAMA Neurology 73, no. 1 (January 1, 2016): 123. http://dx.doi.org/10.1001/jamaneurol.2015.2711.

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23

Martínez-Cuitiño, Macarena, Federico Gonzalo Soriano, Jesica Formoso, Geraldine Borovinsky, Jesica Ferrari, Noelia Pontello, Facundo Manes, and Juan Pablo Barreyro. "P1-531: LEXICAL PROCESSING IN SEMANTIC VARIANT PRIMARY PROGRESSIVE APHASIA." Alzheimer's & Dementia 14, no. 7S_Part_9 (July 1, 2006): P535—P536. http://dx.doi.org/10.1016/j.jalz.2018.06.542.

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24

Volkmer, Anna, Emily Rogalski, Maya Henry, Cathleen Taylor-Rubin, Leanne Ruggero, Rebecca Khayum, Jackie Kindell, Maria Luisa Gorno-Tempini, Jason D. Warren, and Jonathan D. Rohrer. "Speech and language therapy approaches to managing primary progressive aphasia." Practical Neurology 20, no. 2 (July 29, 2019): 154–61. http://dx.doi.org/10.1136/practneurol-2018-001921.

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The term primary progressive aphasia (PPA) describes a group of neurodegenerative disorders with predominant speech and language dysfunction as their main feature. There are three main variants – the semantic variant, the nonfluent or agrammatic variant and the logopenic variant – each with specific linguistic deficits and different neuroanatomical involvement. There are currently no curative treatments or symptomatic pharmacological therapies. However, speech and language therapists have developed several impairment-based interventions and compensatory strategies for use in the clinic. Unfortunately, multiple barriers still need to be overcome to improve access to care for people with PPA, including increasing awareness among referring clinicians, improving training of speech and language therapists and developing evidence-based guidelines for therapeutic interventions. This review highlights this inequity and the reasons why neurologists should refer people with PPA to speech and language therapists.

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Macoir, Joel, Vicent Martel-Sauvageau, Liziane Bouvier, Robert Laforce, and Laura Monetta. "Heterogeneity of repetition abilities in logopenic variant primary progressive aphasia." Dementia & Neuropsychologia 15, no. 3 (September 2021): 405–12. http://dx.doi.org/10.1590/1980-57642021dn15-030014.

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ABSTRACT. The differential diagnosis of primary progressive aphasia (PPA) is challenging due to overlapping clinical manifestations of the different variants of the disease. This is particularly true for the logopenic variant of PPA (lvPPA), in which such overlap was reported with regard to impairments in repetition abilities. In this study, four individuals with lvPPA underwent standard neuropsychological and language assessments. The influence of psycholinguistic variables on their performance of in word, nonword and sentence repetition tasks was also specifically explored. Some level of heterogeneity was found in cognitive functions and in language. The four participants showed impairment in sentence repetition in which their performance was negatively affected by semantic reversibility and syntactic complexity. This study supports the heterogeneity of lvPPA with respect to the cognitive and linguistic status of participants. It also shows that sentence repetition is influenced not only by length, but also by semantic reversibility and syntactic complexity, two psycholinguistic variables known to place additional demands on phonological working memory.

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Antczak-Kujawin, Justyna. "NAMING DISORDERS IN LOGOPENIC VARIANT OF PRIMARY PROGRESSIVE APHASIA." Acta Neuropsychologica 17, no. 1 (February 12, 2019): 87–95. http://dx.doi.org/10.5604/01.3001.0013.1851.

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Language functions, particularly disordered lexical skills were diagnosed in the examined woman based on selected diagnostic tests of the Boston Diagnostic Aphasia Examination (BDAE). Furthermore, an experimental version of the author's original test for assessing lexical-semantic performance in dementia was used. The author presents a case study of a 79-year-old woman diagnosed with logopenic variant primary progressive aphasia (lvPPA) secondary to Alzheimer’s disease. The author describes the symptoms of anomie manifested by the study participant and the supplementary strategies she applied in the case of lexical deficits. The analysis of the findings obtained in the course of language function assessment allowed the author to assess the fluency of speech, speech comprehension, repetition and naming. The study participant diagnosed with lvPPA was observed to manifest the following: an absence of motor speech disorders, absence of characteristics of evident agrammatism, preserved comprehension of individual words, preserved semantic knowledge of objects, disordered retrieval of words in spontaneous speech and in attempts to name, and disordered repetition of sentences and phrases. The analysis of the discussed case study allowed the author to discuss the progressive lexical deficits manifested by the lvPPA patient and to record those supplementary strategies that were most frequently applied in the lexical difficulties experienced by the female patient diagnosed with lvPPA.

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Paolini, Susy, Lucia Paciaroni, Antonio Manca, Roberto Rossi, Daniela Fornarelli, Stefano F. Cappa, Angela M. Abbatecola, and Osvaldo Scarpino. "Change of Accent as an Atypical Onset of non Fluent Primary Progressive Aphasia." Behavioural Neurology 27, no. 2 (2013): 221–27. http://dx.doi.org/10.1155/2013/278976.

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Language disorders can be the first symptom of many neurodegenerative diseases, including Alzheimer's disease (AD) and primary progressive aphasia (PPA). The main variants of PPA are: the non-fluent/agrammatic variant, the semantic variant and the logopenic variant.Several additional variants of PPA, however, have been described and are considered as atypical presentations.We describe the case of a woman presenting a progressive isolated language disturbance, characterized by an early dysprosodia, phonological and semantic paraphasias, agrammatism, impairment in repetition, writing of non-words and sentence comprehension. This clinical picture pointed to an atypical presentation of the non-fluent variety. The frequent symptom overlap between the different variants of PPA, most likely reflecting differences in the topography of the pathological changes, needs to be considered in the definition of diagnostic criteria.

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Gallée, Jeanne, Claire Cordella, Evelina Fedorenko, Daisy Hochberg, Alexandra Touroutoglou, Megan Quimby, and Bradford C. Dickerson. "Breakdowns in Informativeness of Naturalistic Speech Production in Primary Progressive Aphasia." Brain Sciences 11, no. 2 (January 20, 2021): 130. http://dx.doi.org/10.3390/brainsci11020130.

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“Functional communication” refers to an individual’s ability to communicate effectively in his or her everyday environment, and thus is a paramount skill to monitor and target therapeutically in people with aphasia. However, traditional controlled-paradigm assessments commonly used in both research and clinical settings often fail to adequately capture this ability. In the current study, facets of functional communication were measured from picture-elicited speech samples from 70 individuals with mild primary progressive aphasia (PPA), including the three variants, and 31 age-matched controls. Building upon methods recently used by Berube et al. (2019), we measured the informativeness of speech by quantifying the content of each patient’s description that was relevant to a picture relative to the total amount of speech they produced. Importantly, form-based errors, such as mispronunciations of words, unusual word choices, or grammatical mistakes are not penalized in this approach. We found that the relative informativeness, or efficiency, of speech was preserved in non-fluent variant PPA patients as compared with controls, whereas the logopenic and semantic variant PPA patients produced significantly less informative output. Furthermore, reduced informativeness in the semantic variant is attributable to a lower production of content units and a propensity for self-referential tangents, whereas for the logopenic variant, a lower production of content units and relatively ”empty” speech and false starts contribute to this reduction. These findings demonstrate that functional communication impairment does not uniformly affect all the PPA variants and highlight the utility of naturalistic speech analysis for measuring the breakdown of functional communication in PPA.

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Riley, Ellyn A., Elena Barbieri, Sandra Weintraub, M. Marsel Mesulam, and Cynthia K. Thompson. "Semantic Typicality Effects in Primary Progressive Aphasia." American Journal of Alzheimer's Disease & Other Dementiasr 33, no. 5 (March 16, 2018): 292–300. http://dx.doi.org/10.1177/1533317518762443.

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Prototypical items within a semantic category are processed faster than atypical items within the same category. This typicality effect reflects normal representation and processing of semantic categories and when absent may be reflective of lexical–semantic deficits. We examined typicality effects in individuals with semantic and nonsemantic variants of primary progressive aphasia (PPA; semantic—PPA-S, agrammatic—PPA-G), a neurodegenerative disorder characterized by specific decline in language function, and age-matched controls. Using a semantic category verification task, where participants were asked to decide whether visual or auditory words (category typical, atypical, or nonmembers) belonged within a specified superordinate category, we found a typicality effect (ie, faster response times for typical vs atypical items) for all participant groups. However, participants with more severe PPA-S did not show a typicality effect in either modality. Findings may reflect increased intracategory semantic blurring as the disease progresses and semantic impairment becomes more severe.

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Poole, Matthew L., Amy Brodtmann, David Darby, and Adam P. Vogel. "Motor Speech Phenotypes of Frontotemporal Dementia, Primary Progressive Aphasia, and Progressive Apraxia of Speech." Journal of Speech, Language, and Hearing Research 60, no. 4 (April 14, 2017): 897–911. http://dx.doi.org/10.1044/2016_jslhr-s-16-0140.

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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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Gleichgerrcht, Ezequiel, Teresa Torralva, María Roca, Daniela Szenkman, Agustin Ibanez, Pablo Richly, Mariángeles Pose, and Facundo Manes. "Decision Making Cognition in Primary Progressive Aphasia." Behavioural Neurology 25, no. 1 (2012): 45–52. http://dx.doi.org/10.1155/2012/606285.

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We sought to investigate the decision making profile of Primary Progressive Aphasia (PPA) by assessing patients diagnosed with this disease (n= 10), patients diagnosed with behavioral variant frontotemporal dementia (bvFTD,n= 35), and matched controls (n= 14) using the Iowa Gambling Task, a widely used test that mimics real-life decision making. Participants were also evaluated with a complete neuropsychological battery. Patients with PPA were unable to adopt an advantageous strategy on the IGT, which resulted in a flat performance, different to that exhibited by both controls (who showed advantageous decision making) and bvFTD patients (who showed risk-appetitive behavior). The decision making profile of PPA patients was not associated with performance on language tasks and did not differ between sub-variants of the disease (namely, semantic dementia and progressive nonfluent aphasia). Investigating decision making in PPA is crucial both from a theoretical perspective, as it can shed light about the way in which language interacts with other cognitive functions, as well as a clinical standpoint, as it could lead to a more objective detection of impairments of decision making deficits in this condition.

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Battistella, Giovanni, Maya Henry, Benno Gesierich, Stephen M. Wilson, Valentina Borghesani, Wendy Shwe, Zachary Miller, et al. "Differential intrinsic functional connectivity changes in semantic variant primary progressive aphasia." NeuroImage: Clinical 22 (2019): 101797. http://dx.doi.org/10.1016/j.nicl.2019.101797.

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Bouchard, Louis-Olivier, Maximiliano A. Wilson, Robert Laforce, and Simon Duchesne. "White Matter Damage in the Semantic Variant of Primary Progressive Aphasia." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 46, no. 04 (April 29, 2019): 373–82. http://dx.doi.org/10.1017/cjn.2019.37.

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ABSTRACT:Background: The semantic variant of primary progressive aphasia (svPPA) is a form of dementia, mainly featuring language impairment, for which the extent of white matter (WM) damage is less described than its associated grey matter (GM) atrophy. Our study aimed to characterise the extent of this damage using a sensitive and unbiased approach. Methods: We conducted a between-group study comparing 10 patients with a clinical diagnosis of svPPA, recruited between 2011 and 2014 at a tertiary reference centre, with 9 cognitively healthy, age-matched controls. From diffusion tensor imaging (DTI) data, we extracted fractional anisotropy (FA) values using a tract-based spatial statistics approach. We further obtained GM volumetric data using the Freesurfer automated segmentation tool. We compared both groups using non-parametric Wilcoxon rank-sum tests, correcting for multiple comparisons. Results: Demographic data showed that patients and controls were comparable. As expected, clinical data showed lower results in svPPA than controls on cognitive screening tests. Tractography showed impaired diffusion in svPPA patients, with FA mostly decreased in the longitudinal, uncinate, cingulum and external capsule fasciculi. Volumetric data show significant atrophy in svPPA patients, mostly in the left entorhinal, amygdala, inferior temporal, middle temporal, superior temporal and temporal pole cortices, and bilateral fusiform gyri. Conclusions: This syndrome appears to be associated not only with GM but also significant WM degeneration. Thus, DTI could play a role in the differential diagnosis of atypical dementia by specifying WM damage specific to svPPA.

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Ting, Simon Kang Seng, Heidi Foo, Pei Shi Chia, Shahul Hameed, Kok Pin Ng, Adeline Ng, and Nagaendran Kandiah. "Dyslexic Characteristics of Chinese-Speaking Semantic Variant of Primary Progressive Aphasia." Journal of Neuropsychiatry and Clinical Neurosciences 30, no. 1 (January 2018): 31–37. http://dx.doi.org/10.1176/appi.neuropsych.17040081.

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Caplan, Alyssa, Gabe Marx, Jonathan Elofson, Claire Lis, Lea Grinberg, Bruce Miller, and Howard Rosen. "A case of semantic variant primary progressive aphasia with Pick’s pathology." Neurocase 24, no. 2 (March 4, 2018): 90–94. http://dx.doi.org/10.1080/13554794.2018.1447134.

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Mesulam, M., E. Rogalski, C. Wieneke, D. Cobia, A. Rademaker, C. Thompson, and S. Weintraub. "Neurology of anomia in the semantic variant of primary progressive aphasia." Brain 132, no. 9 (June 8, 2009): 2553–65. http://dx.doi.org/10.1093/brain/awp138.

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Stalpaert, Jara, Elissa-Marie Cocquyt, Yana Criel, Lieselot Segers, Marijke Miatton, Tim Van Langenhove, Pieter van Mierlo, and Miet De Letter. "Language and Speech Markers of Primary Progressive Aphasia: A Systematic Review." American Journal of Speech-Language Pathology 29, no. 4 (November 12, 2020): 2206–25. http://dx.doi.org/10.1044/2020_ajslp-20-00008.

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Purpose This systematic review aimed to establish language and speech markers to support the clinical diagnosis of primary progressive aphasia (PPA) and its clinical phenotypes. Our first objective was to identify behavioral language and speech markers of early-stage PPA. Our second objective was to identify the electrophysiological correlates of the language and speech characteristics in PPA. Method The databases MEDLINE, Web of Science, and Embase were searched for relevant articles. To identify behavioral markers, the initial subjective complaints and the language and speech deficits detected during the initial diagnostic evaluation were summarized for PPA in general and each clinical variant according to the 2011 consensus diagnostic criteria (nonfluent variant [NFV], semantic variant, and logopenic variant [LV]). To identify electrophysiological markers, the studies in which event-related potentials (ERPs) were elicited by a language or speech paradigm in patients with PPA were included. Results In total, 114 relevant studies were identified, including 110 behavioral studies and only four electrophysiological studies. This review suggests that patients with the semantic variant could be accurately differentiated from the NFV and LV in the initial stages based on the consensus criteria. Nonetheless, the early differentiation between the NFV and LV is not straightforward. In the four electrophysiological studies, differences in the latency, amplitude, and topographical distribution of the semantic N400 component were found between patients with PPA and healthy controls. Conclusions To accurately differentiate the NFV from the LV, it could be important to assess the language and speech degeneration by more specific assessments and by more objective diagnostic methods that offer insights into the language-related processes. Electrophysiological markers of PPA were not identified in this review due to the low number of studies that investigated language-related ERPs. More controlled ERP studies in larger patient cohorts are needed to investigate the diagnostic applicability of language-related ERPs in PPA. Supplemental Material https://doi.org/10.23641/asha.12798080

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Taylor-Rubin, Cathleen, Karen Croot, Emma Power, Sharon A. Savage, John R. Hodges, and Leanne Togher. "Communication behaviors associated with successful conversation in semantic variant primary progressive aphasia." International Psychogeriatrics 29, no. 10 (June 8, 2017): 1619–32. http://dx.doi.org/10.1017/s1041610217000813.

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ABSTRACTBackground:Primary progressive aphasia (PPA) affects a range of language and cognitive domains that impact on conversation. Little is known about conversation breakdown in the semantic variant of PPA (svPPA, also known as semantic dementia). This study investigates conversation of people with svPPA.Methods:Dyadic conversations about everyday activities between seven individuals with svPPA and their partners, and seven control pairs were video recorded and transcribed. Number of words, turns, and length of turns were measured. Trouble-indicating behaviors (TIBs) and repair behaviors were categorized and identified as successful or not for each participant in each dyad.Results:In general, individuals with svPPA were active participants in conversation, taking an equal proportion of turns, but indicating a great deal of more trouble in conversation, shown by the significantly higher number of TIBs than evidenced by partners or control participants. TIBs were interactive (asking for confirmation with a shorter repetition of the original utterance or a repetition which included a request for specific information) and non-interactive (such as failing to take up or continue the topic or a minimal response) and unlike those previously reported for people with other PPA variants and dementia of the Alzheimer type. Communication behaviors of the partner were critical to conversational success.Conclusions:Examination of trouble and repair in 10-min conversations of individuals with svPPA and their important communication partners has potential to inform speech pathology interventions to enhance successful conversation, in svPPA and should be an integral part of the comprehensive care plan.

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Tippett, Donna C., and Argye E. Hillis. "Ethical and Practical Challenges of the Communication and Behavioral Manifestations of Primary Progressive Aphasia." Seminars in Speech and Language 41, no. 03 (June 2020): 249–56. http://dx.doi.org/10.1055/s-0040-1710062.

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AbstractThe communication and behavioral manifestations of primary progressive aphasia (PPA) present ethical and practical challenges for individuals with this clinical syndrome as well as for individuals who are involved closely in their care. In this article, cases representing all three PPA variants (logopenic variant, nonfluent agrammatic, semantic variant) are presented to illustrate commonly encountered situations in which self-determination is at risk in decisions about housing, driving, social interactions, finances, and treatment interventions. Potential approaches, including patient/family education, implementation of safeguards, redirection to meaningful activities, and protections against vulnerability in treatment decisions, are described to preserve autonomy in patients with this neurodegenerative clinical syndrome.

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Popal, Haroon, Megan Quimby, Daisy Hochberg, Bradford C. Dickerson, and Jessica A. Collins. "Altered functional connectivity of cortical networks in semantic variant Primary Progressive Aphasia." NeuroImage: Clinical 28 (2020): 102494. http://dx.doi.org/10.1016/j.nicl.2020.102494.

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Lee, Hyon, Seongho Seo, Sang-Yoon Lee, Hye Jin Jeong, Sung-Ho Woo, Kyoung-Min Lee, Yeong-Bae Lee, et al. "[18F]-THK5351 PET Imaging in Patients With Semantic Variant Primary Progressive Aphasia." Alzheimer Disease & Associated Disorders 32, no. 1 (2018): 62–69. http://dx.doi.org/10.1097/wad.0000000000000216.

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Chow, T. W., K. A. Links, D. L. Masterman, M. F. Mendez, and H. V. Vinters. "A case of semantic variant primary progressive aphasia with severe insular atrophy." Neurocase 18, no. 6 (December 2012): 450–56. http://dx.doi.org/10.1080/13554794.2011.627343.

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Dalton, Sarah Grace Hudspeth, Christine Shultz, Maya L. Henry, Argye E. Hillis, and Jessica D. Richardson. "Describing Phonological Paraphasias in Three Variants of Primary Progressive Aphasia." American Journal of Speech-Language Pathology 27, no. 1S (March 2018): 336–49. http://dx.doi.org/10.1044/2017_ajslp-16-0210.

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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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Troche, J., A. Garcia, A. Paris, and J. Reilly. "Dissociating Semantic Process versus Content in Visual Confrontation Naming of Semantic Variant Primary Progressive Aphasia." Procedia - Social and Behavioral Sciences 94 (October 2013): 22–23. http://dx.doi.org/10.1016/j.sbspro.2013.09.008.

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Milano, Nicholas J., John B. Williamson, and Kenneth M. Heilman. "Improved verbal learning in the semantic variant of primary progressive aphasia when using semantic cues." Neurocase 21, no. 3 (March 11, 2014): 345–50. http://dx.doi.org/10.1080/13554794.2014.894081.

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Seixas Lima, Bruna, Brian Levine, Naida L. Graham, Carol Leonard, David Tang-Wai, Sandra Black, and Elizabeth Rochon. "Impaired coherence for semantic but not episodic autobiographical memory in semantic variant primary progressive aphasia." Cortex 123 (February 2020): 72–85. http://dx.doi.org/10.1016/j.cortex.2019.10.008.

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Singh, Tarun D., Joseph R. Duffy, Edythe A. Strand, Mary M. Machulda, Jennifer L. Whitwell, and Keith A. Josephs. "Neuropsychiatric Symptoms in Primary Progressive Aphasia and Apraxia of Speech." Dementia and Geriatric Cognitive Disorders 39, no. 3-4 (2015): 228–38. http://dx.doi.org/10.1159/000369062.

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Aim: To conduct a prospective analysis of the neuropsychiatric symptoms (NPS) across the three categories of primary progressive aphasia (PPA) and progressive apraxia of speech (PAOS), compare the prevalence and nature of the symptoms, and look at which symptoms could be helpful to better differentiate these PPA and PAOS categories. Methods: A total of 106 consecutive patients with a diagnosis of semantic variant (n = 13), logopenic variant (n = 37), agrammatic variant (n = 15) or PAOS (n = 41) were included in this prospective study. The NPS were measured by the Neuropsychiatric Inventory Questionnaire. Results: There were 65 patients with PPA and 41 with PAOS diagnosis. The most distinguishing features between the two groups were anxiety, apathy, aberrant motor behavior and appetite, while among the subtypes of PPA they were disinhibition and appetite changes. PPA and PAOS patients initially exhibited depression, but with increased disease duration, PAOS patients showed apathy (55.5%) while PPA patients showed disinhibition (28.6%) and aberrant motor behavior (14.3%). Conclusion: Mood symptoms like anxiety and appetite changes are more likely to be present at initial stages of PPA, whereas behavioral symptoms like aberrant motor behavior and apathy are likely to occur early in PAOS. The NPS seem to evolve with the progression of the disease in both PPA and PAOS.

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Clark, Heather M., Rene L. Utianski, Joseph R. Duffy, Edythe A. Strand, Hugo Botha, Keith A. Josephs, and Jennifer L. Whitwell. "Western Aphasia Battery–Revised Profiles in Primary Progressive Aphasia and Primary Progressive Apraxia of Speech." American Journal of Speech-Language Pathology 29, no. 1S (February 21, 2020): 498–510. http://dx.doi.org/10.1044/2019_ajslp-cac48-18-0217.

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Purpose The primary aim was to examine the utility of the Western Aphasia Battery–Revised (WAB-R; Kertesz, 2007 ) for classifying variants of primary progressive aphasia (PPA). Traditional WAB-R metrics of Aphasia Quotient (AQ), subtest scores, WAB-R classification, and several novel metrics were examined. A secondary aim was to examine these same WAB-R metrics in individuals with primary progressive apraxia of speech (PPAOS). Method A retrospective analysis of WAB-R records from 169 participants enrolled in a study of neurodegenerative speech and language disorders was conducted. PPA/PPAOS classification was determined by consensus review of speech, language, and cognitive profiles. Scores on each of the WAB-R subtests were obtained to derive AQ, WAB-R aphasia profile, and 3 ratios reflecting relative performance on subtests. Results Mean AQ was significantly higher in the PPAOS group compared to all PPA variants except primary fluent aphasia. AQ above the normal cutoff was observed for 20% of participants with PPA. Significant main effects of group were noted for each of the subtests. Follow-up comparisons most frequently discriminated PPAOS, primary agrammatic aphasia (PAA), and logopenic progressive aphasia. Primary fluent aphasia and semantic dementia (SD) subtest scores were less distinctive, with the exception of Naming for SD, which was significantly lower than for PAA and PPAOS. When the WAB-R AQ detected aphasia, a classification of anomic aphasia was most frequently observed; this pattern held true for each of the PPA variants. The mean Information Content:Naming ratio was highest for SD, and the mean Comprehension:Fluency ratio was highest for PAA. Conclusions In the current study, AQ underestimated the presence of PPA and WAB-R classification did not distinguish among PPA classification determined by consensus. Performance on individual subtests and relative performance across subtests demonstrated inconsistent alignment with PPA classification. We conclude the WAB-R in isolation is inadequate to detect or characterize PPA. We instead suggest utilizing the WAB-R as 1 component of a comprehensive language and motor speech assessment when PPA is suspected.

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Collins, Jessica A., Victor Montal, Daisy Hochberg, Megan Quimby, Maria Luisa Mandelli, Nikos Makris, William W. Seeley, Maria Luisa Gorno-Tempini, and Bradford C. Dickerson. "Focal temporal pole atrophy and network degeneration in semantic variant primary progressive aphasia." Brain 140, no. 2 (December 31, 2016): 457–71. http://dx.doi.org/10.1093/brain/aww313.

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Jokel, Regina, Aneta Kielar, Nicole D. Anderson, Sandra E. Black, Elizabeth Rochon, Simon Graham, Morris Freedman, and David F. Tang-Wai. "Behavioural and neuroimaging changes after naming therapy for semantic variant primary progressive aphasia." Neuropsychologia 89 (August 2016): 191–216. http://dx.doi.org/10.1016/j.neuropsychologia.2016.06.009.

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