Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study
Introduction. Primary progressive aphasia (PPA) is a clinically variable syndrome manifesting as slow progressive loss of speech and language with multiple underlying neurodegenerative pathologies. Materials and Methods. We included data from nine PPA patients with available autopsies. We then retro...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Hindawi Limited
2022-01-01
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| Series: | Behavioural Neurology |
| Online Access: | http://dx.doi.org/10.1155/2022/6075511 |
| _version_ | 1797995464418656256 |
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| author | Robert Rusina Radoslava Bajtosova Zsolt Cséfalvay Jiri Keller Anna Kavkova Jaromír Kukal Radoslav Matej |
| author_facet | Robert Rusina Radoslava Bajtosova Zsolt Cséfalvay Jiri Keller Anna Kavkova Jaromír Kukal Radoslav Matej |
| author_sort | Robert Rusina |
| collection | DOAJ |
| description | Introduction. Primary progressive aphasia (PPA) is a clinically variable syndrome manifesting as slow progressive loss of speech and language with multiple underlying neurodegenerative pathologies. Materials and Methods. We included data from nine PPA patients with available autopsies. We then retrospectively reviewed all available medical records, neuropsychology, and MRI results to confirm the corresponding subtypes of PPA and compared them with postmortem neuropathological results. Results. Clinical presentations corresponded to the nonfluent/agrammatic variant in six cases, the semantic variant in one case, the logopenic variant in one case, and the mixed variant (concomitant nonfluent/agrammatic plus semantic variant) in one case. Patients with a broader clinical presentation, i.e., combining manifestations of one PPA subtype and symptoms of another PPA variant, had autopsy comorbidities showing multiple neurodegenerative disorders. Of the nine subjects enrolled in the study, Alzheimer’s disease (AD) was found in eight cases; however, in only one case, AD was detected as an isolated neuropathological substrate of PPA. In eight brain samples, different comorbid neuropathologies were detected: three cases with comorbid AD and dementia with Lewy bodies, two cases with comorbid AD and TDP-43 pathology, one case with comorbid AD and complex tauopathies, and one case with comorbid AD with both tau and TDP-43 deposits. Finally, one case had comorbid tau and TDP-43 pathology but without comorbid AD pathology. Conclusions. Our observation suggests that PPA cases could be more heterogeneous in their etiology than previously thought and underlying neurodegenerative comorbidities should be considered in routine practice, especially if the clinical presentation of PPA is atypical. |
| first_indexed | 2024-04-11T10:01:50Z |
| format | Article |
| id | doaj.art-81ac374420374dc8b511afb25a6a385b |
| institution | Directory Open Access Journal |
| issn | 1875-8584 |
| language | English |
| last_indexed | 2024-04-11T10:01:50Z |
| publishDate | 2022-01-01 |
| publisher | Hindawi Limited |
| record_format | Article |
| series | Behavioural Neurology |
| spelling | doaj.art-81ac374420374dc8b511afb25a6a385b2022-12-22T04:30:23ZengHindawi LimitedBehavioural Neurology1875-85842022-01-01202210.1155/2022/6075511Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center StudyRobert Rusina0Radoslava Bajtosova1Zsolt Cséfalvay2Jiri Keller3Anna Kavkova4Jaromír Kukal5Radoslav Matej6Department of NeurologyDepartment of NeurologyDepartment of Communication DisordersThird Faculty of MedicineThird Faculty of MedicineFaculty of Nuclear Sciences and Physical EngineeringThird Faculty of MedicineIntroduction. Primary progressive aphasia (PPA) is a clinically variable syndrome manifesting as slow progressive loss of speech and language with multiple underlying neurodegenerative pathologies. Materials and Methods. We included data from nine PPA patients with available autopsies. We then retrospectively reviewed all available medical records, neuropsychology, and MRI results to confirm the corresponding subtypes of PPA and compared them with postmortem neuropathological results. Results. Clinical presentations corresponded to the nonfluent/agrammatic variant in six cases, the semantic variant in one case, the logopenic variant in one case, and the mixed variant (concomitant nonfluent/agrammatic plus semantic variant) in one case. Patients with a broader clinical presentation, i.e., combining manifestations of one PPA subtype and symptoms of another PPA variant, had autopsy comorbidities showing multiple neurodegenerative disorders. Of the nine subjects enrolled in the study, Alzheimer’s disease (AD) was found in eight cases; however, in only one case, AD was detected as an isolated neuropathological substrate of PPA. In eight brain samples, different comorbid neuropathologies were detected: three cases with comorbid AD and dementia with Lewy bodies, two cases with comorbid AD and TDP-43 pathology, one case with comorbid AD and complex tauopathies, and one case with comorbid AD with both tau and TDP-43 deposits. Finally, one case had comorbid tau and TDP-43 pathology but without comorbid AD pathology. Conclusions. Our observation suggests that PPA cases could be more heterogeneous in their etiology than previously thought and underlying neurodegenerative comorbidities should be considered in routine practice, especially if the clinical presentation of PPA is atypical.http://dx.doi.org/10.1155/2022/6075511 |
| spellingShingle | Robert Rusina Radoslava Bajtosova Zsolt Cséfalvay Jiri Keller Anna Kavkova Jaromír Kukal Radoslav Matej Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study Behavioural Neurology |
| title | Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study |
| title_full | Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study |
| title_fullStr | Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study |
| title_full_unstemmed | Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study |
| title_short | Comorbid Neurodegeneration in Primary Progressive Aphasia: Clinicopathological Correlations in a Single-Center Study |
| title_sort | comorbid neurodegeneration in primary progressive aphasia clinicopathological correlations in a single center study |
| url | http://dx.doi.org/10.1155/2022/6075511 |
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