Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia

Jessica De Leon, Maria Luisa Mandelli, Amber Nolan, Zachary A. Miller, Christie Mead, Christa Watson, Ariane E. Welch, Maya L. Henry, Viktoriya Bourakova, Renaud La Joie, Lynn P. Bajorek, Lea Grinberg, Gil Rabinovici, Bruce L. Miller, Maria Luisa Gorno-Tempini

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

A 66-year-old woman presented with agrammatism and apraxia of speech, meeting criteria for non-fluent/agrammatic variant primary progressive aphasia (nfvPPA). However, three years later, she developed frontal/executive, short-term phonological memory, visuospatial, and visual memory deficits suggesting involvement of multiple brain networks. Multimodal neuroimaging showed damage of both fronto-striatal and posterior brain regions. She was found to have multiple pathological processes: corticobasal degeneration (CBD), Alzheimer’s disease (AD), and TAR DNA-binding protein (TDP)-43 type A. We hypothesize that cognitive and neuroimaging findings consistent with damage to multiple brain networks, each associated with vulnerability to certain molecular disease subtypes, could indicate mixed pathology.

Original languageEnglish (US)
Pages (from-to)39-47
Number of pages9
JournalNeurocase
Volume25
Issue number1-2
DOIs
StatePublished - Mar 4 2019

Fingerprint

Primary Progressive Nonfluent Aphasia
Pathology
Neuroimaging
Brain
Primary Progressive Aphasia
Broca Aphasia
Corpus Striatum
Apraxias
Memory Disorders
DNA-Binding Proteins
Pathologic Processes
Short-Term Memory
Alzheimer Disease
Damage

Keywords

  • Alzheimer’s disease neuropathology
  • Non-fluent agrammatic primary progressive aphasia
  • TDP-43 type A neuropathology
  • corticobasal degeneration
  • syntax network

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

Cite this

De Leon, J., Mandelli, M. L., Nolan, A., Miller, Z. A., Mead, C., Watson, C., ... Gorno-Tempini, M. L. (2019). Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia. Neurocase, 25(1-2), 39-47. https://doi.org/10.1080/13554794.2019.1609522

Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia. / De Leon, Jessica; Mandelli, Maria Luisa; Nolan, Amber; Miller, Zachary A.; Mead, Christie; Watson, Christa; Welch, Ariane E.; Henry, Maya L.; Bourakova, Viktoriya; La Joie, Renaud; Bajorek, Lynn P.; Grinberg, Lea; Rabinovici, Gil; Miller, Bruce L.; Gorno-Tempini, Maria Luisa.

In: Neurocase, Vol. 25, No. 1-2, 04.03.2019, p. 39-47.

Research output: Contribution to journalArticle

De Leon, J, Mandelli, ML, Nolan, A, Miller, ZA, Mead, C, Watson, C, Welch, AE, Henry, ML, Bourakova, V, La Joie, R, Bajorek, LP, Grinberg, L, Rabinovici, G, Miller, BL & Gorno-Tempini, ML 2019, 'Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia', Neurocase, vol. 25, no. 1-2, pp. 39-47. https://doi.org/10.1080/13554794.2019.1609522
De Leon, Jessica ; Mandelli, Maria Luisa ; Nolan, Amber ; Miller, Zachary A. ; Mead, Christie ; Watson, Christa ; Welch, Ariane E. ; Henry, Maya L. ; Bourakova, Viktoriya ; La Joie, Renaud ; Bajorek, Lynn P. ; Grinberg, Lea ; Rabinovici, Gil ; Miller, Bruce L. ; Gorno-Tempini, Maria Luisa. / Atypical clinical features associated with mixed pathology in a case of non-fluent variant primary progressive aphasia. In: Neurocase. 2019 ; Vol. 25, No. 1-2. pp. 39-47.
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