Preferential Disruption of Auditory Word Representations in Primary Progressive Aphasia with the Neuropathology of FTLD-TDP Type A

Marek-Marsel Mesulam*, Matthew J. Nelson, Jungmoon Hyun, Benjamin Rader, Robert S. Hurley, Rosa Rademakers, Matthew C. Baker, Eileen H Bigio, Sandra Weintraub

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Four patients with primary progressive aphasia displayed a greater deficit in understanding words they heard than words they read, and a further deficiency in naming objects orally rather than in writing. All four had frontotemporal lobar degeneration-Transactive response DNA binding protein Type A neuropathology, three determined postmortem and one surmised on the basis of granulin gene (GRN) mutation. These features of language impairment are not characteristic of any currently recognized primary progressive aphasia variant. They can be operationalized as manifestations of dysfunction centered on a putative auditory word-form area located in the superior temporal gyrus of the left hemisphere. The small size of our sample makes the conclusions related to underlying pathology and auditory word-form area dysfunction tentative. Nonetheless, a deeper assessment of such patients may clarify the nature of pathways that link modality-specific word-form information to the associations that mediate their recognition as concepts. From a practical point of view, the identification of these features in patients with primary progressive aphasia should help in the design of therapeutic interventions where written communication modalities are promoted to circumvent some of the oral communication deficits.

Original languageEnglish (US)
Pages (from-to)46-53
Number of pages8
JournalCognitive and Behavioral Neurology
Volume32
Issue number1
DOIs
StatePublished - Mar 1 2019

Fingerprint

Primary Progressive Aphasia
Frontotemporal Dementia
Communication
Frontotemporal Lobar Degeneration
DNA-Binding Proteins
Temporal Lobe
Sample Size
Language
Pathology
Mutation
Genes
Neuropathology
Therapeutics

Keywords

  • anomia
  • auditory word-form area
  • granulin
  • primary progressive aphasia

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Cognitive Neuroscience
  • Psychiatry and Mental health

Cite this

Mesulam, Marek-Marsel ; Nelson, Matthew J. ; Hyun, Jungmoon ; Rader, Benjamin ; Hurley, Robert S. ; Rademakers, Rosa ; Baker, Matthew C. ; Bigio, Eileen H ; Weintraub, Sandra. / Preferential Disruption of Auditory Word Representations in Primary Progressive Aphasia with the Neuropathology of FTLD-TDP Type A. In: Cognitive and Behavioral Neurology. 2019 ; Vol. 32, No. 1. pp. 46-53.
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Preferential Disruption of Auditory Word Representations in Primary Progressive Aphasia with the Neuropathology of FTLD-TDP Type A. / Mesulam, Marek-Marsel; Nelson, Matthew J.; Hyun, Jungmoon; Rader, Benjamin; Hurley, Robert S.; Rademakers, Rosa; Baker, Matthew C.; Bigio, Eileen H; Weintraub, Sandra.

In: Cognitive and Behavioral Neurology, Vol. 32, No. 1, 01.03.2019, p. 46-53.

Research output: Contribution to journalArticle

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