The logopenic/phonological variant of primary progressive aphasia

M. L. Gorno-Tempini, S. M. Brambati, V. Ginex, J. Ogar, Nina Dronkers, A. Marcone, D. Perani, V. Garibotto, S. F. Cappa, B. L. Miller

Research output: Contribution to journalArticle

372 Citations (Scopus)

Abstract

Objective: Primary progressive aphasia (PPA) is characterized by isolated decline in language functions. Semantic dementia and progressive nonfluent aphasia are accepted PPA variants. A "logopenic" variant (LPA) has also been proposed, but its cognitive and anatomic profile is less defined. The aim of this study was to establish the cognitive and anatomic features of LPA. Methods: Six previously unreported LPA cases underwent extensive neuropsychological evaluation and an experimental study of phonological loop functions, including auditory and visual span tasks with digits, letters, and words. For each patient, a voxel-wise, automated analysis of MRI or SPECT data were conducted using SPM2. Results: In LPA, speech rate was slow, with long word-finding pauses. Grammar and articulation were preserved, although phonological paraphasias could be present. Repetition and comprehension were impaired for sentences but preserved for single words, and naming was moderately affected. Investigation of phonological loop functions showed that patients were severely impaired in digit, letter, and word span tasks. Performance did not improve with pointing, was influenced by word length, and did not show the normal phonological similarity effect. Atrophy or decreased blood flow was consistently found in the posterior portion of the left superior and middle temporal gyri and inferior parietal lobule. Conclusions: Logopenic progressive aphasia (LPA) is a distinctive variant of primary progressive aphasia. Cognitive and neuroimaging data indicate that a deficit in phonological loop functions may be the core mechanism underlying the LPA clinical syndrome. Recent studies suggest that Alzheimer disease may be the most common pathology underlying the LPA clinical syndrome.

Original languageEnglish (US)
Pages (from-to)1227-1234
Number of pages8
JournalNeurology
Volume71
Issue number16
DOIs
StatePublished - Oct 14 2008

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Primary Progressive Aphasia
Primary Progressive Nonfluent Aphasia
Frontotemporal Dementia
Parietal Lobe
Aphasia
Temporal Lobe
Single-Photon Emission-Computed Tomography
Neuroimaging
Atrophy
Alzheimer Disease
Language
Pathology

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Gorno-Tempini, M. L., Brambati, S. M., Ginex, V., Ogar, J., Dronkers, N., Marcone, A., ... Miller, B. L. (2008). The logopenic/phonological variant of primary progressive aphasia. Neurology, 71(16), 1227-1234. https://doi.org/10.1212/01.wnl.0000320506.79811.da

The logopenic/phonological variant of primary progressive aphasia. / Gorno-Tempini, M. L.; Brambati, S. M.; Ginex, V.; Ogar, J.; Dronkers, Nina; Marcone, A.; Perani, D.; Garibotto, V.; Cappa, S. F.; Miller, B. L.

In: Neurology, Vol. 71, No. 16, 14.10.2008, p. 1227-1234.

Research output: Contribution to journalArticle

Gorno-Tempini, ML, Brambati, SM, Ginex, V, Ogar, J, Dronkers, N, Marcone, A, Perani, D, Garibotto, V, Cappa, SF & Miller, BL 2008, 'The logopenic/phonological variant of primary progressive aphasia', Neurology, vol. 71, no. 16, pp. 1227-1234. https://doi.org/10.1212/01.wnl.0000320506.79811.da
Gorno-Tempini ML, Brambati SM, Ginex V, Ogar J, Dronkers N, Marcone A et al. The logopenic/phonological variant of primary progressive aphasia. Neurology. 2008 Oct 14;71(16):1227-1234. https://doi.org/10.1212/01.wnl.0000320506.79811.da
Gorno-Tempini, M. L. ; Brambati, S. M. ; Ginex, V. ; Ogar, J. ; Dronkers, Nina ; Marcone, A. ; Perani, D. ; Garibotto, V. ; Cappa, S. F. ; Miller, B. L. / The logopenic/phonological variant of primary progressive aphasia. In: Neurology. 2008 ; Vol. 71, No. 16. pp. 1227-1234.
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AU - Brambati, S. M.

AU - Ginex, V.

AU - Ogar, J.

AU - Dronkers, Nina

AU - Marcone, A.

AU - Perani, D.

AU - Garibotto, V.

AU - Cappa, S. F.

AU - Miller, B. L.

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N2 - Objective: Primary progressive aphasia (PPA) is characterized by isolated decline in language functions. Semantic dementia and progressive nonfluent aphasia are accepted PPA variants. A "logopenic" variant (LPA) has also been proposed, but its cognitive and anatomic profile is less defined. The aim of this study was to establish the cognitive and anatomic features of LPA. Methods: Six previously unreported LPA cases underwent extensive neuropsychological evaluation and an experimental study of phonological loop functions, including auditory and visual span tasks with digits, letters, and words. For each patient, a voxel-wise, automated analysis of MRI or SPECT data were conducted using SPM2. Results: In LPA, speech rate was slow, with long word-finding pauses. Grammar and articulation were preserved, although phonological paraphasias could be present. Repetition and comprehension were impaired for sentences but preserved for single words, and naming was moderately affected. Investigation of phonological loop functions showed that patients were severely impaired in digit, letter, and word span tasks. Performance did not improve with pointing, was influenced by word length, and did not show the normal phonological similarity effect. Atrophy or decreased blood flow was consistently found in the posterior portion of the left superior and middle temporal gyri and inferior parietal lobule. Conclusions: Logopenic progressive aphasia (LPA) is a distinctive variant of primary progressive aphasia. Cognitive and neuroimaging data indicate that a deficit in phonological loop functions may be the core mechanism underlying the LPA clinical syndrome. Recent studies suggest that Alzheimer disease may be the most common pathology underlying the LPA clinical syndrome.

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