Progressive aphasia secondary to Alzheimer disease vs FTLD pathology

Josephs, Keith A.; Whitwell, Jennifer L.; Duffy, Joseph R.; VanVoorst, Wendy A.; Strand, Edyth A.; Hu, William T.; Boeve, Bradley F.; Graff‐Radford, Neill R.; Parisi, Joseph E.; Knopman, David S.; Dickson, Dennis W.; Jack, Clifford R.; Petersen, Ronald C.

doi:10.1212/01.wnl.0000287073.12737.35

Cited by 145 publications

(166 citation statements)

References 40 publications

(44 reference statements)

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“…However, recent articles have demonstrated that focal, atypical distribution of Alzheimer disease (AD) pathology is responsible for 20% to 30% of cases with various forms of PPA. 2,[33][34][35] Retrospective PET and MRI studies 36,37 demonstrated in PPA patients with AD pathology a pattern of temporoparietal involvement similar to LPA. Furthermore, all US patients described in this article had cortical amyloid binding on PET scans using the Pittsburgh compound B tracer.…”

Section: Verbal Response Pointingmentioning

confidence: 99%

The logopenic/phonological variant of primary progressive aphasia

et al. 2008

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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.

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Section: Verbal Response Pointingmentioning

confidence: 99%

The logopenic/phonological variant of primary progressive aphasia

et al. 2008

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“…12 LPA is associated with left-hemisphere predominant atrophy in posterior temporal cortex and inferior parietal areas. 1,12,13 The term EO-AD applies to patients who meet criteria for AD, 14 but show onset of symptoms before the age of 65. Clinically, EO-AD patients present with an early multidomain cognitive impairment including language, visuospatial, and executive functions difficulties.…”

Section: Supplemental Data At Wwwneurologyorgmentioning

confidence: 99%

“…More surprising is that amyloid plaques and neurofibrillary tangles are also found in younger patients (under age 65) in association with atypical, focal, clinical syndromes in which a single cognitive domain, not related to memory, is predominantly affected. Examples of such syndromes are the logopenic variant of primary progressive aphasia (LPA) 1,2 and posterior cortical atrophy (PCA). [3][4][5] Both of these syndromes have been associated with a posterior pattern of atrophy, with relatively less involvement of the medial temporal lobe.…”

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confidence: 99%

Clinical syndromes associated with posterior atrophy

et al. 2009

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Objective: Posterior cortical atrophy (PCA) and logopenic progressive aphasia (LPA) are clinical syndromes associated with posterior brain atrophy. We compared PCA and LPA to each other and to an age-matched group of patients with early age at onset of Alzheimer disease (EO-AD). We hypothesized that these 3 syndromes are part of a single clinical and biologic continuum. Methods:Voxel-based morphometry (VBM) was used to assess atrophy in 14 PCA, 10 LPA, and 16 EO-AD patients compared to 65 healthy controls. Genetic analysis for APOE was conducted in 30 patients and 44 controls. Four patients came to autopsy. An additional 14 were studied with the beta-amyloid specific PET with tracer 11 C-labeled Pittsburgh Compound-B (PIB).Results: VBM results demonstrated that, compared to controls, each patient group showed a large area of overlapping atrophy in bilateral parietal, occipital, precuneus, posterior cingulate, posterior temporal, and hippocampal regions. Surrounding this common area, group-specific atrophy was found in small, symptom-specific regions for each group: the right ventral-occipital and superior parietal regions in PCA, the left middle and superior temporal gyri in LPA, and the prefrontal cortex in EO-AD. APOE ⑀4 frequency was higher in all patient groups compared to controls. Four PCA, 5 LPA, and 8 EO-AD patients showed evidence of cortical amyloid at pathology (n ϭ 3) or on PIB-PET (n ϭ 14).

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“…Subsequently, as the disease progresses other cognitive domains may become impaired leading to PPA-plus, although the language dysfunction remains the prominent impairment 7 . This particular isolation of language dysfunction is what distinguishes PPA from behavioral variant frontotemporal dementia (bvFTD) 8, 9 and typical forms of Alzheimer’s dementia 7 . PPA is classified into agrammatic (PPA-G), semantic (PPA-S), and logopenic (PPA-L) variants 10, 11 .…”

Section: Introductionmentioning

confidence: 99%

“…Even though there is a growing interest in the investigation of the nosology, neuropsychology, and neuropathology of PPA, little is known about the neuropsychiatric aspects of PPA 8,12 . Banks and Weintraub compared the frequency of NPS in PPA with that of bvFTD 13, 14 .…”

Section: Introductionmentioning

confidence: 99%

Neuropsychiatric Aspects of Primary Progressive Aphasia

Fatemi¹,

Boeve²,

Duffy³

et al. 2011

Journal of Neuropsychiatry

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Few studies have reported neuropsychiatric symptoms (NPS) in Primary Progressive Aphasia (PPA), a neurodegenerative disorder that primarily affects the left hemisphere. Depression is associated with left-sided stroke, but it remains unclear if depression and other NPS are also associated with PPA. The authors compared the frequency of NPS in 55 cases of PPA with 110 cognitively normal persons matched for age, sex and education. Depression, apathy, agitation, anxiety, appetite change, and irritability are associated with PPA. Hallucinations, delusion and night time behavior were not associated with PPA.

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Progressive aphasia secondary to Alzheimer disease vs FTLD pathology

Abstract: Background-The pathology causing progressive aphasia is typically a variant of frontotemporal lobar degeneration, especially with ubiquitin-positive-inclusions (FTLD-U). Less commonly the underlying pathology is Alzheimer disease (AD).

Cited by 145 publications

References 40 publications

The logopenic/phonological variant of primary progressive aphasia

The logopenic/phonological variant of primary progressive aphasia

Clinical syndromes associated with posterior atrophy

Neuropsychiatric Aspects of Primary Progressive Aphasia

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