Primary Progressive Aphasia in the Network of French Alzheimer Plan Memory Centers

Magnin, Éloi; Démonet, Jean‐François; Wallon, David; Dumurgier, Julien; Troussière, Anne-Cécile; Jager, Alain; Duron, Emmanuelle; Gabelle, Audrey; Sayette, Vincent de La; Volpe‐Gillot, Lisette; Tio, Grégory; Evain, S.; Boutoleau‐Bretonnière, Claire; Enderlé, Adeline; Mouton-Liger, François; Robert, Philippe; Hannequin, Didier; Pasquier, Florence; Hugon, Jacques; Paquet, Claire

doi:10.3233/jad-160536

Cited by 33 publications

(32 citation statements)

References 46 publications

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“…Likewise, some patients who fit the semantic or agrammatic classification also fulfilled criteria for lvPPA. The prevalence of mixed/unclassifiable phenotypes is consistent with most large cohorts published to date, 19,22–24,33,38 but lower than others. 64–67 The heterogeneous pathologies found at autopsy of PPA-M/U subjects suggest that the ambiguities in the current PPA classification cannot be easily resolved through the addition of a fourth clinical variant.…”

Section: Discussionsupporting

confidence: 88%

Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia

Bergeron

Gorno‐Tempini

Rabinovici

et al. 2018

Annals of Neurology

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149

111

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Objective: To estimate the prevalence of amyloid positivity, defined by positron emission tomography (PET)/cerebro-spinal fluid (CSF) biomarkers and/or neuropathological examination, in primary progressive aphasia (PPA) variants. Methods: We conducted a meta-analysis with individual participant data from 1,251 patients diagnosed with PPA (including logopenic [lvPPA, n = 443], nonfluent [nfvPPA, n = 333], semantic [svPPA, n = 401], and mixed/unclassifiable [n = 74] variants of PPA) from 36 centers, with a measure of amyloid-β pathology (CSF [n = 600], PET [n = 366], and/or autopsy [n = 378]) available. The estimated prevalence of amyloid positivity according to PPA variant, age, and apolipoprotein E (ApoE) ε4 status was determined using generalized estimating equation models. Results: Amyloid-β positivity was more prevalent in lvPPA (86%) than in nfvPPA (20%) or svPPA (16%; p < 0.001). Prevalence of amyloid-β positivity increased with age in nfvPPA (from 10% at age 50 years to 27% at age 80 years, p < 0.01) and svPPA (from 6% at age 50 years to 32% at age 80 years, p < 0.001), but not in lvPPA (p = 0.94). Across PPA variants, ApoE ε4 carriers were more often amyloid-β positive (58.0%) than noncarriers (35.0%, p < 0.001). Autopsy data revealed Alzheimer disease pathology as the most common pathologic diagnosis in lvPPA (76%), frontotemporal lobar degeneration–TDP-43 in svPPA (80%), and frontotemporal lobar degeneration–TDP-43/tau in nfvPPA (64%). Interpretation: This study shows that the current PPA classification system helps to predict underlying pathology across different cohorts and clinical settings, and suggests that age and ApoE genotype should be considered when interpreting amyloid-β biomarkers in PPA patients.

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Section: Discussionsupporting

confidence: 88%

Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia

Bergeron

Gorno‐Tempini

Rabinovici

et al. 2018

Annals of Neurology

Self Cite

149

111

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“…A majority of patients will have a tauopathy such as progressive supranuclear palsy or corticobasal degeneration at post-mortem though a substantial (and still uncertain) minority represent TDP-43 or Alzheimer pathology [3, 12, 33–35]. While there are currently few reliable predictors of underlying pathology in individual patients [36], prominent apraxia of speech and parkinsonism are more closely associated with tauopathy than with TDP-43 pathology [12, 35].…”

Section: Canonical Syndromes Of Primary Progressive Aphasia: Nonfluenmentioning

confidence: 99%

“…Beyond clinical characterisation, molecular stratification will become increasingly important with trials of candidate disease-modifying treatments on the horizon [ 35 ]. However, most patients presenting with a language complaint will not have PPA and further, a number of patients with PPA (as many as 40% in some series) do not conform closely to one of the canonical syndromic diagnoses [ 3 , 13 , 15 , 16 , 34 ]. Some of these less common, atypical variants are in Table 1 .…”

Section: Toward the Diagnosis: A Path With Pitfallsmentioning

confidence: 99%

“…Despite much recent attention in the scientific literature [ 1 , 2 ], these ‘language-led dementias’ remain daunting for even experienced clinicians to diagnose and manage. This is not surprising: PPA is uncommon (estimated prevalence is conservatively around three cases per 100,000 [ 3 , 4 ]), the underlying pathology is heterogeneous and generally inaccessible and the functions principally targeted are uniquely complex. Although patients with PPA have been described for well over a century [ 5 ], the true significance of these disorders was only appreciated quite recently [ 6 , 7 ] and the paradigm of selective brain network degeneration caused by pathogenic protein spread has transformed our understanding of neurodegenerative disease [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Primary progressive aphasia: a clinical approach

et al. 2018

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The primary progressive aphasias are a heterogeneous group of focal ‘language-led’ dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including ‘clinical pearls’ that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic ‘roadmap’. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.Electronic supplementary materialThe online version of this article (10.1007/s00415-018-8762-6) contains supplementary material, which is available to authorized users.

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“…Despite much recent attention in the scientific literature [1,2], these 'language-led dementias' remain daunting for even experienced clinicians to diagnose and manage. This is not surprising: PPA is uncommon (estimated prevalence is conservatively around three cases per 100,000 [3,4]), the underlying pathology is heterogeneous and generally inaccessible and the functions principally targeted are uniquely complex. Although patients with PPA have been described for well over a century [5], the true significance of these disorders was only appreciated quite recently [6,7] and the paradigm of selective brain network degeneration caused by pathogenic protein spread has transformed our understanding of neurodegenerative disease [8].…”

Section: Introductionmentioning

confidence: 99%

Faculty Opinions recommendation of Primary progressive aphasia: a clinical approach.

Tippett

2020

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature

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The primary progressive aphasias are a heterogeneous group of focal 'language-led' dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including 'clinical pearls' that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic 'roadmap'. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.

show abstract

Primary Progressive Aphasia in the Network of French Alzheimer Plan Memory Centers

Cited by 33 publications

References 46 publications

Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia

Prevalence of amyloid‐β pathology in distinct variants of primary progressive aphasia

Primary progressive aphasia: a clinical approach

Faculty Opinions recommendation of Primary progressive aphasia: a clinical approach.

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