Primary Progressive Aphasia

Cognitive Neurology and Alzheimer's Disease Center, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Alzheimer Disease and Associated Disorders (Impact Factor: 2.44). 10/2007; 21(4):S8-S11. DOI: 10.1097/WAD.0b013e31815bf7e1
Source: PubMed


The diagnosis of primary progressive aphasia (PPA) is made in any patient in whom a language impairment (aphasia), caused by a neurodegenerative disease (progressive), constitutes the most salient aspect of the clinical picture (primary). The language impairment can be fluent or nonfluent and may or may not interfere with word comprehension. Memory for recent events is relatively preserved although memory scores obtained in verbally mediated tests may be abnormal. Lesser changes in behavior and object recognition may be present but are not the leading factors that bring the patient to medical attention. This selective clinical pattern is most conspicuous in the initial stages of the disease. Progressive nonfluent aphasia and some types of semantic dementia can be considered subtypes of PPA. Initially brought to the attention of contemporary literature 25 years ago, PPA has recently witnessed substantial progress related to its neurolinguistic features, neuroanatomy, imaging, neuropathology, genetics, and risk factors.

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    • "Both men and women are equally affected and usually present gradual changes in behavior, social awareness, and language. There are three main clinical subtypes of FTLD: behavioral variant Frontotemporal Dementia (bvFTD), in which changes in behavior and social conduct predominate; Progressive Non-Fluent Aphasia (PNFA) characterized by loss of fluency of speech, agrammatism and intact word comprehension [29] [64] [65]; and Semantic Dementia (SD), with loss of long-established knowledge of words, with otherwise fluent and grammatically faultless speech [38] [86]. In more advanced disease stages, FTLD patients can show overlap of the above clinical presentations and may also develop symptoms of motor neuron disease (MND) and corticobasal syndrome (CBS) [12] [47] [48] [54]. "
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    ABSTRACT: Cerebrospinal fluid (CSF) biomarkers have been increasingly studied for dementia diagnosis, however the accuracy to distinguish between different forms of dementia is still unsatisfactory. In this study, the added value of another CSF Aβ-peptide (Aβ40), along with the core CSF markers t-Tau, p-Tau, and Aβ42, in the discrimination between two large dementia groups of Frontotemporal Lobar Degeneration (FTLD; n=107), Alzheimer's Disease (AD; n=107) and non-demented subjects (n=33) was evaluated. In FTLD, t-Tau and p-Tau were significantly increased in relation to controls, but lower than in AD, while Aβ42 was similar in FTLD and controls, but higher than in AD. Equally reduced Aβ40 levels were seen in both dementia groups, and therefore the combination of Aβ40 with core CSF biomarkers optimally discriminated FTLD and AD patients from controls. Aβ42 and t-Tau were selected as the best biomarker subset to differentiate FTLD from AD, with no added value of Aβ40 to the model. Diagnostic accuracy between FTLD and AD was still sub-optimal, with a significant percentage (23%) of FTLD patients, in particularly women, carrying an ApoE-ε4 allele, showing a CSF-AD biomarkers profile. Although CSF Aβ40 does not appear to have an additional value in the distinction between FTLD and AD, it increases the discrimination between subjects with dementia from controls. A CSF-AD biomarker profile can be seen in patients with a clinical phenotype of FTLD, reinforcing the need for autopsy confirmation.
    Full-text · Article · Sep 2015 · Journal of the neurological sciences
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    • "The inclusion criteria for the bvFTD and PPA samples were based on the Neary and Mesulam criteria, respectively [3] [10] [11]. The criteria focused on a predominance of a frontal or temporal lobe cognitive/ behavioral syndrome and the absence or insignificance of an anterograde amnesia and visuospatial impairment in the initial clinical presentations (i.e. "
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    ABSTRACT: Introduction The Clinical Dementia Rating Scale (CDR) is a tool designed to quantify the severity of dementia symptoms and is also useful to assess disease progression, in Alzheimer‘s disease (AD). A new version of the scale was developed by adding two extra domains that focused on the core aspects of frontotemporal dementia symptomatology, Language and Behavior/Comportment/Personality. Objectives In this study, we adapted and validated the modified CDR scale in our setting and language (Rioplatense-Spanish). Materials and Methods 46 patients with probable AD, 27 behavioral variant of Frontotemporal dementia (bvFTD), 18 Primary Progressive Aphasia (PPA) and 40 healthy participants were included. The adapted version of the scale was administered by a blind rater who interviewed patients together with patient’s caregiver. Results Using ROC curves, the domain language and behavior were superior to the memory domain in accuracy for detecting PPA and bvFTD, respectively, but both of them had equivalent diagnostic accuracies for probable AD. Logistic regression analyses showed that either the LANG or BEHAV domains significantly improved the discrimination between probable AD, bvFTD and PPA. Conclusions The Spanish version of the modified CDR adds value for the characterization of the non-amnestic symptoms in patients with neurodegenerative dementias.
    Full-text · Article · Sep 2014 · Journal of the Neurological Sciences
    • "The nonfluent/ agrammatic variant (nfvPPA) is characterised by agrammatic language production and/or apraxia of speech, the semantic variant (svPPA) by impaired confrontation naming and single-word comprehension, and the logopenic variant (lvPPA) by anomia and impaired repetition of sentences and phrases. Difficulty in word retrieval is an early, prominent, and debilitating symptom for people with all three variants of PPA (Mesulam, 2001). PPA has a profound effect on a person's ability to communicate, which in turn affects a person's relationships, social networks, and ability to participate in a multitude of everyday activities that depend on communication (Nickels & Croot, 2014). "
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    ABSTRACT: Background: The aim of lexical retrieval treatment for people with anomia is not just to improve accessibility of lexical items for confrontation naming but to carry over this improvement to communicative situations. However, there is no consensus on what measures are the most suitable to evaluate whether such changes have occurred. Anomia is one of the core presenting symptoms for people with primary progressive aphasia (PPA), yet while there is increasing evidence for the efficacy of word retrieval treatments in PPA, there is minimal information about how improvements in picture naming transfer to connected speech.
    No preview · Article · May 2014 · Aphasiology
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