The taxonomy of primary progressive aphasia It walks and quacks like a duck ... but which duck?

From the University of Massachusetts Medical School, Worcester.
Neurology (Impact Factor: 8.29). 02/2011; 76(11):942-3. DOI: 10.1212/WNL.0b013e3182104130
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Available from: David A Drachman, Feb 04, 2015
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    • "This supports the hypothesis that different aphasia variants-phenotypes might represent stable patterns of symptoms subtended by specific underlying diseases with predictable histopathological and genetic patterns (Grossman, 2010). This multimodal approach to aphasia is promising; thus far, however, consistent correspondence between aphasia variants and specific pathological entities has not been found (Drachman, 2011). However, aphasia is not just a clinical entity. "
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    ABSTRACT: We investigated whether primary progressive aphasias (PPA) reflect non-random degradation of linguistic dimensions that might be supported by different neural subsystems and to what extent this degradation contributes to the emergence of clinical entities: semantic (S), logopenic (L) and nonfluent (NF) aphasia; apraxia of speech was also considered if associated with language disorders (AOS/aph). Forty-two aphasic patients are reported. Two main definable patterns of linguistic deficits tended to emerge that corresponded with identifiable patterns of brain atrophy, and probably diseases: the S variant, which principally expresses the impact of a "deep" cognitive (semantic) disorder on language, and AOS/aph in which "peripheral" executive components play a significant role. By contrast, NF aphasia emerged as a heterogeneous variant due to disorganization of various dimensions within the linguistic domain, that assumes different patterns depending on the differential distribution of atrophy in the perisylvian regions.
    Brain and Language 06/2014; 135C:57-65. DOI:10.1016/j.bandl.2014.05.004 · 3.22 Impact Factor

  • Journal of neurology, neurosurgery, and psychiatry 07/2012; 83(7):671. DOI:10.1136/jnnp-2012-302534 · 6.81 Impact Factor
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    ABSTRACT: Background: Logopenic variant of primary progressive aphasia (LPPA) is classically considered as an isolated language disorder, but verbal short-term memory deficit induces difficulties in neuropsychological tests that are not intended to evaluate language. Objective: The aim of this study is to describe the initial symptoms and neuropsychological profiles of LPPA. Methods: A retrospective study was conducted with a series of 20 consecutive patients diagnosed with LPPA. Clinical, neuroimaging, neuropsychological, and linguistic examinations are reported. The first neuropsychological examinations (mean time between neuropsychological assessment and diagnosis: 11 months) were then compared to 20 patients with mild cognitive impairment (MCI) and 20 patients with Alzheimer's disease (AD) matched by age, gender, and education level. Results: A recent onset or aggravation of anxiety disorders was frequently reported. An unusual neuropsychological profile, different from that of AD or MCI, was observed: dissociation between verbal and visual memory performances, poor encoding performances on verbal memory tests, and preserved orientation to time, difficulties with mental calculation and fluency tasks. Biparetal abnormality and left hippocampal diaschisis was frequently observed. Asymptomatic dopaminergic depletion was observed in four patients. Conclusion: Our study identifies that de novo or recently worsening anxiety and specific neuropsychological profiles call for screening for LPPA, including a linguistic examination. Sometimes, there may be a continuum between LPPA and corticobasal syndrome.
    Journal of Alzheimer's disease: JAD 05/2013; 36(4). DOI:10.3233/JAD-122335 · 4.15 Impact Factor
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