An Overview on Primary Progressive Aphasia and Its Variants

Abstract

We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: (1) Progressive nonfluent aphasia (PNFA), (2) semantic dementia (SD), (3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension. In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word-finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap within many cognitive domains. The neuroimaging analysis showed left perisylvian region involvement. A comprehensive cognitive, neuroimaging and pathological approach is necessary to identify the clinical and pathogenetic features of different PPA variants.

Syndromes dominated by apraxia of speech show distinct characteristics from agrammatic PPA

“…38 We recognize that some investigators do not support separating PPAOS from agPPA, either because AOS is "adequately captured under the heading progressive nonfluent aphasia" or because subjects with PPAOS will eventually develop agrammatism. 8,39,40 Seventeen subjects from our PPAOS cohort were seen on more than one occasion, either before or after participation in the current study. Those seen before maintained a stable diagnosis of PPAOS.…”

Change of Accent as an Atypical Onset of non Fluent Primary Progressive Aphasia

“…Patients with PPA-LV usually show a pattern of speech output that is slow, syntactically simple but correct, with frequent word-finding pauses, naming difficulties, phonemic paraphasias and impaired repetition [18,19]. In this variant, the core problem is a deficit in phonological short-term memory [3,19,36], which is related to involvement of the inferior parietal lobule [36][37][38].…”

“…Finally, PPA-LV patients exhibit word finding difficulties and decreased output, impaired naming and repetition in the context of spared semantic and syntactic abilities, while mantaining syntactically simple correct language output [3,18]. Phonemic paraphasias are also frequent, as well as an impairment in sentence comprehension especially for long sentences, whereas single word comprehension and semantic memory are preserved [18,19]. In these patients, atrophy is localized in the posterior temporal and inferior parietal regions of dominant hemisphere [16,18,19].…”

“…Phonemic paraphasias are also frequent, as well as an impairment in sentence comprehension especially for long sentences, whereas single word comprehension and semantic memory are preserved [18,19]. In these patients, atrophy is localized in the posterior temporal and inferior parietal regions of dominant hemisphere [16,18,19]. In the literature, diverse clinical presentations of language progressive disorders that do not fit the recent criteria have been described, including progressive anarthria [20][21][22][23] and progressive jargon aphasia [24].…”

Spoken discourse in Alzheimer' disease: a review

“…These observations have been interpreted as representing impairment in auditory verbal short term memory, which has been proposed as a central mechanism in LPA (Gorno-Tempini et al 2004). In addition to impaired language, neuropsychological profiling in LPA has identified impairments on tests of memory (Mesulam et al 2008), calculation (Amici et al 2006;Gorno-Tempini et al 2004;Rohrer et al 2010), and limb apraxia (Rohrer et al 2010).…”