Beyond language impairment: Profiles of apathy in primary progressive aphasia.

Primary progressive aphasia (PPA) is characterised by predominant language and communication impairment. However, behavioural changes, such as apathy, are increasingly recognised. Apathy is defined as a reduction in motivation and goal-directed behaviour. Recent theoretical models have suggested that apathy can be delineated into multiple dimensions: executive apathy (i.e., deficits in maintaining goals and organisation), emotional apathy (i.e., emotional blunting and indifference) and initiation apathy (i.e., reduced self-initiation). Whether the nature of apathy differs between clinical variants of PPA, and across early and late disease stages, remains to be established. Here, carers/informants of 20 semantic variant PPA (svPPA), 15 non-fluent variant PPA (nfvPPA), 16 logopenic variant PPA (lvPPA) and 25 healthy older controls completed the Dimensional Apathy Scale to quantify executive, emotional and initiation apathy. Voxel-based morphometry was used to identify associations between dimensions of apathy and regions of grey matter intensity decrease. Our behavioural results showed greater executive and initiation apathy in late svPPA than in late nfvPPA patients, while late svPPA had greater emotional apathy than both late nfvPPA and late lvPPA groups. Executive and initiation apathy were significantly higher than premorbid levels in all PPA subtypes, while elevated emotional apathy was only seen in early and late svPPA. Distinct neural correlates were identified across apathy dimensions. Executive apathy correlated with grey matter intensity of the left dorsolateral prefrontal and inferior parietal cortices; emotional apathy with the left medial prefrontal, insular and cerebellar regions; and initiation apathy with right parietal areas. Our findings are the first to reveal evidence of the dimensional nature of apathy in PPA, with different clinical signatures observed for each subtype. From a clinical standpoint, these results will inform the development of targeted interventions for specific aspects of apathy which emerge in PPA.

Behavioural and neuroimaging changes after naming therapy for semantic variant primary progressive aphasia.

The objectives of this study were to examine the effects of a successful naming intervention on naming performance and brain activity in individuals with the semantic variant of primary progressive aphasia (svPPA). Four participants with svPPA were scanned while performing phonologically- and semantically-based tasks before and after an intense, 20-h naming therapy that followed the principles of errorless learning whereby errors were eliminated from the learning process. Five healthy control participants were scanned at the outset of the study and did not receive treatment. The results showed that in svPPA participants, successful re-learning of forgotten vocabulary was accompanied by activation of a larger network in bilateral brain regions and that the level of activation in the left anterior lobe may be inversely correlated with severity of semantic impairment. Our findings have implications for treatment in svPPA patients and suggest that semantic cues can improve naming, in spite of significant semantic impairment. The results indicate that intensive language therapy can lead to behavioural gains and neuroplastic changes even in individuals with more advanced anterior temporal lobe atrophy.

Semantic dementia Brazilian study of nineteen cases.

The term semantic dementia was devised by Snowden et al. in 1989 and nowadays, the semantic dementia syndrome is recognized as one of the clinical forms of frontotemporal lobar degeneration (FTLD) and is characterized by a language semantic disturbance associated to non-verbal semantic memory impairment. OBJECTIVES: The aim of this study was to describe a Brazilian sample of 19 semantic dementia cases, emphasizing the clinical characteristics important for differential diagnosis of this syndrome. METHODS: Nineteen cases with semantic dementia were evaluated between 1999 and 2007. All patients were submitted to neurological evaluation, neuroimaging exams and cognitive, language and semantic memory evaluation. RESULTS: All patients presented fluent spontaneous speech, preservation of syntactic and phonological aspects of the language, word-finding difficulty, semantic paraphasias, word comprehension impairment, low performance in visual confrontation naming tasks, impairment on tests of non-verbal semantic memory and preservation of autobiographical memory and visuospatial skills. Regarding radiological investigations, temporal lobe atrophy and/or hypoperfusion were found in all patients. CONCLUSIONS: The cognitive, linguistic and of neuroimaging data in our case series corroborate other studies showing that semantic dementia constitutes a syndrome with well defined clinical characteristics associated to temporal lobe atrophy.

O termo demência semântica foi lançado por Snowden et al. em 1989 e, atualmente, a síndrome da demência semântica é reconhecida como uma das formas clínicas da degeneração lobar fronto-temporal (DLFT) e é caracterizada por distúrbio semântico da linguagem associado a comprometimento semântico não-verbal. OBJETIVOS: Este trabalho teve como objetivo descrever uma amostra brasileira de 19 casos de demência semântica, ressaltando as características clínicas importantes para a realização do diagnóstico diferencial desta síndrome. MÉTODOS: Foram estudados 19 casos com demência semântica avaliados entre 1999 e 2007. Todos os pacientes foram submetidos à avaliação neurológica, exames de neuroimagem e avaliação cognitiva, da linguagem e da memória semântica. RESULTADOS: Todos os pacientes apresentaram produção espontânea fluente, preservação dos aspectos sintáticos e fonológicos da linguagem, dificuldade em encontrar palavras, parafasias semânticas, dificuldade de compreensão de palavras, baixo desempenho em provas de nomeação por confrontação visual, falhas em provas que avaliam a memória semântica não-verbal e preservação da memória autobiográfica e de habilidades visuoespaciais. Em relação aos achados de neuroimagem, o comprometimento do lobo temporal (atrofia e/ou hipoperfusão) foi encontrado em todos os pacientes. CONCLUSÕES: Os achados cognitivos, lingüísticos e de neuroimagem do nosso grupo de pacientes corroboram outros estudos que mostram que a demência semântica constitui uma síndrome com características clínicas bem definidas associada à atrofia do lobo temporal.