Apraxia of Speech in the Spontaneous Speech of Nonfluent/Agrammatic Primary Progressive Aphasia.

Background: Apraxia of speech (AOS) is a core feature of nonfluent/agrammatic primary progressive aphasia (naPPA), but its precise characteristics and the prevalence of AOS features in spontaneous speech are debated. Objective: To assess the frequency of features of AOS in the spontaneous, connected speech of individuals with naPPA and to evaluate whether these features are associated with an underlying motor disorder such as corticobasal syndrome or progressive supranuclear palsy. Methods: We examined features of AOS in 30 patients with naPPA using a picture description task. We compared these patients to 22 individuals with behavioral variant frontotemporal dementia and 30 healthy controls. Each speech sample was evaluated perceptually for lengthened speech segments and quantitatively for speech sound distortions, pauses between and within words, and articulatory groping. We compared subgroups of naPPA with and without at least two features of AOS to assess the possible contribution of a motor impairment to speech production deficits. Results: naPPA patients produced both speech sound distortions and other speech sound errors. Speech segmentation was found in 27/30 (90%) of individuals. Distortions were identified in 8/30 (27%) of individuals, and other speech sound errors occurred in 18/30 (60%) of individuals. Frequent articulatory groping was observed in 6/30 (20%) of individuals. Lengthened segments were observed rarely. There were no differences in the frequencies of AOS features among naPPA subgroups as a function of extrapyramidal disease. Conclusion: Features of AOS occur with varying frequency in the spontaneous speech of individuals with naPPA, independently of an underlying motor disorder.

Echolalia in patients with primary progressive aphasia.

OBJECTIVE: This study aimed to examine echolalia and its related symptoms and brain lesions in primary progressive aphasia (PPA). METHODS: Forty-five patients with PPA were included: 19 nonfluent/agrammatic variant PPA (nfvPPA), 5 semantic variant PPA, 7 logopenic variant PPA, and 14 unclassified PPA patients. We detected echolalia in unstructured conversations. An evaluation of language function and the presence of parkinsonism, grasp reflex, imitation behaviour, and disinhibition were assessed. We also measured regional cerebral blood flow (rCBF) using single-photon emission computed tomography. RESULTS: Echolalia was observed in 12 nfvPPA and 2 unclassified PPA patients. All patients showed mitigated echolalia. We compared nfvPPA patients with echolalia (echolalia group) to those without echolalia (non-echolalia group). The median age of the echolalia group was significantly lower than that of the non-echolalia group, and the echolalia group showed a significantly worse auditory comprehension performance than the non-echolalia group. In contrast, the performance of repetition tasks was not different between the two groups. The prevalence of imitation behaviour in the echolalia group was significantly higher than that in the non-echolalia group. The rCBFs in the bilateral pre-supplementary motor area and bilateral middle cingulate cortex in the echolalia group were significantly lower than those in the non-echolalia group. CONCLUSIONS: These findings suggest that echolalia is characteristic of nfvPPA patients with impaired comprehension. Reduced inhibition of the medial frontal cortex with release activity of the anterior perisylvian area account for the emergence of echolalia.

18F-THK5351 PET Imaging in Nonfluent-Agrammatic Variant Primary Progressive Aphasia.

BACKGROUND AND PURPOSE: To analyze 18F-THK5351 positron emission tomography (PET) scans of patients with clinically diagnosed nonfluent/agrammatic variant primary progressive aphasia (navPPA). METHODS: Thirty-one participants, including those with Alzheimer's disease (AD, n=13), navPPA (n=3), and those with normal control (NC, n=15) who completed 3 Tesla magnetic resonance imaging, 18F-THK5351 PET scans, and detailed neuropsychological tests, were included. Voxel-based and region of interest (ROI)-based analyses were performed to evaluate retention of 18F-THK5351 in navPPA patients. RESULTS: In ROI-based analysis, patients with navPPA had higher levels of THK retention in the Broca's area, bilateral inferior frontal lobes, bilateral precentral gyri, and bilateral basal ganglia. Patients with navPPA showed higher levels of THK retention in bilateral frontal lobes (mainly left side) compared than NC in voxel-wise analysis. CONCLUSIONS: In our study, THK retention in navPPA patients was mainly distributed at the frontal region which was well correlated with functional-radiological distribution of navPPA. Our results suggest that tau PET imaging could be a supportive tool for diagnosis of navPPA in combination with a clinical history.

¹⁸F-THK5351 PET Imaging in Nonfluent-Agrammatic Variant Primary Progressive Aphasia

BACKGROUND AND PURPOSE: To analyze 18F-THK5351 positron emission tomography (PET) scans of patients with clinically diagnosed nonfluent/agrammatic variant primary progressive aphasia (navPPA). METHODS: Thirty-one participants, including those with Alzheimer's disease (AD, n=13), navPPA (n=3), and those with normal control (NC, n=15) who completed 3 Tesla magnetic resonance imaging, 18F-THK5351 PET scans, and detailed neuropsychological tests, were included. Voxel-based and region of interest (ROI)-based analyses were performed to evaluate retention of 18F-THK5351 in navPPA patients. RESULTS: In ROI-based analysis, patients with navPPA had higher levels of THK retention in the Broca's area, bilateral inferior frontal lobes, bilateral precentral gyri, and bilateral basal ganglia. Patients with navPPA showed higher levels of THK retention in bilateral frontal lobes (mainly left side) compared than NC in voxel-wise analysis. CONCLUSIONS: In our study, THK retention in navPPA patients was mainly distributed at the frontal region which was well correlated with functional-radiological distribution of navPPA. Our results suggest that tau PET imaging could be a supportive tool for diagnosis of navPPA in combination with a clinical history.

Characterisation of "Positive" Behaviours in Primary Progressive Aphasias.

BACKGROUND/AIMS: Although some patients with primary progressive aphasia (PPA) exhibit novel or improved skills after the onset of dementia, these changes have yet to be quantified. Therefore, this study systematically explored and identified the emergence of positive behaviours after dementia onset. METHODS: This study included 48 carers of patients with PPA: 12 nonfluent/agrammatic PPA (nfvPPA), 22 semantic variant PPA (svPPA), and 14 logopenic variant PPA (lvPPA). The presence and frequency of positive behaviour changes after dementia onset were established using the Hypersensory and Social/Emotional Scale (HSS). RESULTS: Scores on Sensitivity to Details, Visuospatial Activities, and Music Activities differed significantly among the groups. More specifically, svPPA was associated with increased visuospatial activity, but only in the mild stage of the disease; nfvPPA was associated with increased visuospatial activity and decreased music activity, while lvPPA exhibited the reverse profile. CONCLUSIONS: The results demonstrate that subsets of PPA patients show novel or increased positive behaviours following dementia onset, and differences among subtypes may be helpful for improving diagnostic accuracy. Additionally, harnessing these skills may improve the quality of life of both patients and carers.

Prevalence of frontotemporal dementia in community-based studies in Latin America: a systematic review / Prevalência de demência frontotemporal em estudos de base comunitária na America Latina: uma revisão sistemática

Latin America (LA) is experiencing a rise in the elderly population and a consequent increase in geriatric problemssuch as dementia. There are few epidemiological studies assessing the magnitude of dementia and dementia subtypesin LA. Objective: To identify published community-based studies on the prevalence of FTD in LA countries. Methods: Adatabase search for door-to-door studies on FTD prevalence in LA was performed. The search was carried out on Medline,Embase, and LILACS databases for research conducted between 1994 and 2012. The main inclusion criteria were: use ofany internationally accepted diagnostic criteria and investigation of community samples. Results: Four hundred and ninetytwo articles were found, of which 26 were initially pre-selected by title or abstract review. Five studies from 3 differentcountries were included. The FTD prevalence rates in community-dwelling elderly were 1.2 (Venezuela), 1.3 (Peru) and1.7-1.8 (Brazil) per thousand persons, depending on age group. Conclusion: The FTD prevalence in LA studies showedvalues mid-way between those observed in western and in oriental populations. Despite the magnitude of this problem,epidemiological information on FTD remains scarce in LA.

A América Latina (AL) está experimentando um aumento na população de idosos e um consequente aumento nosproblemas geriátricos, como demência. Existem poucos estudos epidemiológicos avaliando a magnitude de demência edemência subtipos na AL Objetivo: Identificar publicações baseadas em estudos sobre a prevalência da FTD em países daAL. Métodos: A pesquisa realizada foi por estudos de prevalência de FTD em comunidade na AL. A pesquisa foi realizada emMed-line, Embase, e LILACS no período entre 1994 e 2012. Os principais critérios de inclusão foram: utilização de quaisquercritérios internacionalmente aceitos de diagnóstico e investigação de amostras em comunidade. Resultados: Quatrocentose noventa e dois artigos foram encontrados, dos quais 26 foram inicialmente pré-selecionados pelo título ou fiscalização doabstract. Cinco estudos de 3 países diferentes foram incluídos. As taxas de prevalência na comunidade em idosos com FTDeram 1,2 (Venezuela), 1,3 (Peru) e 1,7-1,8 (Brasil) por mil pessoas, dependendo da faixa etária. Conclusão: A prevalênciaFTD em estudos da AL, apresentaram valores intermediários entre os observados em populações ocidentais e orientais. Apesarda magnitude do problema, informações epidemiológicas sobre FTD permanecem escassas em AL.

Prevalence of frontotemporal dementia in community-based studies in Latin America: a systematic review.

Latin America (LA) is experiencing a rise in the elderly population and a consequent increase in geriatric problems such as dementia. There are few epidemiological studies assessing the magnitude of dementia and dementia subtypes in LA. OBJECTIVE: To identify published community-based studies on the prevalence of FTD in LA countries. METHODS: A database search for door-to-door studies on FTD prevalence in LA was performed. The search was carried out on Medline, Embase, and LILACS databases for research conducted between 1994 and 2012. The main inclusion criteria were: use of any internationally accepted diagnostic criteria and investigation of community samples. RESULTS: Four hundred and ninety two articles were found, of which 26 were initially pre-selected by title or abstract review. Five studies from 3 different countries were included. The FTD prevalence rates in community-dwelling elderly were 1.2 (Venezuela), 1.3 (Peru) and 1.7-1.8 (Brazil) per thousand persons, depending on age group. CONCLUSION: The FTD prevalence in LA studies showed values mid-way between those observed in western and in oriental populations. Despite the magnitude of this problem, epidemiological information on FTD remains scarce in LA.

A América Latina (AL) está experimentando um aumento na população de idosos e um consequente aumento nos problemas geriátricos, como demência. Existem poucos estudos epidemiológicos avaliando a magnitude de demência e demência subtipos na AL. OBJETIVO: Identificar publicações baseadas em estudos sobre a prevalência da FTD em países da AL. MÉTODOS: A pesquisa realizada foi por estudos de prevalência de FTD em comunidade na AL. A pesquisa foi realizada em Med-line, Embase, e LILACS no período entre 1994 e 2012. Os principais critérios de inclusão foram: utilização de quaisquer critérios internacionalmente aceitos de diagnóstico e investigação de amostras em comunidade. RESULTADOS: Quatrocentos e noventa e dois artigos foram encontrados, dos quais 26 foram inicialmente pré-selecionados pelo título ou fiscalização do abstract. Cinco estudos de 3 países diferentes foram incluídos. As taxas de prevalência na comunidade em idosos com FTD eram 1,2 (Venezuela), 1,3 (Peru) e 1,7-1,8 (Brasil) por mil pessoas, dependendo da faixa etária. CONCLUSÃO: A prevalência FTD em estudos da AL, apresentaram valores intermediários entre os observados em populações ocidentais e orientais. Apesar da magnitude do problema, informações epidemiológicas sobre FTD permanecem escassas em AL.