Progression to corticobasal syndrome: a longitudinal study of patients with nonfluent primary progressive aphasia and primary progressive apraxia of speech.

BACKGROUND AND OBJECTIVES: Nonfluent variant primary progressive aphasia (nfvPPA) and primary progressive apraxia of speech (PPAOS) can be precursors to corticobasal syndrome (CBS). Details on their progression remain unclear. We aimed to examine the clinical and neuroimaging evolution of nfvPPA and PPAOS into CBS. METHODS: We conducted a retrospective longitudinal study in 140 nfvPPA or PPAOS patients and applied the consensus criteria for possible and probable CBS for every visit, evaluating limb rigidity, akinesia, limb dystonia, myoclonus, ideomotor apraxia, alien limb phenomenon, and nonverbal oral apraxia (NVOA). Given the association of NVOA with AOS, we also modified the CBS criteria by excluding NVOA and assigned every patient to either a progressors or non-progressors group. We evaluated the frequency of every CBS feature by year from disease onset, and assessed gray and white matter volume loss using SPM12. RESULTS: Asymmetric akinesia, NVOA, and limb apraxia were the most common CBS features that developed; while limb dystonia, myoclonus, and alien limb were rare. Eighty-two patients progressed to possible CBS; only four to probable CBS. nfvPPA and PPAOS had a similar proportion of progressors, although nfvPPA progressed to CBS earlier (p-value = 0.046), driven by an early appearance of limb apraxia (p-value = 0.0041). The non-progressors and progressors both showed premotor/motor cortex involvement at baseline, with spread into prefrontal cortex over time. DISCUSSION: An important proportion of patients with nfvPPA and PPAOS progress to possible CBS, while they rarely develop features of probable CBS even after long follow-up.

Speech apraxia and oral apraxia: association or dissociation? A multivariate lesion-symptom mapping study in acute stroke patients.

The anatomical relationship between speech apraxia (SA) and oral apraxia (OA) is still unclear. To shed light on this matter we studied 137 patients with acute ischaemic left-hemisphere stroke and performed support vector regression-based, multivariate lesion-symptom mapping. Thirty-three patients presented with either SA or OA. These two symptoms mostly co-occurred (n = 28), except for few patients with isolated SA (n = 2) or OA (n = 3). All patient with either SA or OA presented with aphasia (p < 0.001) and these symptoms were highly associated with apraxia (p < 0.001). Co-occurring SA and OA were predominantly associated with insular lesions, while the insula was completely spared in the five patients with isolated SA or OA. Isolated SA occurred in case of frontal lesions (prefrontal gyrus and superior longitudinal fasciculus), while isolated OA occurred in case of either temporoparietal or striatocapsular lesions. Our study supports the notion of a predominant, but not exclusive, role of the insula in verbal and non-verbal oral praxis, and indicates that frontal regions may contribute exclusively to verbal oral praxis, while temporoparietal and striatocapsular regions contribute to non-verbal oral praxis. However, since tests for SA and OA so far intrinsically also investigate aphasia and apraxia, refined tests are warranted.

Tradução e adaptação transcultural do protocolo Kaufman Speech Praxis Test for Children (KSPT) / Translation and cross-cultural adaptation of the Kaufman Speech Praxis Test for Children (KSPT) protocol

RESUMO Objetivo Apresentar o instrumento Kaufman Speech Praxis Test for Children - KSPT, bem como sua adaptação transcultural para o Brasil. Método Após a permissão de utilização do KSPT pela detentora dos direitos autorais, e cumprimento dos aspectos éticos, o instrumento foi traduzido e adaptado para o português brasileiro, com manutenção de equivalências semântica, idiomática, experimental e conceitual, quando pertinente. Resultados Após procedimentos de tradução e adaptação transcultural, não houve mudanças na Parte 1 (Nível de movimento oral); na Parte 2 (Nível silábico e fonêmico simples), 12 palavras-teste foram excluídas e 16 incluídas; e na Parte 3 (Nível silábico e fonêmico complexo), 13 palavras-teste foram excluídas por não apresentarem correspondência em relação às características de produção fonoarticulatória (fonético/fonológico) no Português Brasileiro. Na parte 4 (Extensão e complexidade espontânea - medida subjetiva) não houve alterações da versão em inglês do KSPT. Após todo o processo o material final foi aprovado pela autora e aplicado em crianças para a verificação da usabilidade e verificação de equivalências. A aplicação em população significativa no Brasil para a construção de critérios psicométricos será realizada em etapa futura. Conclusão A tradução do KSPT está finalizada com mudanças em palavras-teste do protocolo de aplicação, para respeitar as características transculturais da língua portuguesa falada no Brasil. Foram respeitadas as equivalências semântica, idiomática, experimental e conceitual, no manual de aplicação, guia de teste clínico e nos enunciados das provas. Espera-se que este instrumento integre avanços para o diagnóstico e acompanhamento de procedimentos de intervenção, trazendo uma contribuição efetiva para esta área.

ABSTRACT Purpose To present the Kaufman Speech Praxis Test for Children - KSPT instrument, as well as its Brazilian cross-cultural adaptation. Methods After obtaining permission to use the KSPT by the copyright holder, and compliance with ethical aspects, the instrument was translated and adapted into Brazilian Portuguese, preserving semantic, idiomatic, experimental and conceptual equivalences, when relevant. Results After translation and cross-cultural adaptation procedures, there were no changes in Part 1 (Oral movement); in Part 2 (Simple Phonemic and Syllabic Level), 12 test words were excluded and 16 were included; and in Part 3 (Complex Phonemic and Syllabic Level), 13 test words were excluded as they failed to correspond to the characteristics of phonoarticulatory production (phonetic/phonological) in Brazilian Portuguese. In part 4 (Spontaneous Length and Complexity - subjective measure) there were no changes to the English version of the KSPT. After the process, the final version of the material was approved by the author and applied to children for usability and equivalences check. The application to a significant population in Brazil for the construction of psychometric criteria will be carried out at a future stage. Conclusion The KSPT translation is completed with changes in test words from the application protocol to respect the cross-cultural characteristics of the Brazilian-Portuguese language. Semantic, idiomatic, experimental and conceptual equivalences were respected throughout the application manual, clinical test guide and test statements. This instrument is expected to integrate advances in the diagnosis and monitoring of intervention procedures, effectively contributing to the area.

Disrupted functional connectivity in primary progressive apraxia of speech.

Apraxia of speech is a motor speech disorder thought to result from impaired planning or programming of articulatory movements. It can be the initial or only manifestation of a degenerative disease, termed primary progressive apraxia of speech (PPAOS). The aim of this study was to use task-free functional magnetic resonance imaging (fMRI) to assess large-scale brain network pathophysiology in PPAOS. Twenty-two PPAOS participants were identified from a prospective cohort of degenerative speech and language disorders patients. All participants had a comprehensive, standardized evaluation including an evaluation by a speech-language pathologist, examination by a behavioral neurologist and a multimodal imaging protocol which included a task-free fMRI sequence. PPAOS participants were age and sex matched to amyloid-negative, cognitively normal participants with a 1:2 ratio. We chose a set of hypothesis driven, predefined intrinsic connectivity networks (ICNs) from a large, out of sample independent component analysis and then used them to initialize a spatiotemporal dual regression to estimate participant level connectivity within these ICNs. Specifically, we evaluated connectivity within the speech and language, face and hand sensorimotor, left working memory, salience, superior parietal, supramarginal, insular and deep gray ICNs in a multivariate manner. The spatial maps for each ICN were then compared between PPAOS and control participants. We used clinical measures of apraxia of speech severity to assess for clinical-connectivity correlations for regions found to differ between PPAOS and control participants. Compared to controls, PPAOS participants had reduced connectivity of the right supplementary motor area and left posterior temporal gyrus to the rest of the speech and language ICN. The connectivity of the right supplementary motor area correlated negatively with an articulatory error score. PPAOS participants also had reduced connectivity of the left supplementary motor area to the face sensorimotor ICN, between the left lateral prefrontal cortex and the salience ICN and between the left temporal-occipital junction and the left working memory ICN. The latter connectivity correlated with the apraxia of speech severity rating scale, although the finding did not survive correction for multiple comparisons. Increased connectivity was noted in PPAOS participants between the dorsal posterior cingulate and the left working memory ICN. Our results support the importance of the supplementary motor area in the pathophysiology of PPAOS, which appears to be disconnected from speech and language regions. Supplementary motor area connectivity may serve as a biomarker of degenerative apraxia of speech severity.

The Relationship Between Apraxia of Speech and Oral Apraxia: Association or Dissociation?

Acquired apraxia of speech (AOS) is a motor speech disorder that affects the implementation of articulatory gestures and the fluency and intelligibility of speech. Oral apraxia (OA) is an impairment of nonspeech volitional movement. Although many speakers with AOS also display difficulties with volitional nonspeech oral movements, the relationship between the 2 conditions is unclear. This study explored the relationship between speech and volitional nonspeech oral movement impairment in a sample of 50 participants with AOS. We examined levels of association and dissociation between speech and OA using a battery of nonspeech oromotor, speech, and auditory/aphasia tasks. There was evidence of a moderate positive association between the 2 impairments across participants. However, individual profiles revealed patterns of dissociation between the 2 in a few cases, with evidence of double dissociation of speech and oral apraxic impairment. We discuss the implications of these relationships for models of oral motor and speech control.

Brain areas impaired in oral and verbal apraxic patients.

BACKGROUND: As both oral and verbal apraxia are related to vocal orofacial musculature, this study aimed at identifying brain regions impaired in cases with oral and verbal apraxia. METHODS: In this non-experimental study, 46 left brain damaged subjects (17 females) aged 23-84 years, were examined by oral and verbal apraxia tasks. Impaired and spared Broca's area, insula, and middle frontal gyrus in the left hemisphere were checked from magnetic resonance imaging and computed tomography scans utilizing Talairach Atlas. Data were analyzed using chi-square test. RESULTS: Insula was significantly impaired in both forms of oral and verbal apraxia and different severities and prominent forms of both apraxias (P < 0.05). Broca's area was slightly less involved than insula in two forms of apraxia. CONCLUSION: As the damage of insula was more prominent in both forms of apraxias, it seems that oral and verbal apraxia may have commonalities regarding their underlying brain lesions.