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[PMID]:28452903
[Au] Autor:Maschio M; Dinapoli L; Zarabla A; Maialetti A; Giannarelli D; Fabi A; Vidiri A; Cantelmi T
[Ad] Endereço:*Center for Tumor-Related Epilepsy, UOSD Neurology, †Biostatistic Unit, Departments of ‡Oncology, §Radiology, and ∥Service of Psychiatry, Regina Elena National Cancer Institute, Rome, Italy.
[Ti] Título:Zonisamide in Brain Tumor-Related Epilepsy: An Observational Pilot Study.
[So] Source:Clin Neuropharmacol;40(3):113-119, 2017 May/Jun.
[Is] ISSN:1537-162X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: Epilepsy heavily affects the quality of life (QoL) of patients with brain tumor because in addition to taking treatments for the oncological illness, patients are required to live with the long-term taking of antiepileptic drugs (AEDs). The AEDs' adverse effects are common in these patients and can negatively influence their perceptions of their QoL.We conducted an observational pilot study in patients with brain tumor-related epilepsy to verify efficacy, tolerability, and impact on QoL and global neurocognitive performances of zonisamide (ZNS) in add-on. MATERIALS AND METHODS: We recruited 13 patients (5 females, 8 males; mean age, 49.6 years) presenting uncontrolled seizures. At first visit and at final follow-up at 6 months, patients underwent neurological examination, evaluation of adverse events, and cognitive and QoL tests. A seizure diary was given. RESULTS: Eight patients underwent chemotherapy, 3 underwent radiotherapy, and 5 had disease progression. Mean dosage of ZNS at final follow-up was 300 mg/d.Of 9 patients who reached the sixth month follow-up, the mean weekly seizure number before ZNS had been 3.2 ± 5.0, and at final follow-up, the mean weekly seizure number was 0.18 ± 0.41 (P = 0.05).Compared with baseline, we observed stability in all cognitive domains, except for verbal fluency that significantly worsened.Results on QoL tests showed that QoL remained unchanged over time, which could indicate that ZNS did not influence the patients' perceived QoL. CONCLUSIONS: Zonisamide as add-on in our patients seems to be well tolerated and efficacious in controlling seizures. Despite having limitations represented by the fact that our study is observational, with a small study population and a short follow-up period, our results confirm that when choosing an AED, in addition to efficacy, the drug's effect on patients' QoL also needs to be considered, especially for patients facing many psychosocial challenges, such as those with brain tumor-related epilepsy.
[Mh] Termos MeSH primário: Anticonvulsivantes/uso terapêutico
Neoplasias Encefálicas/fisiopatologia
Disfunção Cognitiva/prevenção & controle
Epilepsia/tratamento farmacológico
Isoxazóis/uso terapêutico
Nootrópicos/uso terapêutico
Qualidade de Vida
[Mh] Termos MeSH secundário: Antineoplásicos/efeitos adversos
Antineoplásicos/uso terapêutico
Neoplasias Encefálicas/tratamento farmacológico
Neoplasias Encefálicas/radioterapia
Disfunção Cognitiva/induzido quimicamente
Disfunção Cognitiva/etiologia
Disfunção Cognitiva/fisiopatologia
Terapia Combinada/efeitos adversos
Resistência a Medicamentos
Quimioterapia Combinada/efeitos adversos
Epilepsia/induzido quimicamente
Epilepsia/etiologia
Epilepsia/fisiopatologia
Feminino
Seguimentos
Seres Humanos
Itália
Transtornos da Linguagem/induzido quimicamente
Transtornos da Linguagem/etiologia
Transtornos da Linguagem/fisiopatologia
Transtornos da Linguagem/prevenção & controle
Masculino
Meia-Idade
Projetos Piloto
Radioterapia/efeitos adversos
Índice de Gravidade de Doença
Comportamento Verbal/efeitos dos fármacos
[Pt] Tipo de publicação:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Nm] Nome de substância:
0 (Anticonvulsants); 0 (Antineoplastic Agents); 0 (Isoxazoles); 0 (Nootropic Agents); 459384H98V (zonisamide)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180306
[Lr] Data última revisão:
180306
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE
[do] DOI:10.1097/WNF.0000000000000218


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[PMID]:29173724
[Au] Autor:Shulman BB
[Ad] Endereço:Office of the Dean, School of Health and Medical Sciences, Seton Hall University, 400 South Orange Avenue, NJ 07079, USA. Electronic address: brian.shulman@shu.edu.
[Ti] Título:Pediatric Speech and Language Perspectives on Interprofessional Practice.
[So] Source:Pediatr Clin North Am;65(1):xvii-xix, 2018 02.
[Is] ISSN:1557-8240
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Audiologia
Relações Interprofissionais
Transtornos da Linguagem
Pediatria
Patologia da Fala e Linguagem
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Gastroenterologia
Seres Humanos
Lactente
Transtornos da Linguagem/diagnóstico
Transtornos da Linguagem/etiologia
Transtornos da Linguagem/terapia
Terapia da Linguagem
Fonoterapia
[Pt] Tipo de publicação:INTRODUCTORY JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171219
[Lr] Data última revisão:
171219
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


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[PMID]:29177494
[Au] Autor:Geranmayeh F; Chau TW; Wise RJS; Leech R; Hampshire A
[Ad] Endereço:Computational Cognitive and Clinical Neuroimaging Laboratory, Imperial College, Hammersmith Hospital Campus, Du Cane Road, London, W12 0NN, UK.
[Ti] Título:Domain-general subregions of the medial prefrontal cortex contribute to recovery of language after stroke.
[So] Source:Brain;140(7):1947-1958, 2017 Jul 01.
[Is] ISSN:1460-2156
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:We hypothesized that the recovery of speech production after left hemisphere stroke not only depends on the integrity of language-specialized brain systems, but also on 'domain-general' brain systems that have much broader functional roles. The presupplementary motor area/dorsal anterior cingulate forms part of the cingular-opercular network, which has a broad role in cognition and learning. Consequently, we have previously suggested that variability in the recovery of speech production after aphasic stroke may relate in part to differences in patients' abilities to engage this domain-general brain region. To test our hypothesis, 27 patients (aged 59 ± 11 years) with a left hemisphere stroke performed behavioural assessments and event-related functional magnetic resonance imaging tasks at two time points; first in the early phase (∼2 weeks) and then ∼4 months after the ictus. The functional magnetic resonance imaging tasks were designed to differentiate between activation related to language production (sentential overt speech production-Speech task) and activation related to cognitive processing (non-verbal decision making). Simple rest and counting conditions were also included in the design. Task-evoked regional brain activations during the early and late phases were compared with a longitudinal measure of recovery of language production. In accordance with a role in cognitive processing, substantial activity was observed within the presupplementary motor area/dorsal anterior cingulate during the decision-making task. Critically, the level of activation within this region during speech production correlated positively with the longitudinal recovery of speech production across the two time points (as measured by the in-scanner performance in the Speech task). This relationship was observed for activation in both the early phase (r = 0.363, P = 0.03 one-tailed) and the late phase (r = 0.538, P = 0.004). Furthermore, presupplementary motor area/dorsal anterior cingulate activity was a predictor of both language recovery over time and language outcome at ∼4 months, over and above that predicted by lesion volume, age and the initial language impairment (general linear model overall significant at P < 0.0001; ExpB 1.01, P = 0.02). The particularly prominent relationship of the presupplementary motor area/dorsal anterior cingulate region with recovery of language was confirmed in voxel-wise correlation analysis, conducted unconstrained for the whole brain volume. These results accord with the hypothesis that the functionality of the presupplementary motor area/dorsal anterior cingulate contributes to language recovery after stroke. Given that this brain region is often spared in aphasic stroke, we propose that it is a sensible target for future research into rehabilitative treatments. More broadly, baseline assessment of domain-general systems could help provide a better prediction of language recovery.
[Mh] Termos MeSH primário: Transtornos da Linguagem/etiologia
Córtex Pré-Frontal/fisiopatologia
Recuperação de Função Fisiológica/fisiologia
Acidente Vascular Cerebral/complicações
[Mh] Termos MeSH secundário: Idoso
Estudos de Casos e Controles
Feminino
Seres Humanos
Processamento de Imagem Assistida por Computador
Modelos Lineares
Imagem por Ressonância Magnética
Masculino
Meia-Idade
Testes Neuropsicológicos
Oxigênio/sangue
Córtex Pré-Frontal/diagnóstico por imagem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
S88TT14065 (Oxygen)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171130
[Lr] Data última revisão:
171130
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE
[do] DOI:10.1093/brain/awx134


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[PMID]:29173723
[Au] Autor:Stanton BF
[Ad] Endereço:Seton Hall-Hackensack Meridian School of Medicine University, 400 South Orange Avenue, South Orange, NJ 07079, USA. Electronic address: bonita.stanton@shu.edu.
[Ti] Título:Pediatric Speech and Language: Perspectives on Interprofessional Practice.
[So] Source:Pediatr Clin North Am;65(1):xv-xvi, 2018 02.
[Is] ISSN:1557-8240
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Audiologia
Relações Interprofissionais
Transtornos da Linguagem
Pediatria
Patologia da Fala e Linguagem
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Seres Humanos
Transtornos da Linguagem/diagnóstico
Transtornos da Linguagem/etiologia
Transtornos da Linguagem/terapia
Terapia da Linguagem
Fonoterapia
[Pt] Tipo de publicação:INTRODUCTORY JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


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[PMID]:29173721
[Au] Autor:Liu XL; Zahrt DM; Simms MD
[Ad] Endereço:Bethel Hearing-Speaking Training Center, 7801 South Stemmons Freeway, Corinth, TX 76210, USA.
[Ti] Título:An Interprofessional Team Approach to the Differential Diagnosis of Children with Language Disorders.
[So] Source:Pediatr Clin North Am;65(1):73-90, 2018 Feb.
[Is] ISSN:1557-8240
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The ability to communicate effectively with others is central to children's development. Delays or disruptions due to isolated expressive language delay, articulation errors, multiple sound production errors with motor planning deficits, or mixed expressive and receptive language delay, often bring widespread consequences. Physical anomalies, neurologic and genetic disorder, cognitive and intellectual disabilities, and emotional disturbances may affect speech and language development. Communication disorders may be misdiagnosed as intellectual impairment or autism. Interdisciplinary evaluation should include speech and language assessment, physical and neurologic status, cognitive and emotional profile, and family and social history. This article describes assessment and reviews common pediatric communication disorders.
[Mh] Termos MeSH primário: Relações Interprofissionais
Transtornos da Linguagem/diagnóstico
Equipe de Assistência ao Paciente
Pediatria
Psicologia da Criança
Patologia da Fala e Linguagem
[Mh] Termos MeSH secundário: Audiologia
Criança
Pré-Escolar
Diagnóstico Diferencial
Seres Humanos
Transtornos da Linguagem/etiologia
Transtornos da Linguagem/psicologia
Testes de Linguagem
Terapia Ocupacional
Fisioterapia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


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[PMID]:29173710
[Au] Autor:Golom FD; Schreck JS
[Ad] Endereço:Department of Psychology, Loyola University Maryland, 4501 North Charles Street, Baltimore, MD 21210, USA.
[Ti] Título:The Journey to Interprofessional Collaborative Practice: Are We There Yet?
[So] Source:Pediatr Clin North Am;65(1):1-12, 2018 Feb.
[Is] ISSN:1557-8240
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Interprofessional collaborative practice (IPCP) is a service delivery approach that seeks to improve health care outcomes and the patient experience while simultaneously decreasing health care costs. The current article reviews the core competencies and current trends associated with IPCP, including challenges faced by health care practitioners when working on interprofessional teams. Several conceptual frameworks and empirically supported interventions from the fields of organizational psychology and organization development are presented to assist health care professionals in transitioning their teams to a more interprofessionally collaborative, team-based model of practice.
[Mh] Termos MeSH primário: Audiologia/organização & administração
Relações Interprofissionais
Transtornos da Linguagem
Equipe de Assistência ao Paciente/organização & administração
Pediatria/organização & administração
Patologia da Fala e Linguagem/organização & administração
[Mh] Termos MeSH secundário: Competência Clínica
Comunicação
Comportamento Cooperativo
Seres Humanos
Transtornos da Linguagem/diagnóstico
Transtornos da Linguagem/terapia
Terapia da Linguagem/organização & administração
Fonoterapia/organização & administração
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171129
[Lr] Data última revisão:
171129
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171128
[St] Status:MEDLINE


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[PMID]:28692932
[Au] Autor:Lei H; Yan Z; Sun X; Zhang Y; Wang J; Ma C; Xu Q; Wang R; Jarvis ED; Sun Z
[Ad] Endereço:Department of Neurobiology, Beijing Institute for Brain Disorders, Beijing Center of Neural Regeneration and Repair, Key Laboratory for Neurodegenerative Diseases of the Ministry of Education, Capital Medical University, Beijing 100069, China. Electronic address: leihm@ccmu.edu.cn.
[Ti] Título:Axon guidance pathways served as common targets for human speech/language evolution and related disorders.
[So] Source:Brain Lang;174:1-8, 2017 Nov.
[Is] ISSN:1090-2155
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Human and several nonhuman species share the rare ability of modifying acoustic and/or syntactic features of sounds produced, i.e. vocal learning, which is the important neurobiological and behavioral substrate of human speech/language. This convergent trait was suggested to be associated with significant genomic convergence and best manifested at the ROBO-SLIT axon guidance pathway. Here we verified the significance of such genomic convergence and assessed its functional relevance to human speech/language using human genetic variation data. In normal human populations, we found the affected amino acid sites were well fixed and accompanied with significantly more associated protein-coding SNPs in the same genes than the rest genes. Diseased individuals with speech/language disorders have significant more low frequency protein coding SNPs but they preferentially occurred outside the affected genes. Such patients' SNPs were enriched in several functional categories including two axon guidance pathways (mediated by netrin and semaphorin) that interact with ROBO-SLITs. Four of the six patients have homozygous missense SNPs on PRAME gene family, one youngest gene family in human lineage, which possibly acts upon retinoic acid receptor signaling, similarly as FOXP2, to modulate axon guidance. Taken together, we suggest the axon guidance pathways (e.g. ROBO-SLIT, PRAME gene family) served as common targets for human speech/language evolution and related disorders.
[Mh] Termos MeSH primário: Orientação de Axônios/genética
Orientação de Axônios/fisiologia
Axônios/metabolismo
Evolução Molecular
Transtornos da Linguagem/genética
Linguagem
Mutação de Sentido Incorreto/genética
Fala/fisiologia
[Mh] Termos MeSH secundário: Criança
Pré-Escolar
Fatores de Transcrição Forkhead/metabolismo
Seres Humanos
Transtornos da Linguagem/metabolismo
Aprendizagem/fisiologia
Masculino
Fatores de Crescimento Neural/metabolismo
Netrina-1
Polimorfismo de Nucleotídeo Único/genética
Receptores do Ácido Retinoico/metabolismo
Semaforinas/metabolismo
Proteínas Supressoras de Tumor/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (FOXP2 protein, human); 0 (Forkhead Transcription Factors); 0 (Nerve Growth Factors); 0 (Receptors, Retinoic Acid); 0 (Semaphorins); 0 (Tumor Suppressor Proteins); 158651-98-0 (Netrin-1)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170711
[St] Status:MEDLINE


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Porta, Gilda
Texto completo SciELO Brasil
[PMID]:28658434
[Au] Autor:de-Paula EM; Porta G; Tannuri ACA; Tannuri U; Befi-Lopes DM
[Ad] Endereço:Departamento de Fisioterapia, Fonoaudiologia e Terapia Ocupacional, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, BR.
[Ti] Título:Language assessment of children with severe liver disease in a public service in Brazil.
[So] Source:Clinics (Sao Paulo);72(6):351-357, 2017 Jun.
[Is] ISSN:1980-5322
[Cp] País de publicação:Brazil
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE:: The aim of this research was to compare language development (expressive and receptive skills) in children awaiting liver transplantation with that of children who have already undergone the surgical procedure. METHODS:: An observational, descriptive, cross-sectional study was conducted with 76 children divided into groups, as follows: 31 children who were candidates for liver transplantation (Group 1; G1), 45 children who had already undergone liver transplantation (Group 2; G2), and a control group (CG) of 60 healthy, normally developing children. Health status information was gathered, and the Test of Early Language Development (TELD)-3 was used to assess language skills. Family household monthly income data were also gathered using a specific questionnaire. RESULTS:: G1 had poorer language performance compared with G2 and the CG. G2 had lower language performance when compared with the CG. However, when considering the TELD-3 standard scores, G2 had scores within normal limits. The regression analysis indicated age as a risk factor for language deficits in Group 1 and family income as a risk factor for language deficits in G2. CONCLUSIONS:: The results suggested that children with chronic liver disease have delays in language development. Transplanted children have linguistic performance within normal limits, but their scores tended to be lower than the CG.
[Mh] Termos MeSH primário: Transtornos da Linguagem/complicações
Hepatopatias/complicações
[Mh] Termos MeSH secundário: Brasil
Estudos de Casos e Controles
Criança
Estudos Transversais
Feminino
Seres Humanos
Transtornos da Linguagem/diagnóstico
Testes de Linguagem
Hepatopatias/cirurgia
Transplante de Fígado
Masculino
Fatores de Risco
Índice de Gravidade de Doença
Fatores Socioeconômicos
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171005
[Lr] Data última revisão:
171005
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170629
[St] Status:MEDLINE


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[PMID]:28602661
[Au] Autor:Jiao Y; Lin F; Wu J; Li H; Chen X; Li Z; Ma J; Cao Y; Wang S; Zhao J
[Ad] Endereço:Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, P. R. China; China National Clinical Research Center for Neurological Diseases, Beijing, P. R. China; Center of Stroke, Beijing Institute for Brain Disorders, Beijing, P. R. China; Beijing Key Laboratory of Tr
[Ti] Título:Brain Arteriovenous Malformations Located in Language Area: Surgical Outcomes and Risk Factors for Postoperative Language Deficits.
[So] Source:World Neurosurg;105:478-491, 2017 Sep.
[Is] ISSN:1878-8769
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: Case selection for surgical treatment of language-area brain arteriovenous malformations (L-BAVMs) remains difficult. This study aimed to determine the surgical outcomes and risk factors for postoperative language deficits (LDs) in patients with L-BAVMs. METHODS: Patients with L-BAVMs who underwent microsurgical resection between September 2012 and June 2016 were reviewed. All patients had undergone preoperative functional magnetic resonance imaging and diffusion tensor imaging. Both functional and angioarchitectural factors were analyzed regarding the postoperative LD. Functional factors included the eloquence involved, the side of blood-oxygenation level-dependent signal activation and the white-matter fibers (anterior segment, long segment [LS], and posterior segment of arcuate fasciculus, and the inferior fronto-occipital fasciculus) involved. RESULTS: Sixty-nine patients with L-BAVMs were reviewed. Postoperative short- and long-term LD was found in 32 (46.4%) and 14 (20.3%) patients, respectively. Twelve of the 14 patients with Geschwind's territory L-BAVMs (85.7%) had short-term LD, compared with 10 (34.5%) in Wernicke's and 10 (38.5%) in Broca's area. LS involvement (P = 0.001) and larger nidus size (P = 0.017) were independent risk factors for the short-term LD. Meanwhile, nidus size (P = 0.007), preoperative LD (P = 0.008), and LS involvement (P = 0.028) were independent risk factors for long-term LD. CONCLUSIONS: L-BAVMs located in Geschwind's territory can cause a high incidence of LD. LS involvement and larger nidus size are risk factors for postoperative short- and long-term LD, and preoperative LD is a risk factor for postoperative, long-term LD.
[Mh] Termos MeSH primário: Fístula Arteriovenosa/diagnóstico por imagem
Fístula Arteriovenosa/cirurgia
Malformações Arteriovenosas Intracranianas/diagnóstico por imagem
Malformações Arteriovenosas Intracranianas/cirurgia
Transtornos da Linguagem/diagnóstico por imagem
Complicações Pós-Operatórias/diagnóstico por imagem
[Mh] Termos MeSH secundário: Adolescente
Adulto
Fístula Arteriovenosa/fisiopatologia
Criança
Feminino
Seres Humanos
Malformações Arteriovenosas Intracranianas/fisiopatologia
Linguagem
Transtornos da Linguagem/fisiopatologia
Masculino
Microcirurgia/efeitos adversos
Meia-Idade
Complicações Pós-Operatórias/fisiopatologia
Estudos Prospectivos
Estudos Retrospectivos
Fatores de Risco
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RANDOMIZED CONTROLLED TRIAL
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171003
[Lr] Data última revisão:
171003
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170613
[St] Status:MEDLINE


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[PMID]:28596248
[Au] Autor:Saxon JA; Thompson JC; Jones M; Harris JM; Richardson AM; Langheinrich T; Neary D; Mann DM; Snowden JS
[Ad] Endereço:Manchester Academic Health Sciences Centre, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK.
[Ti] Título:Examining the language and behavioural profile in FTD and ALS-FTD.
[So] Source:J Neurol Neurosurg Psychiatry;88(8):675-680, 2017 Aug.
[Is] ISSN:1468-330X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: A proportion of patients with behavioural variant frontotemporal dementia (bvFTD) develop amyotrophic lateral sclerosis (ALS). It is currently unknown whether the behavioural and cognitive syndrome in bvFTD with ALS (ALS-FTD) is indistinguishable from that of bvFTD alone. METHODS: A retrospective cohort of 241 patients with clinical diagnoses of bvFTD (n=185) or ALS-FTD (n=56) was examined with respect to behavioural, cognitive and neuropsychiatric symptoms. Features were rated as present or absent based on information recorded from clinical interviews and detailed neuropsychological assessment. RESULTS: A number of behavioural and affective changes were reported more frequently in bvFTD than ALS-FTD: social disinhibition (p<0.001), inertia (p<0.001), loss of sympathy and empathy (p 0.008), repetitive behaviours (p<0.001) and dietary changes (p<0.001). Warmth of affect demonstrated in the clinic setting was reported more often in ALS-FTD than bvFTD (p<0.001). Executive impairments occurred equally in both groups. Language impairments were more common in ALS-FTD than bvFTD: agrammatism (p<0.017) and impaired sentence comprehension (p<0.036). Psychotic features were relatively rare and did not distinguish the groups. CONCLUSIONS: Our findings suggest differences between bvFTD and ALS-FTD. In particular, while changes in social behaviour are prominent in bvFTD alone, there may be a comparatively greater degree of language impairment in ALS-FTD. Prospective exploration of the pattern of differences between these groups will be essential. Identification of a distinct neuropsychological phenotype in ALS-FTD may have clinical implications for early diagnosis, disease management and care planning and theoretical implications for our understanding of the relationship between ALS and FTD.
[Mh] Termos MeSH primário: Esclerose Amiotrófica Lateral/diagnóstico
Demência Frontotemporal/diagnóstico
Transtornos da Linguagem/diagnóstico
Transtornos do Comportamento Social/diagnóstico
[Mh] Termos MeSH secundário: Idoso
Afasia de Broca/diagnóstico
Transtornos da Percepção Auditiva/diagnóstico
Estudos de Coortes
Diagnóstico Diferencial
Progressão da Doença
Diagnóstico Precoce
Feminino
Seres Humanos
Masculino
Meia-Idade
Transtornos do Humor/diagnóstico
Testes Neuropsicológicos
Estudos Retrospectivos
[Pt] Tipo de publicação:COMPARATIVE STUDY; JOURNAL ARTICLE
[Em] Mês de entrada:1708
[Cu] Atualização por classe:171114
[Lr] Data última revisão:
171114
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170610
[St] Status:MEDLINE
[do] DOI:10.1136/jnnp-2017-315667



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