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BACKGROUND: Poststroke depressive symptoms have prospectively predicted impairment of health-related quality of life (HRQOL). However, it is not known whether such predictive effect is independent of HRQOL at 1 month after stroke. This study aimed to investigate the impact of depressive symptoms at 1 and 3 months after stroke on the 3-month poststroke HRQOL and to investigate the influence of the HRQOL measured at 1 month after stroke on these relationships. METHODS: We prospectively evaluated 67 patients at 1 and 3 months after a first-ever ischemic stroke from 106 eligible patients who have been consecutively admitted to the neurology ward of a teaching hospital. A psychiatrist assessed the presence of depressive symptoms using the 31-item version of the Hamilton Rating Scale for Depression and the HRQOL was assessed with the 36-item Short-Form Health Survey from the Medical Outcomes Study. We used linear regression to measure the impact of depressive symptoms, HRQOL at 1 month, and potential confounders on HRQOL at 3 months. RESULTS: We found an association between depressive symptoms at 1 month and HRQOL at 3 months after the stroke; however, this association was not significant when adjusting for the 1 month poststroke HRQOL. Depressive symptoms at 3 months were associated with HRQOL at 3 months after stroke, independently of the poststroke HRQOL at 1 month and potential confounders. CONCLUSIONS: Current depressive symptoms at 3 months are important for HRQOL at 3 months after stroke; however, regarding the prospective prediction, HRQOL at 1 month is the most relevant factor.
Assuntos
Depressão/psicologia , Acidente Vascular Cerebral/psicologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/complicações , Isquemia Encefálica/psicologia , Depressão/etiologia , Feminino , Humanos , Masculino , Estado Civil , Pessoa de Meia-Idade , Testes Neuropsicológicos , Estudos Prospectivos , Qualidade de Vida , Fatores Socioeconômicos , Acidente Vascular Cerebral/complicações , Inquéritos e Questionários , Adulto JovemRESUMO
The relationship between severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and neurodegenerative diseases is yet to be fully clarified. Rapid worsening and even new-onset cases of those disorders have been reported in association with coronavirus disease 2019 (COVID-19). We describe three cases of neurodegenerative diseases in patients with SARS-CoV-2: a case of Creutzfeldt-Jakob disease during the COVID-19 acute phase, to our knowledge, is the second one described in the literature; a rapidly progressive Alzheimer's Disease; and a patient with frontotemporal dementia, and a quick decline of both cognitive and behavioral domains. This report suggests an association between SARS-CoV-2 infection and a higher probability of developing or accelerating neurodegenerative chronic neurologic conditions. We reinforce the need for a close cognitive follow-up in the aftermath of Sars-Cov2 infection.
RESUMO
Early after stroke, there is loss of intracortical facilitation (ICF) and increase in short-interval intracortical inhibition (SICI) in the primary motor cortex (M1) contralateral to a cerebellar infarct. Our goal was to investigate intracortical M1 function in the chronic stage following cerebellar infarcts (>4 months). We measured resting motor threshold (rMT), SICI, ICF, and ratios between motor-evoked potential amplitudes (MEP) and supramaximal M response amplitudes (MEP/M; %), after transcranial magnetic stimulation was applied to the M1 contralateral (M1(contralesional)) and ipsilateral (M1(ipsilesional)) to the cerebellar infarct in patients and to both M1s of healthy age-matched volunteers. SICI was decreased in M1(contralesional) compared to M1(ipsilesional) in the patient group in the absence of side-to-side differences in controls. There were no significant interhemispheric or between-group differences in rMT, ICF, or MEP/M (%). Our results document disinhibition of M1(contralesional) in the chronic phase after cerebellar stroke.
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Infarto Encefálico/fisiopatologia , Doenças Cerebelares/fisiopatologia , Lateralidade Funcional/fisiologia , Córtex Motor/fisiopatologia , Acidente Vascular Cerebral/fisiopatologia , Adulto , Potencial Evocado Motor , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estimulação Magnética Transcraniana , Adulto JovemRESUMO
OBJECTIVE: To present the development of an adapted version of the Boston Naming Test for Portuguese speakers, and to investigate the effects of age, education and gender on both the original and the adapted Boston Naming Test in respect of Brazilian Portuguese speakers. METHOD: Eighty items, including the 60 original ones and 20 adapted items were administered to 739 healthy Brazilian subjects aged between 6 and 77 years who received 0 to 17 years of education. RESULTS: The coefficients of the General Linear Model estimation suggested that both age and education were statistically significant to predict total scores. In addition, score variances, justified by such predictors, were 41.20% in the original Boston Naming Test against 25.84% in the adapted Boston Naming Test. These results suggest that the scores from the original BNT are more dependent on age and education than those from the adapted Boston Naming Test. CONCLUSION: These findings demonstrated the suitability of the adapted Boston Naming Test version for the Brazilian population and described provisional norms for the original and adapted Boston Naming Test for Portuguese speakers.
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Testes de Linguagem , Testes Neuropsicológicos , Adolescente , Adulto , Fatores Etários , Idoso , Brasil , Criança , Características Culturais , Escolaridade , Feminino , Humanos , Idioma , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Fatores Sexuais , Tradução , Adulto JovemRESUMO
In this study, the effectiveness of a group-based attention and problem solving (APS) treatment approach to executive impairments in patients with frontal lobe lesions was investigated. Thirty participants with lesions in the frontal lobes, 16 with left frontal (LF) and 14 with right frontal (RF) lesions, were allocated into three groups, each with 10 participants. The APS treatment was initially compared to two other control conditions, an information/education (IE) approach and treatment-as-usual or traditional rehabilitation (TR), with each of the control groups subsequently receiving the APS intervention in a crossover design. This design allowed for an evaluation of the treatment through assessment before and after treatment and on follow up, six months later. There was an improvement on some executive and functional measures after the implementation of the APS programme in the three groups. Size, and to a lesser extent laterality, of lesion affected baseline performance on measures of executive function, but there was no apparent relationship between size, laterality or site of lesion and level of benefit from the treatment intervention. The results were discussed in terms of models of executive functioning and the effectiveness of domain specific interventions in the rehabilitation of executive dysfunction.
Assuntos
Atenção , Transtornos Cognitivos/reabilitação , Função Executiva , Resolução de Problemas , Adulto , Lesões Encefálicas/reabilitação , Estudos Cross-Over , Feminino , Seguimentos , Lobo Frontal/lesões , Lateralidade Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Modelos Psicológicos , Testes Neuropsicológicos , Inquéritos e Questionários , Resultado do TratamentoRESUMO
INTRODUCTION: Purposes of this study were: evaluate complications and length of stay of patients admitted with diagnosis of ischemic stroke (IS) in the acute or subacute phase, in a general Neurology ward in São paulo, Brazil; investigate the influence of age, risk factors for vascular disease, arterial territory and etiology. METHODS: Data from 191 IS patients were collected prospectively. RESULTS: Fifty-one patients (26.7%) presented at least one clinical complication during stay. pneumonia was the most frequent complication. Mean length of stay was 16.8+-13.8 days. Multivariate analysis revealed a correlation between younger age and lower complication rates (OR=0.92-0.97, p < 0.001). presence of complications was the only factor that independently influenced length of stay (OR=4.20; CI=1.928.84; p<0.0001). CONCLUSION: These results should be considered in the planning and organization of IS care in Brazil.
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Tempo de Internação/estatística & dados numéricos , Acidente Vascular Cerebral/etiologia , Adulto , Análise de Variância , Brasil/epidemiologia , Feminino , Unidades Hospitalares/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia/epidemiologia , Pneumonia/etiologia , Estudos Prospectivos , Acidente Vascular Cerebral/patologiaRESUMO
OBJECTIVE: To describe characteristics and provision of care for patients admitted with cerebrovascular disorders (CVD), focusing on ischemic stroke (IS), in a large, public, academic hospital in São Paulo, Brazil. METHOD: We retrieved information about 357 patients with CVD admitted to the Neurology Emergency Department (NED) and Neurology Ward (NW) of our institution. We described patient characteristics and management of IS in NED and in NW. RESULTS: IS was diagnosed in 79.6% of CVD patients admitted to NED; 2.7% were submitted to thrombolysis. Extent of IS investigation and management were significantly different in NED and NW. CONCLUSION: IS patients in our center were younger than in developed countries. IS management was significantly influenced by patient characteristics. This information can aid in planning strategies to decrease stroke burden.
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Serviços Médicos de Emergência/normas , Hospitalização/estatística & dados numéricos , Avaliação de Processos e Resultados em Cuidados de Saúde , Acidente Vascular Cerebral/tratamento farmacológico , Terapia Trombolítica , Adolescente , Adulto , Idoso , Brasil/epidemiologia , Países Desenvolvidos , Serviços Médicos de Emergência/estatística & dados numéricos , Serviço Hospitalar de Emergência , Métodos Epidemiológicos , Feminino , Hospitais Universitários , Humanos , Ataque Isquêmico Transitório/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/epidemiologia , Adulto JovemRESUMO
Somatosensory stimulation enhances aspects of motor function in patients with chronic, predominantly subcortical infarcts. We investigated the effects of somatosensory stimulation on motor function in stroke patients with predominantly cortical involvement in the middle cerebral artery territory in a double-blind, pseudorandomized crossover trial. Motor performance was evaluated with the Jebsen-Taylor test before, after 2-hour somatosensory stimulation, and after subsequent motor training (n=11). In one experimental session, patients were submitted to median nerve stimulation (MNS) and in the other session, to control stimulation (CS). The order of the sessions was counterbalanced across patients. Improvement in performance in the Jebsen-Taylor test after somatosensory stimulation and after motor training was significantly greater in the MNS session than in the CS session. Additionally, patients who received MNS in the second session maintained the beneficial effects of training 30 days later. A single MNS session improves hand motor function in patients with chronic cortico-subcortical strokes and appears to favor consolidation of training effects. Somatosensory stimulation may be an adjuvant tool for stroke rehabilitation in patients with cortical lesions.
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Córtex Cerebral/patologia , Potenciais Somatossensoriais Evocados/fisiologia , Força da Mão/fisiologia , Desempenho Psicomotor/fisiologia , Acidente Vascular Cerebral/patologia , Acidente Vascular Cerebral/fisiopatologia , Adulto , Análise de Variância , Estudos Cross-Over , Relação Dose-Resposta à Radiação , Método Duplo-Cego , Terapia por Estimulação Elétrica/métodos , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Nervo Mediano/fisiopatologia , Nervo Mediano/efeitos da radiação , Pessoa de Meia-Idade , Medição da Dor , Estimulação Física/métodos , Reabilitação do Acidente Vascular CerebralRESUMO
PURPOSE: To report the "salt and pepper"(SP) eye pain as a herald symptom of pontine ischemia. DESIGN: Observational case series. METHODS: We reviewed clinical and neuroimaging findings of four patients who presented initially with the sensation of SP in the eyes and then developed paramedian pontine infarcts confirmed by neuroimaging. RESULTS: All of the patients developed other neurologic symptoms or signs, either in association with the sensation of SP in the eyes, from hours to days later. Magnetic resonance imaging (MRI) showed paramedian pontine infarcts in all of the patients and angiography showed basilar artery occlusive disease in three of them. CONCLUSION: Impending pontine ischemia is an important differential diagnosis in patients with acute ocular pain. Prompt neurovascular evaluation and treatment may avoid devastating brainstem infarcts that cause death or long-term disability, particularly in patients with basilar artery occlusive disease.
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Arteriopatias Oclusivas/diagnóstico , Artéria Basilar/patologia , Isquemia Encefálica/diagnóstico , Oftalmopatias/diagnóstico , Dor/diagnóstico , Ponte/irrigação sanguínea , Adulto , Angiografia Digital , Infartos do Tronco Encefálico/diagnóstico , Angiografia Cerebral , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Medição da Dor , Prognóstico , Índice de Gravidade de DoençaRESUMO
White matter lesions (WML) and epilepsy have been occasionally seen in Wilson's disease. No cases of generalized myoclonus have been reported so far. We present a patient with psychiatric symptoms starting at age 16, followed by tremor, generalized dystonia and severe generalized myoclonus. In addition to classical findings, the MRI showed also extensive WML in temporal, parietal and frontal regions, preserving interhemispheric fibers. Necropsy revealed marked alterations of white matter, cortex and basal ganglia. We subsequently review the literature concerning WML and myoclonus in Wilson's disease.
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Córtex Cerebral/patologia , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/patologia , Mioclonia/complicações , Mioclonia/patologia , Adolescente , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Literatura de Revisão como AssuntoRESUMO
The persistent hypoglossal artery (PHA) is the second most common persistent embryological carotid-basilar connection and usually represents an incidental finding in cerebral arteriograms. The hypoglossal artery connects the primordial carotid artery with the longitudinal neural arteries, which later form the basilar artery. The PHA leaves the internal carotid artery as an extracranial branch, enters the skull through the anterior condyloid foramen, the hypoglossal canal and joins the caudal portion of the basilar artery. We report magnetic resonance and digital subtraction angiography findings in the first case of bilateral occipital infarctions associated with PHA and carotid atherosclerosis. The probable mechanism underlying bilateral occipital infarcts was embolism from the carotid territory to the posterior cerebral arteries. PHA may present a challenge in diagnosis and management of patients with carotid atherosclerosis and vertebrobasilar ischemia.
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Artéria Basilar/anormalidades , Artéria Carótida Interna/anormalidades , Estenose das Carótidas/diagnóstico , Infarto Cerebral/diagnóstico , Dominância Cerebral/fisiologia , Lobo Occipital/irrigação sanguínea , Artéria Vertebral/anormalidades , Idoso , Angiografia Digital , Artéria Basilar/patologia , Artéria Carótida Interna/patologia , Cerebelo/irrigação sanguínea , Cerebelo/patologia , Angiografia Cerebral , Hemianopsia/diagnóstico , Hemianopsia/etiologia , Humanos , Achados Incidentais , Infarto da Artéria Cerebral Média/diagnóstico , Infarto da Artéria Cerebral Posterior/diagnóstico , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino , Tálamo/irrigação sanguínea , Tálamo/patologia , Tomografia Computadorizada por Raios X , Ultrassonografia Doppler , Artéria Vertebral/patologiaRESUMO
PURPOSE: Data are conflicting concerning the risk for ischemic stroke associated with a common polymorphism in the gene encoding 5,10-methylenetetrahydrofolate reductase C677T, which predisposes carriers to hyperhomocysteinemia. A meta-analysis study suggested that the 5,10-methylenetetrahydrofolate reductase 677TT genotype might have a small influence in determining susceptibility to ischemic stroke. METHODS: We analyzed the 5,10-methylenetetrahydrofolate reductase 677TT genotype polymorphism in Brazilian subjects with ischemic stroke, using a case-control design. RESULTS: We compared 5,10-methylenetetrahydrofolate reductase genotypes in groups of subjects presenting ischemic stroke (n = 127) and normal control (n = 126) and found an odds ratio of 1.97 (95% CI, 0.84-4.64) in a multivariate analysis in which results were adjusted to baseline clinical characteristics of study participants. CONCLUSION: We found that the homozygous 5,10-methylenetetrahydrofolate reductase C677T genotype was not a risk factor for ischemic stroke in these Brazilian subjects.
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Isquemia Encefálica/genética , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Polimorfismo Genético/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Feminino , Marcadores Genéticos/genética , Predisposição Genética para Doença/genética , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Fatores de RiscoRESUMO
Otolith function can be evaluated by subjective visual vertical (SVV) that determine the capacity of a subject to judge if the objects are on vertical position with absence of any visual reference. The aim of this study was to evaluate the SVV in a sample of normal Brazilian subjects using a portable device. Measurements of SVV were performed in 160 normal subjects (aged from 16 to 85). SVV mean value was obtained after ten adjustments. SVV mean values ranged from -2.0 degrees to +2.4 degrees (mean=0.18 degrees, and SD=0.77). Considering all age groups, there was no difference of SVV mean values (Kruskal-Wallis test; p=0.40), but older groups had a greater variance (Levene test; p=0.016). SVV values observed in this study are comparable to those described in previous studies. Although there was no difference in mean SVV-inclination according to age, there was a greater variance in older subjects.
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Membrana dos Otólitos/fisiologia , Reflexo Vestíbulo-Ocular/fisiologia , Percepção Visual/fisiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Brasil , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valores de ReferênciaRESUMO
PURPOSE: It has been suggested that mitochondrial disease may be responsible for a substantial proportion of strokes of indetermined origin. We have preliminarily screened for MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes) mutations in young patients with cryptogenic strokes. METHOD: The mitochondrial mutations A3243G and T3271C were investigated in 38 subjects aged less than 46 years. Group 1: 15 patients with cryptogenic strokes; Group 2: 3 patients with diagnosis of MELAS syndrome, including stroke-like episodes; Group 3: 20 healthy subjects. RESULTS: The A3243G mutation was absent in all subjects in Groups 1 and 3 but was present in all subjects in Group 2. CONCLUSION: Our results do not support screening for these mutations to diagnose oligosymptomatic forms of MELAS in cryptogenic strokes in the absence of other features of the syndrome. We suggest that clinical findings should guide mitochondrial genetic testing.
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DNA Mitocondrial/genética , Síndrome MELAS/genética , Mutação/genética , Acidente Vascular Cerebral/genética , Adolescente , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Programas de Rastreamento , Linhagem , Reação em Cadeia da PolimeraseRESUMO
Basilar artery (BA) dissecting aneurysms pose difficulties to treatment because both bleeding and thrombosis can happen in the same patient, clinical course is unpredictable and high morbidity is usual. We report the case of a 37-year-old woman with a BA aneurysm probably caused by arterial dissection, presenting embolic and hemorrhagic complications. The aneurysm was submitted to endovascular treatment with stenting and coil embolization. Clinical and radiological results were excellent and no complications were observed, suggesting that BA stenting and coil embolization may be a safe and effective treatment for this condition.
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Dissecção Aórtica/terapia , Artéria Basilar , Embolização Terapêutica/métodos , Aneurisma Intracraniano/terapia , Stents , Adulto , Dissecção Aórtica/diagnóstico , Feminino , Seguimentos , Humanos , Aneurisma Intracraniano/diagnóstico , Imageamento por Ressonância Magnética , Resultado do TratamentoRESUMO
Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age ranged from 16 to 95 years old. SIRS was caused by infection in 68.8% of patients, trauma in 10.9%, burns in 7.8%, and elective surgery in 4.7%. The central nervous system involvement occurred in 56.3% of patients and was characterized as encephalopathy in 75%, seizures in 13.9%, non-epileptic myoclonus in 2.8%, and ischemic stroke in 8.3%. The magnetic resonance imaging, cerebrospinal fluid and electroencephalographic changes were unremarkable in encephalopathic patients. Neuromuscular disorders were diagnosed in 43.7%. Critical ill polyneuropathy was characterized in 57.1%, critical ill myopathy in 32.1%, demyelinating neuropathy in 7.2%, and pure motor neuropathy in 3.6%. Nerve and muscle pathological studies dismissed inflammatory abnormalities. The identification of these conditions has important economic implications and may change the critically ill patients' prognosis.
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Doenças do Sistema Nervoso/epidemiologia , Sistema Nervoso/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/epidemiologia , Isquemia Encefálica/fisiopatologia , Sistema Nervoso Central/patologia , Sistema Nervoso Central/fisiopatologia , Comorbidade , Encefalite/epidemiologia , Encefalite/fisiopatologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mioclonia/epidemiologia , Mioclonia/fisiopatologia , Sistema Nervoso/patologia , Doenças do Sistema Nervoso/fisiopatologia , Nervos Periféricos/patologia , Nervos Periféricos/fisiopatologia , Polineuropatias/epidemiologia , Polineuropatias/fisiopatologia , Polirradiculoneuropatia/epidemiologia , Polirradiculoneuropatia/fisiopatologia , Síndrome de Resposta Inflamatória Sistêmica/fisiopatologiaRESUMO
Intravenous thrombolysis is an important procedure that has significant impact on ischemic stroke prognosis. However, intracranial hemorrhage (ICH) is a feared complication of this procedure. It has been suggested that cerebral microbleeds (CMBs) may increase the risk of ICH after thrombolysis. We report on a 69 years-old woman with multiple CMBs submitted to intravenous thrombolysis without complications.
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Hemorragia Cerebral/tratamento farmacológico , Acidente Vascular Cerebral/complicações , Terapia Trombolítica/métodos , Ativador de Plasminogênio Tecidual/uso terapêutico , Idoso , Hemorragia Cerebral/etiologia , Feminino , Humanos , Infusões Intravenosas , Imageamento por Ressonância Magnética , Resultado do TratamentoRESUMO
PURPOSE: We preliminarily investigated the relevance of performing digital subtraction angiography (DSA) in addition to magnetic resonance angiography (MRA) in definition of ischemic stroke etiology in young patients. METHOD: DSAs and MRAs from 17 young patients with nonlacunar ischemic stroke were blindly analyzed and their impact on stroke management was evaluated. RESULTS: Etiologies were the same considering results of either DSA or MRA in 12/17 cases. In 15/17 patients no changes would have been made in treatment, regardless of the modality of angiography considered. CONCLUSION: These preliminary results suggest that DSA may be redundant in two thirds of ischemic strokes in young patients. Further larger prospective studies are necessary to determine indications of DSA in this age group.
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Angiografia Digital , Ataque Isquêmico Transitório/diagnóstico , Angiografia por Ressonância Magnética , Acidente Vascular Cerebral/diagnóstico , Adolescente , Adulto , Feminino , Humanos , Ataque Isquêmico Transitório/diagnóstico por imagem , Ataque Isquêmico Transitório/etiologia , Masculino , Reprodutibilidade dos Testes , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologiaRESUMO
Alteration in TP53 is the most common genetic event reported for many tumors, including astrocytomas. The majority of studies, on analyzing TP53 mutations, have not included all splice junctions. Consequently, splice site mutations are thought to be relatively infrequent. TP53 were examined for mutations by polymerase chain reaction, single strand conformation polymorphism and direct sequencing in cases of diffuse astrocytomas. We found TP53 mutations in 17.8% (8 out of 45) of the tumors tested: 3 splicing, 3 missense and 2 silent mutations. We have shown that splice site mutations of TP53 are more frequent than previously reported. These findings emphasize the importance of thorough screening of TP53 mutations in gliomas.
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Astrocitoma/genética , Neoplasias Encefálicas/genética , Genes p53 , Sítios de Splice de RNA , Análise Mutacional de DNA , Humanos , Mutação de Sentido Incorreto , Reação em Cadeia da PolimeraseRESUMO
BACKGROUND AND PURPOSE: Wilson disease (WD) is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. The literature regarding proton MR spectroscopy (MRS) in WD is scarce and controversial. The purpose of this study was to determine the brain metabolic changes due to WD by using MRS. To our knowledge, this is the first time that MRS was performed in such a large sample of patients with WD. METHODS: Thirty-six patients with WD and 37 healthy volunteers were examined with MRS in the parieto-occipital cortex, frontal white matter, and basal ganglia (BG). Ratios of the following metabolites were calculated in relation to creatine (Cr): N-acetylaspartate (NAA), choline (Cho), myo-inositol (mI), and glutamine/glutamate (Glx). The mean peak line width was measured on each spectrum. RESULTS: Compared with control subjects, patients with WD had significantly decreased NAA/Cr ratios in the three studied areas (P < .005) and an increased mI/Cr ratio in the BG (P < .001). Cho/Cr and Glx/Cr did not differ between the groups. The mean peak line in the BG was wider in patients than in control subjects. CONCLUSION: WD is unequivocally associated with MRS changes that could possibly be assigned to neuronal loss (in the three studied areas), to gliosis, and to iron and/or copper deposition in the BG.