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Focal atrophy of the left anterior temporal lobe has been associated with the semantic type of primary progressive aphasia evolving to semantic dementia. In contrast, focal atrophy of the right temporal lobe has more recently been described as a controverse entity reported as the right temporal variant of FTD. We describe two cases of FTD dementia syndromes: in Case 1, atrophy of the right temporal lobe led to significant behavioural impairment and difficulties in recognizing known people. In Case 2, atrophy of the left temporal lobe was associated with severe aggressive, ritualistic behaviour and aphasia.
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BACKGROUND: After recently published randomized clinical trials, the choice of the best anesthetic procedure for mechanical thrombectomy (MT) in acute ischemic stroke (AIS) due to large vessel occlusion (LVO) is not definite. OBJECTIVE: To compare the efficacy and safety of general anesthesia (GA) versus conscious sedation (CS) in patients with AIS who underwent MT, explicitly focusing on procedural and clinical outcomes and the incidence of adverse events. METHODS: PubMed, Embase, and Cochrane were systematically searched for randomized controlled trials (RCTs) comparing GA versus CS in patients who underwent MT due to LVO-AIS. Odds ratios (ORs) were calculated for binary outcomes, with 95% confidence intervals (CIs). Random effects models were used for all outcomes. Heterogeneity was assessed with I2 statistics. RESULTS: Eight RCTs (1,300 patients) were included, of whom 650 (50%) underwent GA. Recanalization success was significantly higher in the GA group (OR 1.68; 95% CI 1.26-2.24; p < 0.04) than in CS. No significant difference between groups were found for good functional recovery (OR 1.13; IC 95% 0.76-1.67; p = 0.56), incidence of pneumonia (OR 1.23; IC 95% 0.56- 2,69; p = 0.61), three-month mortality (OR 0.99; IC 95% 0.73-1.34; p = 0.95), or cerebral hemorrhage (OR 0.97; IC 95% 0.68-1.38; p = 0.88). CONCLUSION: Despite the increase in recanalization success rates in the GA group, GA and CS show similar rates of good functional recovery, three-month mortality, incidence of pneumonia, and cerebral hemorrhage in patients undergoing MT.
ANTECEDENTES: A trombectomia mecânica (TM) é o padrão de tratamento para pacientes com acidente vascular cerebral isquêmico agudo (AVCI) devido à oclusão de grandes vasos (OGV). No entanto, ainda não está claro qual é o procedimento anestésico mais benéfico para a TM. OBJETIVO: Nosso objetivo foi comparar a eficácia e a segurança da anestesia geral (AG) versus sedação consciente (SC) em pacientes com AVCI submetidos à TM, focando especificamente nos resultados procedimentais e clínicos, bem como na incidência de eventos adversos. MéTODOS: Foram realizadas buscas sistemáticas nas bases PubMed, Embase e Cochrane por ensaios clínicos randomizados (ECRs) comparando AG versus SC em pacientes submetidos à TM devido a AVCI por OGV. Razões de chances (ORs) foram calculadas para desfechos binários, com intervalos de confiança de 95% (ICs). Modelos de efeitos aleatórios foram usados para todos os resultados. A heterogeneidade foi avaliada com estatísticas I2. RESULTADOS: Oito ensaios clínicos randomizados (1.300 pacientes) foram incluídos, dos quais 650 (50%) foram submetidos à AG. O sucesso da recanalização foi significativamente maior no grupo AG (OR 1,68; IC 95% 1,262,24; p < 0,04) em comparação com SC. No entanto, não houve diferença significativa entre os grupos para recuperação funcional adequada (OR 1,13; IC 95% 0,761,67; p = 0,56), incidência de pneumonia (OR 1,23; IC 95% 0,56- 2,69; p = 0,61), mortalidade em três meses (OR 0,99; IC 95% 0,73- 1,34; p = 0,95) ou hemorragia cerebral (OR 0,97; IC 95% 0,68- 1,38; p = 0,88). CONCLUSãO: Apesar do aumento significativo nas taxas de sucesso de recanalização no grupo AG, AG e SC mostram taxas semelhantes de recuperação funcional, mortalidade, pneumonia e hemorragia em pacientes com AVCI submetidos à TM.
Assuntos
Anestesia Geral , Sedação Consciente , AVC Isquêmico , Ensaios Clínicos Controlados Aleatórios como Assunto , Trombectomia , Humanos , Anestesia Geral/métodos , Sedação Consciente/métodos , AVC Isquêmico/cirurgia , Trombectomia/métodos , Resultado do TratamentoRESUMO
Abstract Background After recently published randomized clinical trials, the choice of the best anesthetic procedure for mechanical thrombectomy (MT) in acute ischemic stroke (AIS) due to large vessel occlusion (LVO) is not definite. Objective To compare the efficacy and safety of general anesthesia (GA) versus conscious sedation (CS) in patients with AIS who underwent MT, explicitly focusing on procedural and clinical outcomes and the incidence of adverse events. Methods PubMed, Embase, and Cochrane were systematically searched for randomized controlled trials (RCTs) comparing GA versus CS in patients who underwent MT due to LVO-AIS. Odds ratios (ORs) were calculated for binary outcomes, with 95% confidence intervals (CIs). Random effects models were used for all outcomes. Heterogeneity was assessed with I2 statistics. Results Eight RCTs (1,300 patients) were included, of whom 650 (50%) underwent GA. Recanalization success was significantly higher in the GA group (OR 1.68; 95% CI 1.26-2.24; p < 0.04) than in CS. No significant difference between groups were found for good functional recovery (OR 1.13; IC 95% 0.76-1.67; p = 0.56), incidence of pneumonia (OR 1.23; IC 95% 0.56- 2,69; p = 0.61), three-month mortality (OR 0.99; IC 95% 0.73-1.34; p = 0.95), or cerebral hemorrhage (OR 0.97; IC 95% 0.68-1.38; p = 0.88). Conclusion Despite the increase in recanalization success rates in the GA group, GA and CS show similar rates of good functional recovery, three-month mortality, incidence of pneumonia, and cerebral hemorrhage in patients undergoing MT.
Resumo Antecedentes A trombectomia mecânica (TM) é o padrão de tratamento para pacientes com acidente vascular cerebral isquêmico agudo (AVCI) devido à oclusão de grandes vasos (OGV). No entanto, ainda não está claro qual é o procedimento anestésico mais benéfico para a TM. Objetivo Nosso objetivo foi comparar a eficácia e a segurança da anestesia geral (AG) versus sedação consciente (SC) em pacientes com AVCI submetidos à TM, focando especificamente nos resultados procedimentais e clínicos, bem como na incidência de eventos adversos. Métodos Foram realizadas buscas sistemáticas nas bases PubMed, Embase e Cochrane por ensaios clínicos randomizados (ECRs) comparando AG versus SC em pacientes submetidos à TM devido a AVCI por OGV. Razões de chances (ORs) foram calculadas para desfechos binários, com intervalos de confiança de 95% (ICs). Modelos de efeitos aleatórios foram usados para todos os resultados. A heterogeneidade foi avaliada com estatísticas I2. Resultados Oito ensaios clínicos randomizados (1.300 pacientes) foram incluídos, dos quais 650 (50%) foram submetidos à AG. O sucesso da recanalização foi significativamente maior no grupo AG (OR 1,68; IC 95% 1,26-2,24; p < 0,04) em comparação com SC. No entanto, não houve diferença significativa entre os grupos para recuperação funcional adequada (OR 1,13; IC 95% 0,76-1,67; p = 0,56), incidência de pneumonia (OR 1,23; IC 95% 0,56- 2,69; p = 0,61), mortalidade em três meses (OR 0,99; IC 95% 0,73- 1,34; p = 0,95) ou hemorragia cerebral (OR 0,97; IC 95% 0,68- 1,38; p = 0,88). Conclusão Apesar do aumento significativo nas taxas de sucesso de recanalização no grupo AG, AG e SC mostram taxas semelhantes de recuperação funcional, mortalidade, pneumonia e hemorragia em pacientes com AVCI submetidos à TM.
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Leukodystrophies are a group of genetic diseases with diverse clinical features and prominent involvement of the central nervous system white matter. We describe a 27-year-old man who presented with a progressive neurological disease, and striking involvement of the brainstem and symmetrical white matter lesions on MR scanning. Having excluded several other causes of leukodystrophy, we confirmed Alexander disease when a genetic panel showed a probable pathogenic variant in GFAP: p.Leu359Pro. Clinicians should suspect Alexander disease in people with a progressive neurological motor decline who has pyramidal and bulbar signs and compatible neuroimaging.
Assuntos
Doença de Alexander , Medula Cervical , Masculino , Humanos , Adulto , Doença de Alexander/complicações , Doença de Alexander/diagnóstico por imagem , Doença de Alexander/genética , Proteína Glial Fibrilar Ácida/genética , Medula Cervical/patologia , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/patologia , Imageamento por Ressonância Magnética/métodosRESUMO
OBJECTIVE: To describe head computed tomography (CT) findings in neonates with congenital Zika virus infection confirmed in cerebrospinal fluid. MATERIALS AND METHODS: This was a study of 16 newborn infants who exhibited abnormal head CT findings during an outbreak of Zika virus infection. Those infants had the following features: brain imaging suggestive of congenital infection; brain calcifications and negative results on tests for other main infectious causes of primary microcephaly, namely toxoplasmosis, cytomegalovirus, rubella, and HIV; positivity for Zika virus on IgM antibody capture enzyme-linked immunosorbent assay in cerebrospinal fluid. RESULTS: Decreased brain volume was observed in 13 (81.2%) of the infants. All of the infants showed cortico-subcortical calcifications, mainly located in the frontal lobe. In 15 neonates (93.7%), ventriculomegaly was observed. Colpocephaly was a common finding, being observed in 10 patients (62.5%). A prominent occipital bone was identified in 9 patients (56.2%). CONCLUSION: Our study proves that Zika virus infection can cause congenital brain damage, with or without microcephaly. Some predominant head CT findings in neonates with congenital Zika virus infection, although not pathognomonic, are strongly suggestive of a pattern.
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Abstract Objective: To describe head computed tomography (CT) findings in neonates with congenital Zika virus infection confirmed in cerebrospinal fluid. Materials and Methods: This was a study of 16 newborn infants who exhibited abnormal head CT findings during an outbreak of Zika virus infection. Those infants had the following features: brain imaging suggestive of congenital infection; brain calcifications and negative results on tests for other main infectious causes of primary microcephaly, namely toxoplasmosis, cytomegalovirus, rubella, and HIV; positivity for Zika virus on IgM antibody capture enzyme-linked immunosorbent assay in cerebrospinal fluid. Results: Decreased brain volume was observed in 13 (81.2%) of the infants. All of the infants showed cortico-subcortical calcifications, mainly located in the frontal lobe. In 15 neonates (93.7%), ventriculomegaly was observed. Colpocephaly was a common finding, being observed in 10 patients (62.5%). A prominent occipital bone was identified in 9 patients (56.2%). Conclusion: Our study proves that Zika virus infection can cause congenital brain damage, with or without microcephaly. Some predominant head CT findings in neonates with congenital Zika virus infection, although not pathognomonic, are strongly suggestive of a pattern.
Resumo Objetivo: Descrever os achados da tomografia computadorizada (TC) de crânio em neonatos com infecção congênita pelo vírus Zika confirmada no líquido cefalorraquiano. Materiais e Métodos: Série de 16 recém-nascidos que apresentaram achados anormais na TC de crânio durante o surto de infecção por vírus Zika. Essas crianças apresentavam: imagens cerebrais sugestivas de infecção congênita; calcificações do parênquima cerebral e sorologias negativas para as outras principais causas infecciosas de microcefalia primária, como toxoplasmose, citomegalovírus, rubéola; IgM pelo método ELISA para vírus Zika positivo no líquido cefalorraquiano. Resultados: Redução volumétrica do parênquima encefálico foi observada em 13 (81,2%) pacientes. Todos os recém-nascidos apresentaram calcificações na junção corticossubcortical, localizadas principalmente no lobo frontal (100%). Em 15 neonatos (93,7%), ventriculomegalia esteve presente. Colpocefalia foi um achado comum, ocorrendo em 10 pacientes (62,5%). Proeminência do osso occipital foi identificada em 9 pacientes (56,2%). Conclusão: Nosso estudo comprova que a infecção pelo vírus Zika pode causar lesões encefálicas congênitas, com e sem microcefalia. Há um conjunto de achados tomográficos predominantes na TC de crânio em recém-nascidos com infecção congênita pelo vírus Zika, não patognomônicos, mas fortemente sugestivos de um padrão.
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Objective To compare initial brain computed tomography (CT) scans with follow-up CT scans at one year in children with congenital Zika syndrome, focusing on cerebral calcifications.Design Case series study.Setting Barão de Lucena Hospital, Pernambuco state, Brazil.Participants 37 children with probable or confirmed congenital Zika syndrome during the microcephaly outbreak in 2015 who underwent brain CT shortly after birth and at one year follow-up.Main outcome measure Differences in cerebral calcification patterns between initial and follow-up scans.Results 37 children were evaluated. All presented cerebral calcifications on the initial scan, predominantly at cortical-white matter junction. At follow-up the calcifications had diminished in number, size, or density, or a combination in 34 of the children (92%, 95% confidence interval 79% to 97%), were no longer visible in one child, and remained unchanged in two children. No child showed an increase in calcifications. The calcifications at the cortical-white matter junction which were no longer visible at follow-up occurred predominately in the parietal and occipital lobes. These imaging changes were not associated with any clear clinical improvements.Conclusion The detection of cerebral calcifications should not be considered a major criterion for late diagnosis of congenital Zika syndrome, nor should the absence of calcifications be used to exclude the diagnosis.