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OBJECTIVE: To establish the utility of long-term electroencephalogram (EEG) in forecasting epilepsy onset in children with autism spectrum disorder (ASD). STUDY DESIGN: A single-institution, retrospective analysis of children with ASD, examining long-term overnight EEG recordings collected over a period of 15 years, was conducted. Clinical EEG findings, patient demographics, medical histories, and additional Autism Diagnostic Observation Schedule data were examined. Predictors for the timing of epilepsy onset were evaluated using survival analysis and Cox regression. RESULTS: Among 151 patients, 17.2% (n = 26) developed unprovoked seizures (Sz group), while 82.8% (n = 125) did not (non-Sz group). The Sz group displayed a higher percentage of interictal epileptiform discharges (IEDs) in their initial EEGs compared with the non-Sz group (46.2% vs 20.0%, P = .01). The Sz group also exhibited a greater frequency of slowing (42.3% vs 13.6%, P < .01). The presence of IEDs or slowing predicted an earlier seizure onset, based on survival analysis. Multivariate Cox proportional hazards regression revealed that the presence of any IEDs (HR 3.83, 95% CI 1.38-10.65, P = .01) or any slowing (HR 2.78, 95% CI 1.02-7.58, P = .046 significantly increased the risk of developing unprovoked seizures. CONCLUSION: Long-term EEGs are valuable for predicting future epilepsy in children with ASD. These findings can guide clinicians in early education and potential interventions for epilepsy prevention.
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Transtorno do Espectro Autista , Eletroencefalografia , Epilepsia , Humanos , Masculino , Feminino , Estudos Retrospectivos , Transtorno do Espectro Autista/complicações , Transtorno do Espectro Autista/epidemiologia , Transtorno do Espectro Autista/diagnóstico , Transtorno do Espectro Autista/fisiopatologia , Eletroencefalografia/métodos , Criança , Epilepsia/diagnóstico , Pré-Escolar , Adolescente , Modelos de Riscos ProporcionaisRESUMO
BACKGROUND: Birdshot retinochoroiditis (BRC) is a rare and chronic bilateral uveitis mostly found in Caucasians. As few data are available about the clinical course of BRC in Hispanic patients, we aimed to report the clinical findings and the evolution of BRC in Brazilian patients. METHODS: This retrospective cohort multicenter nationwide study was performed by analyzing the records of patients with BRC diagnoses from Brazilian ophthalmological centers from April 1995 to May 2020. RESULTS: Forty patients (80 eyes) with a diagnosis of BRC were evaluated. The mean age was 53 years, and there was no sex predominance. All tested patients (34/40) were positive for HLA-A29. The diagnosis of BRC was made following the Levinson et al. criteria, and all ancillary tests were performed to exclude differential diagnoses. Clinical signs and symptoms, such as complications and treatment, were described. CONCLUSIONS: BRC evolution in Brazilian patients seems to have some peculiarities that diverge from the published literature available about Caucasians, as AS inflammation is higher in this population.
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Purpose: To assess the effectiveness of intravitreal (IV) bevacizumab and the need for systemic immunosuppressive therapy (IMT) in choroidal neovascularization (CNV) in Vogt-Koyanagi-Harada disease (VKHD).Methods: CNV treatment consisted of three monthly IV bevacizumab injections as a loading dose; if intra/subretinal fluid (IRF) persisted, further injections were proceeded besides increment in systemic IMT. Outcome analyses at 3, 6, and 12 months were visual acuity, central foveal thickness, macular volume, IRF, and addition of IMT.Results: Seven eyes of six patients were included. Five patients (five eyes) completed a 12-month follow-up and received 12 IV bevacizumab injections. At the 12-month follow-up, visual acuity improved in four out of five eyes (p = .0568); all eyes had decreased macular volume (p = .0431) but they still had persistent IRF; and all cases needed IMT introduction/increment.Conclusion: Intravitreal bevacizumab in association with systemic IMT was effective for CNV in VKHD. Active CNV may indicate disease of inadequate clinical control.
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Inibidores da Angiogênese/uso terapêutico , Bevacizumab/uso terapêutico , Neovascularização de Coroide/tratamento farmacológico , Síndrome Uveomeningoencefálica/complicações , Adulto , Neovascularização de Coroide/etiologia , Neovascularização de Coroide/fisiopatologia , Feminino , Angiofluoresceinografia , Seguimentos , Humanos , Injeções Intravítreas , Pessoa de Meia-Idade , Estudos Prospectivos , Líquido Sub-Retiniano , Tomografia de Coerência Óptica , Resultado do Tratamento , Síndrome Uveomeningoencefálica/fisiopatologia , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Acuidade Visual/fisiologiaRESUMO
Tropical forests modify the conditions they depend on through feedbacks at different spatial scales. These feedbacks shape the hysteresis (history-dependence) of tropical forests, thus controlling their resilience to deforestation and response to climate change. Here, we determine the emergent hysteresis from local-scale tipping points and regional-scale forest-rainfall feedbacks across the tropics under the recent climate and a severe climate-change scenario. By integrating remote sensing, a global hydrological model, and detailed atmospheric moisture tracking simulations, we find that forest-rainfall feedback expands the geographic range of possible forest distributions, especially in the Amazon. The Amazon forest could partially recover from complete deforestation, but may lose that resilience later this century. The Congo forest currently lacks resilience, but is predicted to gain it under climate change, whereas forests in Australasia are resilient under both current and future climates. Our results show how tropical forests shape their own distributions and create the climatic conditions that enable them.
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Florestas , Clima Tropical , África , Sudeste Asiático , Austrália , Mudança Climática , Conservação dos Recursos Naturais , Ecossistema , Retroalimentação , Chuva , América do SulRESUMO
PURPOSE: To analyze the contribution of IgG anti-Toxocara antibodies in the diagnosis of ocular toxocariasis (OT) in serum samples of patients attending a public referral center for ophthalmology in Brazil and to determine the most frequent ocular signs and symptoms. STUDY DESIGN: This was a retrospective descriptive study of a cohort of outpatients followed from December 1989 to May 2006. METHODS: IgG anti-Toxocara antibody titers were determined by enzyme-linked immunosorbent assay. The results were correlated with the clinical and funduscopic findings. RESULTS: Of the diagnoses for the 126 patients, 42 were considered as probable OT, 48 as negative, and 36 as inconclusive. The median IgG anti-Toxocara antibody titers of the patients with probable OT were significantly higher than those of the patients in the negative or inconclusive groups (P = 0.0001). Higher titers were observed more frequently in patients in the probable OT group (P = 0.01) than in the negative group, in which titers were absent or lower in most patients (P < 0.0001). The right eye was affected more in the OT group than in the negative group (P = 0.01), and inflammation was located mostly in the intermediate and posterior poles. The mean age of the patients diagnosed with probable OT was significantly lower (mean, 8 years) than that of the negative patients (mean, 14.5 years) (P = 0.006). CONCLUSIONS: Higher levels of antibodies could help with the diagnosis of OT in patients with symptoms and clinical signs; however, negative results cannot exclude possible OT. Conversely, patients with negative serology or low titers make the diagnosis of OT less likely.
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Anticorpos Anti-Idiotípicos/sangue , Anticorpos Anti-Helmínticos/sangue , Infecções Oculares Parasitárias/diagnóstico , Imunoglobulina G/sangue , Toxocara/imunologia , Toxocaríase/diagnóstico , Adolescente , Albendazol/uso terapêutico , Animais , Anti-Helmínticos/uso terapêutico , Antígenos de Helmintos/imunologia , Brasil/epidemiologia , Criança , Terapia Combinada , Quimioterapia Combinada , Ensaio de Imunoadsorção Enzimática , Infecções Oculares Parasitárias/epidemiologia , Infecções Oculares Parasitárias/parasitologia , Feminino , Glucocorticoides/uso terapêutico , Hospitais Públicos , Humanos , Masculino , Oftalmologia , Prednisolona/uso terapêutico , Encaminhamento e Consulta , Estudos Retrospectivos , Estudos Soroepidemiológicos , Toxocaríase/epidemiologia , Toxocaríase/parasitologia , VitrectomiaRESUMO
PURPOSE: To analyse the rate of clinical recurrences in Brazilian patients with Vogt-Koyanagi-Harada (VKH) disease after early high-dose corticosteroid treatment. METHODS: Retrospective study including patients treated with early high-dose corticosteroids (prednisone, 1-1.5 mg/kg/day, or 3-day 1 g methylprednisolone pulsetherapy) within 1 month from disease onset followed by slow taper (at least 6 months). Patients with a minimum 12-month follow-up were subdivided based on the presence of disease recurrence or persistence after 6 months from initial presentation into: acute-resolved (AR, no recurrences), chronic-recurrent (CR), and chronic-recurrent with subretinal fibrosis (SRF). Recurrences were defined as the presence of clinical and/or fluorescein angiography findings. RESULTS: Twenty-nine patients (58 eyes) with a median follow-up of 65 months were included. Six (21 %), 11 (38 %) and 12 (41 %) patients were allocated to AR, CR, and SRF groups respectively. Though having received treatment within 1 month of onset, median time to initial treatment differed among groups (11, 15, and 25 days, in AR, CR, and SRF groups respectively). Intensity of immunosuppression, cataract development, and longer time to achieve logMAR visual acuity ≤0.8 differed significantly among the groups, being more severe in SRF group. HLA-DRB1*0405 allele followed the same trend, though not reaching significance (0.5 in AR group, 0.6 in CR, and 0.8 in SRF). CONCLUSION: VKH disease in Brazilian patients evolved to chronic-recurrent disease in 79 % of cases; 38 % developed subretinal fibrosis, in spite of similar initial treatment regimens. Time to initiate treatment influenced outcomes.
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Glucocorticoides/administração & dosagem , Metilprednisolona/administração & dosagem , Prednisona/administração & dosagem , Síndrome Uveomeningoencefálica/diagnóstico , Síndrome Uveomeningoencefálica/tratamento farmacológico , Adolescente , Adulto , Brasil/epidemiologia , Criança , Doença Crônica , Feminino , Fibrose , Angiofluoresceinografia , Cadeias HLA-DRB1/genética , Humanos , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Prognóstico , Pulsoterapia , Recidiva , Retina/patologia , Estudos Retrospectivos , Síndrome Uveomeningoencefálica/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Detection of choroidal inflammation in Vogt-Koyanagi-Harada (VKH) disease is still a challenge. Progression to sunset glow fundus has been observed despite apparent good clinical control of inflammation. Indocyanine green angiography (ICGA) permits choroid inflammation detection, though it is invasive, time consuming, and costly. The purpose of the present study is to report a sign indicative of probable inflammation on enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT): a localized increase in choroidal thickness with bulging of the outer retina ('choroidal bulging') in patients with VKH disease in the non-acute uveitic stage. FINDINGS: This is a retrospective observational study. The choroidal bulging was a particular finding observed in four eyes of three patients with VKH disease in the non-acute uveitic stage (median disease duration 55.3 ± 40.3 months, range 10 to 108). This study is part of an ongoing longitudinal study in patients with VKH disease carried out in the Uveitis Service, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil. In all eyes, the choroidal bulging was identified in the presence of anterior chamber cells and/or on fundus angiographic (fluorescein and indocyanine green) findings, indicative of disease activity. Changes in the thickness of the choroidal bulging accompanied the variation in the clinical and angiographic signs of inflammation. CONCLUSION: The choroidal bulging is a particular finding detected on EDI-OCT that may indicate ongoing inflammation in the posterior segment of the eye. This EDI-OCT feature may assist in the treatment-monitoring of patients with Vogt-Koyanagi-Harada disease in the non-acute uveitic stage.
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OBJECTIVE: To describe the prevalence, characteristics, and predictors of electrographic seizures after convulsive status epilepticus (CSE). STUDY DESIGN: This was a multicenter retrospective study in which we describe clinical and electroencephalographic (EEG) features of children (1 month to 21 years) with CSE who underwent continuous EEG monitoring. RESULTS: Ninety-eight children (53 males) with CSE (median age of 5 years) underwent subsequent continuous EEG monitoring after CSE. Electrographic seizures (with or without clinical correlate) were identified in 32 subjects (33%). Eleven subjects (34.4%) had electrographic-only seizures, 17 subjects (53.1%) had electroclinical seizures, and 4 subjects (12.5%) had an unknown clinical correlate. Of the 32 subjects with electrographic seizures, 15 subjects (46.9%) had electrographic status epilepticus. Factors associated with the occurrence of electrographic seizures after CSE were a previous diagnosis of epilepsy (P = .029) and the presence of interictal epileptiform discharges (P < .0005). The median (p25-p75) duration of stay in the pediatric intensive care unit was longer for children with electrographic seizures than for children without electrographic seizures (9.5 [3-22.5] vs 2 [2-5] days, Wilcoxon test, Z = 3.916, P = .0001). Four children (4.1%) died before leaving the hospital, and we could not identify a relationship between death and the presence or absence of electrographic seizures. CONCLUSIONS: After CSE, one-third of children who underwent EEG monitoring experienced electrographic seizures, and among these, one-third experienced entirely electrographic-only seizures. A previous diagnosis of epilepsy and the presence of interictal epileptiform discharges were risk factors for electrographic seizures.
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Eletroencefalografia , Monitorização Fisiológica/métodos , Convulsões/complicações , Estado Epiléptico/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Convulsões/diagnóstico , Convulsões/epidemiologia , Espanha/epidemiologia , Estado Epiléptico/diagnóstico , Estado Epiléptico/epidemiologia , Adulto JovemRESUMO
BACKGROUND: To investigate indocyanine green angiography (ICGA) findings in patients with long-standing Vogt-Koyanagi-Harada (VKH) disease and their correlation with disease activity on clinical examination as well as with systemic corticosteroid therapy. METHODS: Twenty-eight patients (51 eyes) with long-standing (≥6 months from disease onset) VKH disease whose treatment was tapered based only in clinical features were prospectively included at a single center in Brazil. All patients underwent standardized clinical evaluation, which included fundus photography, fluorescein angiography and ICGA. Clinical disease activity was determined based in the Standardization in Uveitis Nomenclature Working Group. Fisher exact test and logistic regression models were used for statistical analysis. RESULTS: Disease-related choroidal inflammation on ICGA was observed in 72.5% (31 of 51 eyes). Angiographic findings suggestive of (choroidal and/or retinal) disease activity were not observed on FA. Clinically active disease based on clinical evaluation was observed in 41.2% (21 of 51 eyes). In these 21 eyes, disease-related choroidal inflammation on ICGA was observed in 76.2% (16 of 21 eyes); in the remaining eyes (without clinical active disease) disease-related choroidal inflammation on ICGA was observed in 70.0% (21 of 30 eyes). In respect to systemic corticosteroid therapy, 10 patients (18 of 51 eyes) were under treatment with prednisone. In these 10 (18 of 51 eyes) patients, disease-related choroidal inflammation on ICGA was observed in 83.3% (15 of 18 eyes); in the remaining patients (33 of 51 eyes) disease-related choroidal inflammation on ICGA was observed in 66.7% (22 of 33 eyes). CONCLUSION: ICGA findings suggestive of disease-related choroidal inflammation were observed in a considerable proportion of patients with long-standing VKH disease, independent of the inflammatory status of the disease on clinical examination or current use of systemic corticosteroid. Therefore, the current study reinforces the crucial role of ICGA to assist the management and treatment of patients with long-standing VKH disease.
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Verde de Indocianina , Retina/patologia , Síndrome Uveomeningoencefálica/diagnóstico , Adulto , Brasil/epidemiologia , Corantes , Estudos Transversais , Feminino , Angiofluoresceinografia , Seguimentos , Fundo de Olho , Humanos , Incidência , Masculino , Estudos Prospectivos , Síndrome Uveomeningoencefálica/epidemiologiaRESUMO
Children and adults can have recurrent infection with invasive encapsulated bacterial pathogens such as Streptococcus pneumoniae as a result of a selective inability to respond to polysaccharide antigens. We have identified a mutation in the gene encoding Bruton's tyrosine kinase in a male patient with selective anti-polysaccharide antibody deficiency.