Clinical Course and Ophthalmologic Findings in Idiopathic Intracranial Hypertension and Pregnancy.

Idiopathic intracranial hypertension (IIH) has its highest prevalence among women of childbearing age and therefore frequently coincides with pregnancy. This retrospective cohort study aimed to explore the impact of pregnancy on the clinical course, ophthalmologic findings and on the therapeutic management of IIH patients. Individual patient records were reviewed for neuro-ophthalmologic findings, treatment strategy, adherence to therapy and pregnancy complications. Sixteen patients with 19 documented pregnancies were identified. The visual acuity, visual field defects and the grade of papilledema at baseline and after pregnancy were compared. The visual acuity and visual field mean deviation at baseline and at follow-up after pregnancy did not significantly differ. Papilledema at baseline was more pronounced in patients who had been diagnosed with IIH during pregnancy than in patients with established IIH. In this cohort, the visual acuity and the visual field were not lastingly impacted by pregnancy. The adherence to therapy was low, with 69% discontinuing treatment or medication.

Neurofilament light chain marks severity of papilledema in idiopathic intracranial hypertension.

BACKGROUND: Neurofilament light chain (NfL) reflects axonal damage in neurological disorders. It has recently been evaluated in idiopathic intracranial hypertension (IIH). A biomarker indicating the severity of optic nerve damage in IIH could support diagnostic accuracy and therapeutic decisions. METHODS: We retrospectively reviewed NfL concentrations in the cerebrospinal fluid (CSF) of 35 IIH patients and 12 healthy controls, who had received diagnostic workup for IIH in our clinic. The diagnosis of IIH was made according to the modified Friedman criteria for IIH and for IIH without papilledema Friedman DI et al Neurol 81:1159-1165 (2013) [1]. NfL in the CSF (CSF-NfL) was correlated with the severity of papilledema and with CSF opening pressure. RESULTS: CSF-NfL correlated with CSF opening pressure at the time of collection. In patients with IIH and moderate or severe papilledema, CSF-NfL was significantly increased compared to patients with mild or no papilledema. Healthy controls with raised intracranial pressure showed no relevant elevation of CSF-NfL. CONCLUSION: CSF-NfL appears to correlate with the severity of papilledema in IIH and with CSF opening pressure and may therefore be a predictor of optic nerve damage in IIH patients.

Noninvasive Detection of Intracranial Hypertension by Novel Ultrasound Time-Harmonic Elastography.

OBJECTIVE: A method for measuring intracranial pressure (ICP) noninvasively has long been sought after in neurology and neurosurgery. Treatment failure in individuals presenting with unspecific symptoms such as headache, gait disturbance, or visual impairment occurring in response to increased ICP can lead to irreversible brain injury, progressive disability, and death. Guidelines for diagnostic ICP measurement recommend intracranial placement of pressure tip catheters or lumbar puncture (LP) despite their invasiveness and possible complications. As ICP fluctuations are closely associated with changes in brain stiffness, ultrasound elastography could be a valid method to detect ICP noninvasively and with short examination times. MATERIALS AND METHODS: In this pilot study, we have investigated the use of time-harmonic shear waves, introduced into the brain by an external shaker, and measured in real-time by transtemporal ultrasound, for deducing a noninvasive imaging marker sensitive to elevated ICP. To this end, we developed cerebral ultrasound time-harmonic elastography for the noninvasive quantification of shear wave speed (SWS) as a surrogate marker of cerebral stiffness in a short examination time of a few minutes. RESULTS: We found that SWS in patients enrolled for LP with confirmed intracranial hypertension was 1.81 ± 0.10 m/s, distinguishing them from healthy volunteers with excellent diagnostic accuracy (1.55 ± 0.08 m/s; P < 0.001; area under the curve, 0.99). Interestingly, values in symptomatic patients decreased to normal stiffness immediately after LP (1.56 ± 0.06 m/s, P < 0.001). Moreover, invasively measured opening pressure correlated with SWS measured before LP and liquid volume drained through the spinal tap with the SWS difference between the 2 measurements. CONCLUSIONS: Collectively, our results suggest a tight link between cerebral stiffness and ICP and demonstrate that intracranial hypertension can be detected noninvasively within short examination times, opening avenues for diagnostic applications of cerebral ultrasound time-harmonic elastography in neurology and emergency medicine.

Improving functional outcome in bipolar disorder: A pilot study on metacognitive training.

BACKGROUND: Effective group psychological interventions in bipolar disorder are rare. In this study, we present "metacognitive training (MCT) for bipolar disorder"-an adaption of a group intervention that has proven effective in other severe psychiatric disorders. MCT is a structured, interactive approach that addresses cognitive biases, social cognition, and self-esteem. In this pilot study, we investigated psychosocial functioning as primary outcome measure, as well as the feasibility of MCT and its acceptance among bipolar patients. METHODS: Thirty-four outpatients with bipolar disorder were recruited. Inclusion criteria were euthymia and psychosocial functioning with a score >11 assessed by the Functional Assessment Short Test. The subjects received eight weekly MCT sessions. Before and after the intervention, psychosocial functioning, quality of life (QoL), and patient views were assessed. RESULTS: Patients improved significantly in global psychosocial functioning, with a large effect size from baseline to post-treatment. Over the intervention period, patient QoL improved significantly in terms of their physical health, however not for other QoL subdomains. Treatment adherence was 80%, and patients' appraisal of the training was positive. LIMITATIONS: As this study lacks a control group, it is not possible to ascertain whether the positive treatment effects are attributable to MCT. Additionally, it is unclear whether gains in psychosocial functioning would have been maintained long term. CONCLUSIONS: This pilot trial conclusively shows that MCT is feasible and provides preliminary evidence for both the acceptance and efficacy of MCT. Further studies with larger samples and control condition will be necessary to build on these findings.

No differences in visual theory of mind abilities between euthymic bipolar patients and healthy controls.

BACKGROUND: Research on theory of mind (ToM) abilities in patients with bipolar disorder has yielded conflicting results. Meta-analyses point to a stable moderate impairment in remitted patients, but factors such as subsyndromal symptoms, illness severity, and deficits in basic neurocognitive functions might act as confounders. Also, differences in deficits depending on task area (cognitive or affective) or task modality (visual or verbal) have been observed. This study aimed to test the hypothesis that euthymic bipolar patients would perform more poorly than healthy subjects on visual cognitive and visual affective ToM tasks. Furthermore, we aimed to explore the relationship between ToM performance and basic neurocognitive functions, subsyndromal symptom severity, and illness burden. Twenty-nine clinically stable outpatients with bipolar disorder and 29 healthy comparison subjects completed a measure of visual cognitive ToM (Mental State Attribution Task, MSAT), a measure of visual affective ToM (Reading the Mind in the Eyes Test, RMET), and a battery of tests assessing neurocognitive functioning (attention, verbal memory, executive functions, and intelligence). RESULTS: Patients did not differ significantly from healthy controls for the ToM tasks or any of the neurocognitive measures, suggesting a high level of neurocognitive functioning in the bipolar group. On average, patients were slower than controls to complete the ToM tasks. Within the bipolar group, ToM performance was moderately correlated with attention, verbal memory and reasoning abilities. Performance on the RMET was positively correlated with clinician-rated depressive symptoms with a small effect. Number of years of illness was weakly and negatively correlated with performance on the MSAT. Overall, no moderate or strong correlations were found between ToM performance, subsyndromal depressive or manic symptoms, illness duration, and number of depressive or (hypo)manic episodes. Moderate correlations between ToM performance and age were found for patients but nor for controls. CONCLUSIONS: Our findings suggest preserved visual cognitive and affective ToM abilities in euthymic bipolar patients characterized by a high level of neurocognitive functioning.

Behavioral measures and self-report of impulsivity in bipolar disorder: no association between Stroop test and Barratt Impulsiveness Scale.

BACKGROUND: Impulsivity as a tendency to act quickly without considering future consequences has been proposed as a dimensional factor in bipolar disorder. It can be measured using behavioral tasks and self-report questionnaires. Previous findings revealed patients to show worse performance on at least one behavioral measure of impulsivity. Additionally, self-reported impulsivity seems to be higher among bipolar patients, both parameters being possibly associated with a more severe course of illness. In this study, our primary aim was to investigate the relationship between these two constructs of impulsivity among bipolar patients. METHODS: A total of 40 euthymic patients with bipolar disorder (21 female, 22 Bipolar I) and 30 healthy controls were recruited for comprehensive neuropsychological assessment. To assess inhibition control as a behavioral measure of impulsivity, the Stroop Color and Word Test (Stroop) was used. Additionally, both groups completed the Barratt Impulsiveness Scale (BIS) as a self-report of impulsivity. To compare the groups' performance on the Stroop and ratings on the BIS, the non-parametric Mann-Whitney U test was used. Within the bipolar group, we additionally examined the possibility of an association between Stroop performance and BIS total scores using Pearson's Correlation r. RESULTS: Patients and controls differed significantly on the Stroop and BIS, with patients performing worse on the Stroop and scoring higher on the BIS. However, there was no association between the Stroop and BIS within the bipolar group. As an exploratory analysis, a positive correlation between Stroop performance and number of episodes was found. Further, we detected a statistical trend in the direction of poorer Stroop performance among patients treated with polypharmacy. CONCLUSIONS: Both difficulties with behavioral inhibition and self-reported impulsivity were observed to be higher in bipolar patients than controls in the current study. However, within the patient group we did not observe an association between patients' behavioral performance and self-report. This indicates that the parameters likely constitute distinct, dimensional factors of bipolar disorder. In future research, studies with larger samples should investigate which of the two markers constitutes the better marker for the illness and is more suitable to differentiate the most severe patients.

Infarto do miocárdio e mixoma em átrio esquerdo / Myocardial infarction and myxoma in the left atrium / Infarto del Miocardio y Mixoma en Aurícula Izquierda

Os mixomas são os tumores cardíacos primários benignos mais comuns, sendo que a grande maioria localiza-se no átrio esquerdo; 80 por cento têm sua origem no septo interatrial e 5 por cento deles são biatrial. Clinicamente, apresentam-se, quase sempre, com sinais e sintomas de doença valvular mitral ou de eventos tromboembólicos. Este relato ilustra um caso de mixoma em átrio esquerdo, projetando-se através da valva mitral para o ventrículo esquerdo e simulando uma estenose mitral, que evoluiu com acidente vascular cerebral isquêmico (AVCi) e infarto agudo do miocárdio (IAM), como complicações tromboembólicas. O ecocardiograma continua a ser uma ferramenta valiosa no diagnóstico e o tratamento cirúrgico imediato é necessário para evitar desfechos fatais.

Myxomas are the most common benign primary cardiac tumors, the vast majority located in the left atrium and 80 percent originates in the atrial septum and 5 percent of them are biatrial.Usually present, often with signs and symptoms of mitral valve disease or thromboembolic events. This report illustrates a case of myxoma in left atrium protrudind through the mitral valve into the left ventricle, simulating stenotic mitral valve disease, which evolved with ischemic stroke (AIS) and acute myocardial infarction (MI) as thromboembolic events. Echocardiography continues to be a valuable tool in the diagnosis and surgical treatment is immediately necessary to prevent fatal outcomes.

Los mixomas son los tumores cardíacos primarios benignos más comunes, siendo que la gran mayoría se localiza en la aurícula izquierda; el 80% tiene su origen en el septo interauricular y el 5% de ellos son biauriculares. Clínicamente, se presentan, casi siempre, con señales y síntomas de enfermedad valvular mitral o de eventos tromboembólicos. Este relato ilustra un caso de mixoma en aurícula izquierda, proyectándose a través de la válvula mitral hacia el ventrículo izquierdo y simulando una estenosis mitral, que evolucionó con accidente vascular cerebral isquémico (AVCi) e infarto agudo del miocardio (IAM), con complicaciones tromboembólicas. El ecocardiograma continua siendo una herramienta valiosa en el diagnóstico y el tratamiento quirúrgico inmediato es necesario para evitar desenlaces fatales.

Cardiomiopatia secundária á distrofia de Steinert / Cardiomyopathy secondary to Steinert's dystrophy / Miocardiopatía secundaria a distrofia de Steinert

A distrofia miotônica tipo 1 (DM1 ou doença de Steinert) é uma doença genética com repercussões multissistêmicas, na qual é comum o paciente buscar diversos especialistas antes da suspeição clínica. O envolvimento cardíaco é uma das características principais da evolução da DM1 e explica, em parte, a menor expectativa de vida dos pacientes. Relata-se caso de paciente masculino, 28 anos, com admissão por insuficiência cardíaca descompensada após oito anos de diagnóstico quando apresentou episódio de síncope secundária a bloqueio atrioventricular total. Sob marca-passo definitivo desde então. Discutem-se os aspectos etiológicos, fisiopatológicos e clínicos da DM1 com ênfase nas manifestações cardiovasculares.

Myotonic Dystrophy type 1 (MD1 or Steinert's Disease) is a genetic syndrome with multissistemic repercussions, and it is usual to the patient to seek for several specialists before clinical suspicion. It is related a case of a 28 years old male patient admitted with congestive heart failure. MD1 has been diagnosed eight years before. At the time of the diagnosis, he presented syncope secondary to Complete Atrioventricular Block. From that time on, he had a permanent pacemaker implanted. Etiological, pathophysiological and clinical aspects of MD1 with emphasis on cardiovascular manifestations are discussed.

La distrofia miotónica tipo 1 (DM1 o enfermedad de Steinert) es un síndrome genético con efectos multisistémicos, siendo común que los pacientes consulten con varios especialistas antes de la sospecha clínica. El compromiso cardíaco es una de las características principales de la evolución de la DM1 y explica, en parte, una menor expectativa de vida de los pacientes. Se presenta el caso de un paciente masculino de 28 años, que ingresó por insuficiencia cardíaca descompensada. Se le había diagnosticado DM1 ocho años antes, al presentar episodios sincopales secundarios a bloqueo aurículo-ventricular completo. A partir de entonces, se le realiza el implante de un marcapasos definitivo. Se discuten los aspectos etiológicos, fisiopatológicos y clínicos de la DM1 con énfasis en las manifestaciones cardiovasculares.

Cardiomiopatia secundária á distrofia de Steinert / Cardiomyopathy secondary to Steinerts dystrophy / Miocardiopatía secundaria a distrofia de Steinert

A distrofia miot¶nica tipo 1 (DM1 ou doenþa de Steinert) é uma doenþa genética com repercuss§es multissistÛmicas, na qual é comum o paciente buscar diversos especialistas antes da suspeiþÒo clínica. O envolvimento cardíaco é uma das características principais da evoluþÒo da DM1 e explica, em parte, a menor expectativa de vida dos pacientes. Relata-se caso de paciente masculino, 28 anos, com admissÒo por insuficiÛncia cardíaca descompensada após oito anos de diagnóstico quando apresentou episódio de síncope secundária a bloqueio atrioventricular total. Sob marca-passo definitivo desde entÒo. Discutem-se os aspectos etiológicos, fisiopatológicos e clínicos da DM1 com Ûnfase nas manifestaþ§es cardiovasculares.(AU)

Myotonic Dystrophy type 1 (MD1 or Steinerts Disease) is a genetic syndrome with multissistemic repercussions, and it is usual to the patient to seek for several specialists before clinical suspicion. It is related a case of a 28 years old male patient admitted with congestive heart failure. MD1 has been diagnosed eight years before. At the time of the diagnosis, he presented syncope secondary to Complete Atrioventricular Block. From that time on, he had a permanent pacemaker implanted. Etiological, pathophysiological and clinical aspects of MD1 with emphasis on cardiovascular manifestations are discussed.(AU)

La distrofia miotónica tipo 1 (DM1 o enfermedad de Steinert) es un síndrome genético con efectos multisistémicos, siendo común que los pacientes consulten con varios especialistas antes de la sospecha clínica. El compromiso cardíaco es una de las características principales de la evolución de la DM1 y explica, en parte, una menor expectativa de vida de los pacientes. Se presenta el caso de un paciente masculino de 28 años, que ingresó por insuficiencia cardíaca descompensada. Se le había diagnosticado DM1 ocho años antes, al presentar episodios sincopales secundarios a bloqueo aurículo-ventricular completo. A partir de entonces, se le realiza el implante de un marcapasos definitivo. Se discuten los aspectos etiológicos, fisiopatológicos y clínicos de la DM1 con énfasis en las manifestaciones cardiovasculares.(AU)

Hipertensão arterial pulmonar: dificuldades diagnósticas / Pulmonary hypertension: diagnostic difficulties

Mulher, 54 anos, relatou episódio de tromboembolismo pulmonar em 2007 que evoluiu para cor pulmonalecrônico. A partir de julho de 2011, apresentou piora progressiva da dispneia, estase jugular, hepatopatiacongestiva e edema de membros inferiores. O ecocardiograma evidenciou disfunção importante do ventrículo direito associado à grave hipertensãopulmonar. A paciente evoluiu com choque cardiogênico, hipoxemia refratária e óbito. Objetiva-se discutir ospossíveis mecanismos fisiopatológicos relacionados à ocorrência de hipertensão pulmonar, suas dificuldadesdiagnósticas, bem como suas implicações clínicas e abordagens terapêuticas.

Qualidade da abordagem diagnóstica e terapêutica da insuficiência cardíaca descompensada em uma série consecutiva de pacientes internados em hospital universitário / Quality of diagnostic and therapeutic approach to descompensated heart failure in a consecutive series of patients in a university hospital

A aplicação de intervenções baseadas em evidências é fundamental para a redução da morbimortalidade associada à insuficiência cardíaca (IC). Avaliar a qualidade da abordagem dos pacientes internados com IC descompensada (ICD) em um hospital universitário (HU) e comparar com registros internacionais (RI). Estudo retrospectivo de 261 hospitalizações consecutivas por ICD no período de 01/01/2006 a 30/06/2007. Utilizaram-se como instrumentos de medida de qualidade (JCAHO, ACC/AHA, OPTIMIZE): taxa de uso de IECA...

O valor prognóstico da largura do QRS nos pacientes com cardiopatias chagásica crônica / Prognostic value of QRS width in patients with chronic chagas cardiomyopathy

Fundamentos: O valor prognóstico da largura do complexo QRS(LQRS) na cardiopatia chagásica crônica(CCC) ainda não foi estudado. A alta prevalência do BRD nessa população, bem como o seu surgimento precoce, torna essa informação prognóstica fundamental Objetivos: 1. Avaliar o valor prognóstico da LQRS em relação à mortalidade em uma coorte de pacientes com CCC e nos seguintes subgrupos: fração de ejeção do ventrículo esquerdo (FEVE) <45%; insuficiência cardíaca (IC); e sem bloqueio do ramo direito (BRD). 2. Correlacionar a LQRS com a FEVE ao ecocardiograma. Métodos: Estudo observacional, de coorte, com 612 pacientes portadores de CCC acompanhados de março de 1990 a dezembro de 2003 e submetidos à avaliação clínica, eletrocardiográfica, radiológica e ecocardiográfica. Foi realizada análise retrospectiva da LQRS dos eletrocardiogramas iniciais, correlacionando-a com dados clínicos e ecocardiográficos prospectivos. Na análise univariada foram utilizados o teste qui-quadrado e o teste t de Student. Análise uni e multivariada de Cox foram utilizadas para avaliar o valor prognóstico da LQRS. Curvas de sobrevida foram construídas e comparadas pelo teste de log-rank. Resultados: Acompanhamento de 67±44 meses, seguimento completo em 89%. Ocorreram 91 óbitos (76 cardíacos). A correlação entre LQRS e FEVE foi fraca (r=-0,187). A LQRS, testada como variável contínua, foi preditora de morte total (p=0,004) e cardíaca (p=0,011); porém quando dicotomizada em 120ms perdeu seu poder preditivo. No modelo multivariado, a LQRS deixou de ser preditora de morte. O mesmo aconteceu nos subgrupos pré-especificados. Conclusão: Diferente de estudos em cardiopatia nãochagásica, a LQRS não foi uma variável prognóstica em pacientes com CCC.

Background: The prognostic value of QRS width (QRSW) in chronic Chagas cardiomyopathy (CCC) has not yet been studied. The high prevalence of right bundle branch block (RBBB) in this population, together with its early appearance, makes this information vital for prognoses. Objectives: 1. To evaluate the prognostic value of QRSW for mortality in a cohort of CCC and the following subgroups: left ventricular ejection fraction (LVEF) <45%, heart failure (HF) and patients without RBBB. 2. To correlate the QRSW with the LVEF on the echocardiogram. Methods: Observational study of a cohort of 612 patients with CCC monitored from March 1990 to December 2003 and undergoing clinical, electrocardiography, radiological and echocardiography evaluations. A retrospective analysis of the QRSW in the initial electrocardiograms was correlated to prospective echocardiographic and clinical data. For the univariate analysis, chi-square and t-Student tests were conducted. To analyze the prognostic value of QRSW, univariate and Cox multivariate analyses were performed. Survival curves were constructed and compared (log-rank test). Results: Follow-up for 67±44 months (with complete followup for 89%), with 91 deaths (76 cardiac). There was a weak correlation between QRSW and LVEF (r=-0.187). Tested as a continuous variable), the QRSW served as a predictor of all cause mortality (p=0.004) and cardiac mortality (p=0.011). However, when dichotomizing at 120ms, QRSW has lost its predictive power. For the multivariate model, QRSW was not a predictor for cardiac and all cause mortality, with the same occurring in the pre-specified sub-groups. Conclusion: In contrast to studies of non-Chagas cardiomyopathy, QRSW was not a prognostic variable for CCC patients.

Mecanismo de morte e grau de acometimento miocárdico na fase crônica da doença de chagas / Death mechanisms and myocardial effect levels in the chronic phase of Chagas disease

Objetivo: Avaliar os mecanismos de morte, correlacionando-os com o grau de acometimento miocárdico na fase crônica da doença de Chagas (DC). Métodos: Foi analisada uma coorte de 1167 pacientes com DC recrutados entre 03/1990 e 12/2003. Todos foram submetidos a exame clínico, ECG, RX tórax e ECO. Na avaliação da gravidade do acometimento miocárdico, foi utilizada a classificação preconizada pelo Consenso Brasileiro em DC (2005) e a FE do VE estimada ao ECO. Resultados: Durante 67 mais ou menos 43 meses de seguimento ocorreram 106 óbitos, 82 de causa cardíaca, sendo 54 mortes súbitas (MS), 22 mortes por IC refratária e 6 mortes decorrentes de AVE embólico. O mecanismo de óbito variou de acordo com o estágio da cardiopatia (p menor 0,0001) e com o grau de disfunção ventricular (p menor 0,0001). MS foi o mecanismo mais frequente de óbito em todos os estágios, exceto no stágio D em que predominou a morte por IC. A maioria dos óbitos por AVE ocorreu em pacientes sem IC. A média da FE do VE foi diferente nos diversos mecanismos de morte: pacientes com morte não-cardíaca (p menor 0,0001). Conclusões: MS foi o mecanismo predominante de óbito, ocorrendo frequentemente em pacientes sem IC e/ou com FE maior 35 por cento. IC progressiva foi o principal mecanismo de óbito entre os pacientes no estágio D e foi associada à disfunção sistólica do VE mais avançada. AVE embólico foi uma causa de óbito minoritária nesta coorte, sendo associada a menor grau de acometimento miocárdio.

Valor prognóstico independente da taquicardia ventricular não-sustentada na fase crônica da Doença de Chagas / Independent prognostic value of nonsustained ventricular tachycardia in the chronic phase of Chagas' disease

Fundamentos: o valor prognóstico da taquicardia ventricular não sustentada (TVNS) na fase crônica da doença de Chagas (DC) permanece indefinido. Objetivo: avaliar o valor prognóstico da TVNS na fase crônica da DC. Métodos: estudo observacional de coorte constituída por 262 pacientes portadores de DC submetidos à avaliação clínica, ECG, ecocardiograma, RX de tórax e Holter de 24 horas, no período de março de 1990 a dezembro de 2003. Análise...