Thermoregulatory Instability in Childhood: Linking the Normal Brain to Hypothalamic Storm.

Central core temperature is tightly controlled by hypothalamic centers, a feature that makes sudden changes in body temperature very unusual. A dysfunction of these hypothalamic pathways leads to Shapiro's syndrome, comprising spontaneous hypothermia, hyperhidrosis, and corpus callosum dysgenesis. Although it may affect any age, usually it presents in childhood. Variants to this syndrome with completely normal brain anatomy have been consistently reported, expanding the clinical spectrum of the syndrome. Herein, we report the case of a 4-year-old girl with Shapiro's syndrome and unaffected corpus callosum.

Holmes' tremor as a delayed complication of thalamic stroke.

Movement disorders are not commonly associated with stroke. Accordingly, thalamic strokes have rarely been associated with tremor, pseudo-athetosis and dystonic postures. We present a 75-year-old man who developed a disabling tremor 1 year after a posterolateral thalamic stroke. This tremor had low frequency (3-4 Hz), did not disappear on focus and was exacerbated by maintaining a static posture and on target pursuit, which made it very difficult to perform basic functions. MRI demonstrated an old ischemic lesion at the left posterolateral thalamus. Treatment with levodopa led to symptom control. Lesions in the midbrain, cerebellum and thalamus may cause Holmes' tremor. Delayed onset of symptoms is usually seen, sometimes appearing 2 years after the original injury. This may be due to maturation of a complex neuronal network, leading to slow dopaminergic denervation. Further studies are needed to improve our understanding of this unique disconnection syndrome.

Comparative analysis of the accuracy of urinary hCG tests in vitro.

OBJECTIVE: To identify whether cutoff for sensitivity advertised by three pregnancy tests in urine are compatible to those reported by the manufacturer and to describe their diagnostic performance. METHODS: The urine of a male volunteer was used to dilute recombinant ß-hCG at defined concentrations of 0, 6.25, 12.5, 25, 50, and 100 mIU/mL. The tubes containing each of the concentrations were coded and blindly assessed for positivity in three different lots of hCG tests: Strip Test Plus®, BioEasy®, and Visitect Pregnancy®. The sample size was calculated for an alpha error of 5%, with a power of 99%. RESULTS: All three brands, in their three lots analyzed, had 100% of sensitivity for detecting ß-hCG, with 100% negative predictive value, using only negative controls and samples with concentrations equal or higher than the test cutoff (n = 180/brand). The accuracy of the tests was 83% (BioEasy®), 84%(Visitect®) and 91% (Strip Test Plus®). Strip Test Plus® had the best positive likelihood ratio (52.5), while Visitect® had the best negative likelihood ratio (zero). CONCLUSION: The three brands have adequate sensitivity for the advertised cutoffs. The Strip Test Plus® test had the best performance to identify urinary concentrations of ß-hCG > 12.5 mIU/mL, and consequently, to confirm pregnancy, while Visitect® had the best performance to exclude ß-hCG in urine (negative post-test probability: zero).

Análise comparativa da acurácia in vitro de testes de detecção de hCG urinário / Comparative analysis of the accuracy of urinary hCG tests in vitro

OBJETIVO: Identificar se os pontos de corte de sensibilidade anunciados por três testes de gravidez na urina são compatíveis com os anunciados pelo fabricante e descrever os seus desempenhos diagnósticos. MÉTODOS: A urina de um voluntário masculino foi usada para diluir β-hCG recombinante em concentrações definidas de 0; 6,25; 12,5; 25; 50; e 100 mUI/mL. As amostras foram codificadas e cegamente analisadas para a positividade em três diferentes lotes dos testes hCG Strip Test Plus®, BioEasy® e Visitect Pregnancy®. O tamanho da amostra foi calculado para um erro alfa de 5 por cento, com um poder de 99 por cento. RESULTADOS: As três marcas apresentaram sensibilidade de 100 por cento na detecção do β-hCG nos três lotes analisados, com 100 por cento de valor preditivo negativo, usando somente controles negativos e amostras com concentrações iguais ou superiores ao limite do teste (n = 180/marca). A acurácia dos testes foi 83 por cento (BioEasy®), 84 por cento (Visitect®) e 91 por cento (Strip Test Plus®). O Strip Test Plus® apresentou o melhor desempenho para a razão de probabilidade positiva (52,5), enquanto que o produto Visitect® teve a melhor razão de probabilidade negativa (zero). CONCLUSÃO: Os três produtos analisados têm a sensibilidade dos pontos de corte anunciados. O produto Strip Test Plus® tem o melhor desempenho para identificar concentrações urinárias de β-hCG > 12,5 mUI/mL, consequentemente, confirmando gravidez, enquanto que o Visitect® tem o melhor desempenho para descartar a presença de β-hCG na urina (probabilidade pós-teste negativo: zero).

OBJECTIVE: To identify whether cutoff for sensitivity advertised by three pregnancy tests in urine are compatible to those reported by the manufacturer and to describe their diagnostic performance. METHODS: The urine of a male volunteer was used to dilute recombinant β-hCG at defined concentrations of 0, 6.25, 12.5, 25, 50, and 100 mIU/mL. The tubes containing each of the concentrations were coded and blindly assessed for positivity in three different lots of hCG tests: Strip Test Plus®, BioEasy®, and Visitect Pregnancy®. The sample size was calculated for an alpha error of 5 percent, with a power of 99 percent. RESULTS: All three brands, in their three lots analyzed, had 100 percent of sensitivity for detecting β-hCG, with 100 percent negative predictive value, using only negative controls and samples with concentrations equal or higher than the test cutoff (n = 180/brand). The accuracy of the tests was 83 percent (BioEasy®), 84 percent(Visitect®) and 91 percent (Strip Test Plus®). Strip Test Plus® had the best positive likelihood ratio (52.5), while Visitect® had the best negative likelihood ratio (zero). CONCLUSION: The three brands have adequate sensitivity for the advertised cutoffs. The Strip Test Plus® test had the best performance to identify urinary concentrations of β-hCG > 12.5 mIU/mL, and consequently, to confirm pregnancy, while Visitect® had the best performance to exclude β-hCG in urine (negative post-test probability: zero).

Prospective, randomized comparison between raloxifene and clomiphene citrate for ovulation induction in polycystic ovary syndrome.

OBJECTIVE: To compare the ovulation rate between raloxifene and clomiphene citrate (CC) in patients with polycystic ovary syndrome (PCOS). DESIGN: Double-blind, randomized, superiority clinical trial. SETTING: Tertiary university hospital. PATIENT(S): Women with ovulatory dysfunction and PCOS based on the Rotterdam criteria. INTERVENTION(S): One of two oral treatments: 5 days of 100 mg/day of CC or R. MAIN OUTCOME MEASURE(S): Ovulation, based on follicle collapse on serial ultrasound and midsecretory serum progesterone concentration (≥3 ng/dL). RESULT(S): The women with PCOS (n = 82) were randomized to receive CC (n = 40) or raloxifene (n = 42). From these, 68 patients finished the trial according to the protocol (CC: n = 37; raloxifene: n = 31). There were no statistically significant differences between the groups in ovulation rates per an intention-to-treat analysis based on ultrasound alone (CC: 21 of 40 vs. raloxifene: 17 of 42) or on progesterone levels (CC: 16 of 40 vs. raloxifene: 11 of 42). No serious adverse events were observed in either group. CONCLUSION(S): No statistically significant difference in ovulation was observed between raloxifene and clomiphene citrate in patients with PCOS with ovulatory dysfunction.

Restless legs syndrome and multiple sclerosis: a Brazilian multicenter study and meta-analysis of the literature.

OBJECTIVE: The increased prevalence of restless legs syndrome (RLS) in multiple sclerosis (MS) has recently been the subject of a few papers. The present study investigated the prevalence of RLS symptoms in MS patients and in controls in four Brazilian cities. Additionally, a systematic review and meta-analysis of the literature was carried out on the subject of RLS-MS. METHOD: MS patients and controls were investigated regarding the presence of the four typical symptoms of RLS. A questionnaire assessing RLS severity was also used for patients and controls presenting the four RLS symptoms criteria. The systematic review and meta-analysis on the subject were carried out according to the strict international criteria. RESULTS: In the present report, the RLS-MS association was confirmed as being more than fortuitous in Brazilian MS patients, in a multicenter case-control study. MS patients also presented RLS symptoms of greater severity than did the control population. A systematic review and meta-analysis of the literature showed that MS patients had a fourfold higher chance of presenting RLS than did the controls. CONCLUSION: Although underlying mechanisms to explain the association RLS-MS are still a matter of discussion, there is a clear association of these two neurological conditions.

Restless legs syndrome and multiple sclerosis: a Brazilian multicenter study and meta-analysis of the literature / Síndrome das pernas inquietas e esclerose múltipla: um estudo brasileiro multicêntrico e metanálise da literatura

OBJECTIVE: The increased prevalence of restless legs syndrome (RLS) in multiple sclerosis (MS) has recently been the subject of a few papers. The present study investigated the prevalence of RLS symptoms in MS patients and in controls in four Brazilian cities. Additionally, a systematic review and meta-analysis of the literature was carried out on the subject of RLS-MS. METHOD: MS patients and controls were investigated regarding the presence of the four typical symptoms of RLS. A questionnaire assessing RLS severity was also used for patients and controls presenting the four RLS symptoms criteria. The systematic review and meta-analysis on the subject were carried out according to the strict international criteria. RESULTS: In the present report, the RLS-MS association was confirmed as being more than fortuitous in Brazilian MS patients, in a multicenter case-control study. MS patients also presented RLS symptoms of greater severity than did the control population. A systematic review and meta-analysis of the literature showed that MS patients had a fourfold higher chance of presenting RLS than did the controls. CONCLUSION: Although underlying mechanisms to explain the association RLS-MS are still a matter of discussion, there is a clear association of these two neurological conditions.

OBJETIVO: A maior prevalência de síndrome das pernas inquietas (SPI) em esclerose múltipla (EM) foi recentemente assunto de algumas publicações. O presente trabalho investigou a prevalência de sintomas de SPI em pacientes com EM e em controles em quatro cidades brasileiras. Além disto, uma revisão sistemática da literatura e metanálise dos dados foi feita considerando o tema SPI-EM. MÉTODO: Pacientes com EM e controles foram investigados com relação aos quarto sintomas típicos de SPI. Um questionário avaliando a intensidade da SPI também foi utilizado para pacientes e controles que apresentassem os quatro sintomas para critério diagnóstico de SPI. A revisão sistemática e meta-análise do tema foram realizadas de acordo com os rígidos critérios internacionais. RESULTADOS: No presente estudo, a associação de SPI-EM foi confirmada como sendo mais que coincidência nos pacientes brasileiros com EM, através do estudo caso-controle multicêntrico. Os pacientes com EM também apresentaram maior gravidade dos sintomas de SPI que os controles. A revisão sistemática e metanálise da literatura mostraram que os pacientes com EM tiveram quatro vezes mais chance de apresentar SPI do que controles. CONCLUSÃO: Embora os mecanismos envolvidos na associação SPI-EM ainda sejam motivo de discussão, existe uma clara associação entre estas duas condições neurológicas.

The influence of hydrosalpinx on endometrial elafin expression.

The endometrium of women with hydrosalpinx has an increased number of neutrophils and lower expression of elafin, an elastase inhibitor and natural antimicrobial molecule. These findings suggest that women with hydrosalpinx have a reduced antimicrobial and antielastase activity.

Are antibiotics necessary after 48 hours of improvement in infected/septic abortions? A randomized controlled trial followed by a cohort study.

OBJECTIVE: We sought to investigate whether oral antibiotics are necessary, after 48 hours of clinical improvement, in uncomplicated septic abortion. STUDY DESIGN: In a randomized double-blind clinical trial, 56 women with uncomplicated septic abortion were treated with intravenous antibiotics, followed by uterine evacuation. On hospital discharge (day 1), patients were randomized to receive either oral doxycycline plus metronidazole or placebo, until completing 10 days of treatment. Clinical cure was defined by the absence of fever (<37.7°C), reduced vaginal bleeding, and minimal or no pelvic pain. RESULTS: Cure was observed in all 56 patients. The institutional review board stopped the treatment arm as it was adding risk with no further benefit to the patients. An observational cohort with additional 75 cases was followed up in the no treatment arm and no failure was identified (probability of an adverse event, 0%; 95% confidence interval, 0-0.03). CONCLUSION: After 48 hours of clinical improvement, antibiotics may not be necessary.

Anatomia microcirúrgica e abordagens ao seio cavernoso: um estudo tridimensional estereoscópico com correlação clínico-radiológica / Microsurgical anatomy and approaches to the cavernous sinus: an stereoscopic 3-D study with clinical-radiological correlations

O seio cavernoso é uma das regiões mais complexas do corpo humano e pode ser acometido por diversas patologias. Atualmente,diversos tipos de tratamento podem ser usados para tratar doenças nesta região, porém o conhecimento anatômico ainda é fundamental para estabelecer o melhor manejo. Nosso objetivo é apresentar a anatomia microcirúrgica do seio cavernoso,as abordagens cirúrgicas (incluindo a abordagem endonasalendoscópica), a correlação radiológica desta anatomia,bem como uma série inicial de casos. As fotos são apresentadas pelo método convencional e anaglífico estereoscópico. São descritas as estruturas neuro vasculares do seio cavernoso bem como suas relações ósseas e durais, as paredes e os triângulos da base do crânio relacionados ao seio cavernoso. As abordagens crânio-órbito zigomática, transpetrosa, zigomática e endonasal endoscópica são apresentadas, sendo as estruturas anatômicas identificadas também nos exames de imagem. Casos ilustrativos ilustram esta anatomia.

The cavernous sinus is one of the most complex regions of thehuman body and can be affected by several diseases. Currently,several types of treatment can be used in the management ofdiseases in this region. The anatomical knowledge is essentialto establish the best management. Our goal is to present themicrosurgical anatomy of the cavernous sinus, its surgical approaches(including the endoscopic endonasal approach), thecorrelation of radiological anatomy, including an initial seriesof cases. The anatomic features are presented by the conventionaland stereoscopic anaglyphic method. The neurovascularstructures of the cavernous sinus and its dural and bony relationships,triangles and the walls of the skull base related toit are presented with. We discuss the cranio-orbital zygomatic,transpetrosal, zygomatic and endonasal endoscopic approaches;MRI studies also show the anatomical structures, includingillustrative cases.

Does bupivacaine in laparoscopic ports reduce postsurgery pain in tubal ligation by electrocoagulation? A randomized controlled trial.

BACKGROUND: Tubal ligation (TL) is the most popular method of permanent contraception. In order to reduce postoperative pain, different analgesic techniques have been proposed. The objective of this study was to compare the level of postoperative pain in patients submitted to TL with electrocoagulation, under general anesthesia, using bupivacaine infiltration vs. placebo in trocar ports. STUDY DESIGN: Consecutive patients scheduled for laparoscopic TL were randomized by sequenced coded envelopes to receive bupivacaine 0.5% (n=29) or placebo (n=24). Pain was blindly assessed at 15 min, 30 min, 120 min and 14 h postoperatively, by verbal analogue scale (VAS). Standard pain medications (morphine, dipyrone and sodium diclofenac) were prescribed for the subjects and compared between groups. RESULTS: No difference in pain assessment was found between bupivacaine and placebo groups at all times [median (25-75 quartiles)] (all p>.05): 15 min: 3 (1-6.3) vs. 4 (0-7); 30 min: 1.5 (0-4.3) vs. 2 (0-5); 2 h: 0 (0-0.5) vs. 0 (0-1); 14 h: 1 (0-4) vs. 0 (0-4); and for use of analgesics: dipyrone (g): 1 (0-1) vs. 1 (0-1); morphine (mg): 3 (0-3) vs. 3 (0-3.5); sodium diclofenac (mg): 0 (0-50) vs. 0 (0-50). CONCLUSION: The use of local injection of bupivacaine 0.5% in the trocar ports was not superior to placebo to reduce pain after laparoscopic TL with electrocoagulation under general anesthesia.

Clinical features of multiple sclerosis in the south of Brazil: a partial analysis / Características clínicas da esclerose múltipla no sul do Brasil: uma análise parcial

There are no published studies on the characteristics of multiple sclerosis (MS) patients from the south of Brazil. OBJECTIVE: To identify the clinical features of a series of MS patients and to compare to other Brazilian series. METHOD: Retrospective study with 67 patients followed in The MS Reference Center - RS, Brazil during the year of 2008. We analyzed demographic and clinical data. RESULTS: Most were women (74.6 percent), the general average age was 43.5 years old, and the general average EDSS score was 4.1. Of those patients, 81.8 percent had relapsing-remitting MS. Sexual dysfunction prevalence was 31.1 percent in men and 68.9 percent in women (p<0.01). We found a positive correlation (Spearman=0.444, p<0.05) between EDSS and depressive symptoms. CONCLUSION: This study showed a very similar sample compared to other states of Brazil. Moreover, there was found a high prevalence of sexual dysfunction and a straight relation between EDSS and depressive symptoms.

Não há dados publicados sobre as características clínicas da esclerose múltipla (EM) no estado do Rio Grande do Sul (RS). OBJETIVO: Identificar e comparar as características clínicas de uma série de pacientes com EM no RS com as de outras séries nacionais. MÉTODO: Foram coletados e avaliados dados demográficos e clínicos de 67 pacientes atendidos em nosso centro. RESULTADOS: Houve predomínio de mulheres (74,6 por cento), idade média geral foi de 43,5 anos e EDSS médio de 4,1. O tipo surto-remissão correpondeu a 81,8 por cento. A prevalência de disfunção sexual foi de 31,1 por cento nos homens e 68,9 por cento nas mulheres (p<0,01). Encontrou-se correlação de 0,444 (p<0,05) entre depressão e EDSS. CONCLUSÃO: Este estudo demonstrou uma amostra semelhante às demais amostras brasileiras, apresentando, adicionalmente, elevada prevalência de sintomas sexuais e a estreita associação entre depressão e o grau de incapacidade.

Clinical features of multiple sclerosis in the south of Brazil: a partial analysis.

UNLABELLED: There are no published studies on the characteristics of multiple sclerosis (MS) patients from the south of Brazil. OBJECTIVE: To identify the clinical features of a series of MS patients and to compare to other Brazilian series. METHOD: Retrospective study with 67 patients followed in The MS Reference Center - RS, Brazil during the year of 2008. We analyzed demographic and clinical data. RESULTS: Most were women (74.6%), the general average age was 43.5 years old, and the general average EDSS score was 4.1. Of those patients, 81.8% had relapsing-remitting MS. Sexual dysfunction prevalence was 31.1% in men and 68.9% in women (p<0.01). We found a positive correlation (Spearman=0.444, p<0.05) between EDSS and depressive symptoms. CONCLUSION: This study showed a very similar sample compared to other states of Brazil. Moreover, there was found a high prevalence of sexual dysfunction and a straight relation between EDSS and depressive symptoms.

Encaminhamentos da atenção primária para avaliação neurológica em Porto Alegre, Brasil / Referrals from primary care to neurological assessment in the city of Porto Alegre, Brazil

No Sistema Único de Saúde do Brasil (SUS), os níveis de atenção à saúde se inserem no modelo hierárquico através do sistema de referência e contrarreferência. Em um projeto para atender às demandas represadas do nível primário ao secundário, denominado "Mutirão da Saúde", realizado em Porto Alegre, os médicos neurologistas do Hospital de Clínicas de Porto Alegre, após cada atendimento realizado, responderam a um instrumento para avaliar os encaminhamentos realizados pela rede de atenção primária à saúde (APS). A avaliação foi positiva, mostrando que os encaminhamentos foram realmente necessários em 85 por cento dos casos; entretanto, foi parcialmente contraditória, tendo em vista que 41,7 por cento dos encaminhamentos eram situações clínicas que deveriam ser manejadas no atendimento primário. A avaliação também revelou que 50 por cento dos casos necessitavam de exames complementares. Devido a uma possível regionalização aleatória dos encaminhamentos, o resultado não nos possibilitou uma estimativa apropriada da prevalência por territórios das unidades de APS, informação esta importante para a organização do fluxo de encaminhamentos e planejamento dos recursos alocados pelos gestores, tanto locais quanto municipais.

In the Unified Health System in Brazil (SUS), the levels of health care fall within the hierarchical model with the reference and counter-reference systems. In a project to meet the repressed demands of primary and secondary levels, called "Mutirão da Saúde", held in Porto Alegre, the neurologists of the Hospital de Clinicas de Porto Alegre, after each call made, answered a questionnaire to assess referrals performed by the network of primary health care (PHC). The evaluation was positive, showing that the referrals were really needed in 85 percent of cases; however, it was partially contradictory, given that 41.7 percent of referrals were medical conditions that should be managed in primary care. The evaluation also revealed that 50 percent of cases needed further investigation. Due to a possible randomized regionalization of referrals, the result did not allow us to estimate the appropriate prevalence according to areas of the PHC units, information that is important to organize the flow of referrals and planning of resources allocated by managers, both local and municipal.