IL-6 levels are associated with clinical status in patients with Myasthenia Gravis / Os níveis de IL-6 estão associados com o status clínico de pacientes com Miastenia Gravis

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease marked by fluctuating course of muscle weakness. OBJECTIVES: The current study was designed to evaluate plasma levels of cytokines (IL-2, IL-4, IL-6, IL-10, TNF, IFN-γ, and IL17A) in patients with MG and controls and to investigate whether cytokines levels are associated with clinical parameters. This study was conducted at the Neuromuscular Diseases Outpatient Clinic, Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Brazil. METHODS: Peripheral blood was drawn, and plasma levels of cytokines were measured by cytometric bead array (CBA) in 80 treated patients with MG and 50 controls. The MG Composite (MGC) was used to evaluate muscle weakness and severity of typical motor symptoms of MG. RESULTS: Patients with MG undergoing treatment exhibit lower levels of all evaluated cytokines compared to controls. There was a negative correlation between IL-6 levels and the MG Composite score, indicating that higher levels of IL-6 were associated with better control of the disease. CONCLUSION: This exploratory study suggests that IL-6 is associated with MG clinical status, as assessed by the MGC.

INTRODUÇÃO: A Miastenia Gravis (MG) é uma doença autoimune caracterizada por fraqueza muscular flutuante. OBJETIVOS: avaliar os níveis plasmáticos de citocinas (IL-2, IL-4, IL-6, IL-10, TNF, IFN-γ, e IL-17A) em pacientes com MG e controles e investigar se essas citocinas estão associadas com parâmetros clínicos. Este estudo foi conduzido no ambulatório de doenças neuromusculares do Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG), Brasil. MÉTODOS: Foi coletado sangue periféricos e os níveis plasmáticos das citocinas foram medidos por citometria em 80 pacientes com MG tratados e em 50 controles. O MG composite (MGC) foi utilizado para avaliar a fraqueza muscular e a gravidade dos sintomas motores típicos da MG. RESULTADOS: Os pacientes com MG em tratamento apresentaram menores níveis de todas as citocinas avaliadas comparados ao controle. Houve uma correlação negativa entre os níveis de IL-6 e o MGC, indicando que altos níveis de IL-6 estão associados com melhor controle da doença. CONCLUSÃO: este estudo exploratório sugere que a IL-6 está associada com o status clínico da MG, quando avaliado pelo MGC.

Altered Serum Levels of Renin-Angiotensin System Markers in Migraine.

OBJECTIVE: To compare the serum levels of renin-angiotensin system (RAS) components between patients with migraine and healthy controls, and to evaluate whether these levels are associated with migraine severity. We hypothesized that migraine would be associated with the activation of the inflammatory arm of the RAS, possibly leading to increased levels of angiotensin (Ang) II. BACKGROUND: Recent studies have proposed the use of drugs that interfere with RAS, a hormonal system primarily implicated in blood pressure regulation, as a prophylactic strategy for migraine. However, no previous studies have directly assessed RAS components in migraine. METHODS: This was a cross-sectional study involving 30 patients with episodic migraine who were in the interictal period and 20 healthy controls. This study was conducted at Hospital das Clínicas (Universidade Federal de Minas Gerais, Belo Horizonte, Brazil) outpatient clinic. Headache severity was evaluated using the Headache Impact Test, version 6 (HIT-6) and the Migraine Disability Test (MIDAS) questionnaires. Given that migraine is comorbid with mood disorders, depressive and anxious symptoms were evaluated using the Beck Anxiety and Depression Inventories (BDI and BAI), respectively. Clinical and demographic data were also collected. Serum levels of angiotensin-converting enzyme (ACE), ACE2, Ang II, and Ang (1-7) were measured by enzyme-linked immunosorbent assay. RESULTS: Patients with migraine and controls were comparable in age, body mass index, blood pressure, and depressive and anxious symptoms. Patients with migraine showed lower levels of ACE [85.2 (66.8, 101.2) vs 65.5 (54.2, 77.5); P = .005] and lower ACE/ACE2 ratio [4.3 (3.4, 5.2) vs 3.5 (2.9, 4.1); P = .032] than controls. Conversely, patients with migraine had higher levels of Ang II [309.7 ± 147.4 vs 605.4 ± 200.4; difference: -287.1 (95% CI: -391.4--182.8), P < .001] and Ang (1-7) [214.4 ± 155.8 vs 397.9 ± 217.9; difference: -184.6 (95% CI: -296.7--72.6), P = .001] than controls. There were no correlations between RAS serum markers and migraine severity scores (HIT and MIDAS) or depressive and anxious symptoms (BDI and BAI) (P > .05). CONCLUSIONS: Altogether, our results suggest the participation of RAS in migraine pathophysiology, but not in its severity.

Double-blind placebo-controlled randomized clinical trial of ginger (Zingiber officinale Rosc.) in the prophylactic treatment of migraine.

BACKGROUND: Previous studies have shown an analgesic effect of ginger in the acute treatment of migraine, and there is anecdotal evidence of its efficacy in migraine prophylaxis. OBJECTIVE: This study aimed to evaluate the potential of ginger to prevent migraine attacks. METHODS: This double-blind, placebo-controlled randomized clinical trial took place at the Headache Clinic, Universidade Federal de Minas Gerais (Belo Horizonte, Minas Gerais, Brazil), involving 107 patients. Only subjects diagnosed with episodic migraine, aged between 18 and 60 years old, and who were not taking any prophylactic medication, were enrolled in the study. After one month of observation, subjects selected for the study were randomized 1:1 into placebo and treatment groups. Patients received capsules three times per day of 200 mg of dry extract of ginger (5% active ingredient) or placebo (cellulose) for three months. Visits were performed monthly and the patients were asked to fill in a migraine diary. The adherence to treatment was evaluated by counting capsules. RESULTS: The percentage of patients who responded to treatment (i.e. a reduction of 50% in the number of migraine attacks at the end of treatment) did not differ between the groups. There was a decrease in the number of days with severe pain, analgesic use for acute migraine and duration of migraine attacks in both groups, without significant difference between ginger and placebo groups. CONCLUSIONS: Ginger provides no greater benefit in the prophylactic treatment of migraine when compared to placebo. This trial is registered at ClinicalTrials.gov (NCT02570633).

Hospital-acquired intestinal toxemia botulism in a newly diagnosed adult colon cancer patient.

This manuscript reports a case of intestinal toxemia botulism in an adult with recently diagnosed metastatic colon cancer in whom botulism symptoms began 23 days after hospital admission. Representing the rarest form of botulism presentation in clinical practice, this infectious disease may have developed due to a cluster of predisposing factors that favored Clostridium botulinum colonization and the endogenous production of neurotoxins, among which are previous use of broad-spectrum antibiotics and colon changes related to the development of the neoplasia. This case highlights the importance of considering intestinal toxemia botulism in the differential diagnosis of a patient presenting with symmetrical descending flaccid paralysis, since immediate treatment with botulinum antitoxin may improve clinical outcomes.

Personality disorders are associated with more severe forms of migraine.

To investigate the clinical manifestation, disease course, and prognosis of migraine patients with or without personality disorders. This cross-sectional study evaluated 61 patients with migraine diagnosed according to the criteria of the International Headache Society (IHS). Personality disorders were assessed with the Structured Clinical Interview for DSM-IV (SCID-II). Migraine severity was assessed with the Headache Impact Test-6 (HIT-6). We also used a structured clinical interview to diagnose comorbid mood disorders. Of the 61 patients, 20 (32.8%) had personality disorders. Personality disorders included obsessive-compulsive 14/61 (23.0%), avoidant 6/61 (9.8%), borderline 6/61 (9.8%), paranoid 6/61 (9.8%), schizoid 2/61 (3.3%), histrionic 1/61 (1.6%) and dependent 1/61 (1.6%) types. Compared to migraine patients without personality disorders, comorbidity with any personality disorders was associated with an increased frequency of chronic migraine (p < 0.001) and more severe headache as assessed by the HIT-6 (p < 0.001). Comorbidity with personality disorders was associated with more severe forms of migraine symptoms.

Evolução Clínica e Preditores da Neuropatia Periférica Induzida por Quimioterapia / Clinical Evolution and Predictors of Chemotherapy-Induced Peripheral Neuropathy / Evolución Clínica y Predictores de la Neuropatía Periférica Inducida por Quimioterapia

Introdução: Drogas antineoplásicas neurotóxicas estão frequentemente associadas à neuropatia periférica induzida por quimioterapia (NPIQ). Objetivo: Avaliar a evolução clínica dos pacientes expostos a tratamento antineoplásico potencialmente neurotóxico e identificar possíveis preditores clínicos e sociodemográficos para o desenvolvimento da NPIQ. Método: Estudo de coorte prospectiva com pacientes com diagnóstico de câncer de mama, ovário ou intestino em tratamento quimioterápico com paclitaxel, docetaxel ou oxaliplatina. Foram avaliados antes da quimioterapia (T1), no terceiro mês (T2) e 30-60 dias após interrupção do tratamento (T3). Todos responderam ao questionário de perfis sociodemográfico e clínico, foram avaliados por meio de exame clínico neurológico, pela escala de performance ECOG, escala hospitalar de ansiedade e depressão (HAD), escala de dor Short-cGuill, autorrelato de sintomas de NPIQ e avaliação com o questionário de neurotoxicidade induzida por antineoplásicos (CINQ). Resultados: Por meio de autorrelato, 75% da dos pacientes informaram apresentar sintomas de NPIQ. O CINQ evidenciou que 90% apresentaram algum grau de NPIQ em T2, enquanto 82,5% ainda persistiam em T3. Dor neuropática acometeu 42% da população (RR=1,429; IC95%=1,130-1,806). Os escores de ansiedade e depressão reduziram significativamente quando comparados ao início de tratamento (redução de 2,5 pontos na escala HAD, p<0,05). A capacidade funcional da população não mostrou alterações significativas. No T2, a escolaridade foi considerada preditora para autorrelato de sintomas de NPIQ (OR=1,314, IC95%=1,002-1,723, p=0,048). Conclusão:A baixa escolaridade pode comprometer a capacidade do paciente em relatar os sintomas da NPIQ. Este estudo chama a atenção para a necessidade de utilização de instrumentos específicos para detecção precoce da NPIQ.

Introduction: Neurotoxic antineoplastic drugs are frequently associated to chemotherapy-induced peripheral neuropathy (CIPN). Objective: To evaluate the clinical evolution of patients exposed to potentially neurotoxic antineoplastic treatment and to identify possible clinical and sociodemographic predictors for the development of CIPN. Method: Cohort prospective study with patients with breast, ovary or intestine diagnosis of cancer in chemotherapy treatment with paclitaxel, docetaxel or oxaliplatin. They were assessed before the chemotherapy (T1), in the third month (T2) and 30-60 days after the interruption of the treatment (T3). All the patients responded to the questionnaire of clinical and sociodemographic profiles, were evaluated through neurologic clinical exam, by the performance scale ECOG, by the Hospital Anxiety and Depression Scale - HAD, pain scale of Short-cGuill, self-report of symptoms of CIPN and evaluation with the questionnaire of antineoplastic-induced neurotoxicity (QAIN). Results: Through self-report, 75% of the patients presented symptoms of CIPN. The QAIN showed that 90% presented a certain degree of CIPN in T2, while 82.5% still persisted in T3. Neuropathic pain affected 42% of the population (RR = 1.429, CI95% = 1.130-1.806). Anxiety and depression scores significantly reduced when compared with the beginning of the treatment (reduction of 2.5 points in the scale HAD, p < 0.05). The functional capacity of the population did not show any significant change. The school level was considered a predictor of self-report of CIPN symptoms in T2 (OR = 1.314, CI95% = 1.002-1.723, p = 0.048). Conclusion: The low school level may taint the patient capacity to report CIPN symptoms. This study draws attention for the necessity to use specific instruments for early detection of CIPN.

Introducción: Los fármacos antineoplásicos neurotóxicos a menudo se asocian con neuropatía periférica inducida por quimioterapia (CIPN). Objetivo: Evaluar la evolución clínica de pacientes expuestos a tratamientos antineoplásicos potencialmente neurotóxicos e identificar posibles predictores clínicos y sociodemográficos para el desarrollo de CIPN. Método: Estudio de cohorte prospectivo con pacientes diagnosticadas con cáncer de mama, ovario o intestino sometidos a quimioterapia con paclitaxel, docetaxel u oxaliplatino. Se evaluaron antes de la quimioterapia (T1), en el tercer mes (T2) y 30-60 días después de la interrupción del tratamiento (T3). Todos respondieron el cuestionario de perfil sociodemográfico y clínico, se evaluaron mediante un examen neurológico clínico, la escala de rendimiento ECOG, la escala de ansiedad y depresión hospitalaria (HAD), la escala de dolor Short-cGuill, el autoinforme de los síntomas de CIPN y la evaluación con el cuestionario de neurotoxicidad inducida por antineoplásicos (CINQ). Resultados: Por autoinforme, el 75% de la población informó presentar síntomas de CIPN. El CINQ mostró que el 90% tenía algún grado de NPIQ en T2, mientras que el 82.5% aún persistía en T3. El dolor neuropático afectó al 42% de la población (RR = 1.429; IC del 95% = 1.130-1.806). Las puntuaciones de ansiedad y depresión disminuyeron significativamente en comparación con el valor inicial (reducción de 2.5 puntos HAD, p <0.05). La capacidad funcional de la población no mostró cambios significativos. En T2, la educación se consideró un predictor de síntomas CIPN autoinformados (OR=1.314, IC 95%=1.002-1.723, p=0,048). Conclusión: La baja educación puede comprometer la capacidad del paciente para informar los síntomas de CIPN. Este estudio llama la atención sobre la necesidad de utilizar instrumentos específicos para la detección temprana de CIPN.

Longitudinal assessment of clinical and inflammatory markers in patients with amyotrophic lateral sclerosis.

OBJECTIVE: To evaluate potential associations between clinical features and inflammatory markers in patients with amyotrophic lateral sclerosis (ALS). METHODS: A consecutive series of 68 patients (39 males and 29 females) with sporadic ALS were subjected to a comprehensive clinical assessment and blood draw. A subset of these patients underwent a new assessment within 6-12 months after the baseline visit. In addition, a group of 62 subjects composed by age and sex-matched healthy subjects (38 males and 24 females) was enrolled in this study. Peripheral blood was drawn and plasma levels of chemokines and cytokines were measured by cytometric bead array and enzyme-linked immunosorbent assay. RESULTS: Our sample was composed by patients with ALS with an average age of 58 (±12.3) years old and 3 (±2.7) years of disease length at the baseline visit. Patients with ALS presented increased plasma levels of interleukin (IL)-6 and IL-8 in comparison with controls. After multivariate analysis, higher levels of IL-6 and lower levels of IL-2 were significantly associated with increased likelihood of ALS diagnosis. When evaluating the subset of patients assessed longitudinally, we did not find any significant difference in the levels of inflammatory markers between the two time points. Older age at ALS onset was the only factor associated with a faster rate of disease progression. CONCLUSIONS: IL-6 levels could discriminate between ALS and controls and may be regarded as a potential biomarker of ALS diagnosis. An increase in IL-2 levels was associated with a protective effect on the odds of ALS diagnosis. Older age at ALS onset predicted a fast rate of disease progression.

Muscle strength and psychiatric symptoms influence health-related quality of life in patients with myasthenia gravis.

Myasthenia gravis (MG) is a neuromuscular autoimmune disease characterized by skeletal muscle weakness which can impact motor function and, furthermore, produce negative impact on the health-related quality of life (HRQOL). OBJECTIVE: To evaluate the predictors for HRQOL in patients with MG. METHODS: Eighty patients were evaluated with the MG Foundation of America classification and the MG Composite scale. HRQOL was estimated by the MGQOL15, while anxious and depressive symptoms were evaluated with the Hospital Anxiety and Depression Scale (HAD). RESULTS: The mean age of patients was 41.9 years with mean illness duration of 13.5 years. Almost half of the patients (43.75%) had significant anxiety and more than a quarter (27.50%) had depressive symptoms. Factors that influenced the HRQOL in MG were skeletal muscle weakness and anxiety and depressive symptoms (p < .001 in logistic regression model). CONCLUSION: Anxiety and depressive symptoms, besides motor symptoms, influence HRQOL in MG. Mental health must be a clinical focus in addition to the treatment of somatic symptoms during the course of MG.

Características Clínicolaboratoriais da Criptococose em Pacientes Admitidos em Pronto Socorro Hospital das Clínicas, Universidade Federal de Minas Gerais, Referência Terciária do Sistema Único de Saúde: Análise Retrospectiva desde 2000 a 2013 / Clinical Laboratory Characteristics of Cryptococcosis in Patients Admitted to the Emergency Room Hospital das Clínicas, Federal University of Minas Gerais, Tertiary Reference of the Unified Health System: Retrospective Analysis from 2000 to 2013

A criptococose é uma micose sistêmica, de ocorrência relativamente rara, potencialmente grave, geralmente oportunista e de elevada frequência em pacientes imunossuprimidos, com amplo espectro de acometimento de órgãos, tropismo especial para o sistema nervoso central (SNC), evolução subaguda ou crônica, e manifestações clínicas variadas. Este estudo descritivo, retrospectivo, observacional, transversal, objetivou descrever os dados demográficos, clínicos, comorbidades, sintomas ou sinais, e o prognóstico de pacientes com neurocriptococose, atendidos e internados no Hospital das Clínicas (HC) da Universidade Federal de Minas Gerais desde 2000 até 2013. O HC é unidade universitária, pública e geral, de nível terciário e quaternário, com 450 leitos de internação, integrado ao Sistema Único de Saúde (SUS), com clientela universalizada, cerca de 40% do total proveniente do interior do estado de Minas Gerais, predominando da região Metropolitana de Belo Horizonte, aberto à transferência de pacientes de todo o território mineiro, com área de abrangência de população de mais de cinco milhões de pessoas, de todas as faixas etárias e todas as especialidades médicas, encaminhados pela intensidade de sua expressão clínica, especialmente em situação crítica, o que torna sua casuísticade máxima gravidade. Os pacientes foram internados a partir do Pronto Socorro do HC que admite, em média, 80 pacientes com urgência clínicas por dia, incluindo obstétricas, e excluídas aquelas devido à acidente ou violência de qualquer natureza. Foram analisados 40 pacientes com neurocriptococose o que significou 0,13% de toda demanda de admissão de urgência para o período estudado, cerca de 603.000 pessoas, isto é 12% da população referida, e associou-se à letalidade de 25%; com frequência da distribuição de acordo com o gênero em 2:1, entre homens e mulheres, respectivamente; e nas faixas etárias entre 20-40, 40-60 e mais de 60 anos de idade, de 36%, 42%, e 22%, respectivamente, sendo a proporção entre 20 a 60 e mais de 60 de aproximadamente, 2:1. A neurocriptococose associou-se em mais de 50% dos pacientes com a: SIDA (57,5%); internação prévia (52,5%) relacionada à quimio e corticoterapia, transplante, cirurgias para ressecção de neoplasias; e, em menos de 20% com doença cardiovascular hipertensiva sistêmica (17,5%), cirurgia prévia (15%) e tuberculose (5%). A sintomatologia isolada presente em pelo menos 40% dos pacientes foi: cefaleia (70%), astenia (50%), febre (45%), vômitos (40%); entretanto, em até um terço deles constituiu-se de: emagrecimento (30%), tontura (30%), dor abdominal (27,5%), convulsão (22,5%). As anormalidades mais e menos especificamente indicadoras de acometimento do SNC foram cefaleia; e, vômito, tontura e convulsão,respectivamente. As alterações do exame neurológico foram relacionadas aos distúrbios da consciência (35%), lesão focal (30%), alteração da marcha (25%) e distúrbio do comportamento (15%). A concomitância de cefaléia, convulsão e vômitos foi anotada em 5% dos pacientes; enquanto de cefaléia e convulsão em 22,5%. Foi observada, à admissão hospitalar, em 40%, dos pacientes a associação de cefaléia e vômito; mas todos os pacientes com vômito e também os com lesão focal apresentavam cefaleia. A presença de cefaleia não foi descrita em 35% dos pacientes com alteração da consciência à admissão hospitalar. O diagnóstico presuntivo de neurocriptococose deve ser realizado, independentemente da sintomatologia clínica neurológica, o que realça a percepção geral do paciente, incluindo epidemiologia, história familiar, história prévia, manifestações clínicas, presença de imunossupressão, para surpreender a criptococose, e iniciar a terapêutica o mais apidamente possível para que possa ser reduzida sua letalidade. A limitação deste estudo relaciona-se ao fato de ter sido retrospectivo, em que o controle dos dados registrados é muito limitada, sendo impossível corrigir a ausência de dados registrados. (AU)

Cryptococcosis is a systemic, relatively rare, potentially severe, often opportunistic and systemic mycosis in immunosuppressed patients with a broad spectrum of organ involvement, a special central nervous system (CNS) tropism, subacute or chronic clinical manifestations. This descriptive, retrospective, observational, cross-sectional study aimed to describe the demographic, clinical, comorbidities, symptoms or signs, and the prognosis of patients with neurocryptococcosis, attended and hospitalized at the Hospital das Clínicas (HC) of the Universidade Federal de Minas Gerais since 2000 until 2013. The HC is a university unit, public and general, tertiary and quaternary level, with 450 beds of hospitalization, integrated into the Unified Health System (SUS), with a universalized clientele, about 40% of the total coming from the interior of the state of Minas Gerais, predominating in the metropolitan region of Belo Horizonte, which is open to the transfer of patients from all over Minas Gerais, with an area of population of more than five million people, of all age groups and all medical specialties. intensity of its clinical expression, especially in a critical situation, which makes its series of age.The patients were hospitalized from the HC Emergency Room, which admitted, on average, 80 urgently needed clinics per day, including obstetrics, and excluded due to accidents or violence of any kind. We analyzed 40 patients with neurocryptococcosis, which represented 0.13% of all urgent admission demands for the period studied, about 603,000 people, ie 12% of the referred population, and was associated with a 25% lethality; with frequency of distribution according to gender in 2: 1, between men and women, respectively; and in the age groups between 20-40, 40-60 and over 60 years of age, of 36%, 42%, and 22% respectively, the ratio being between 20 to 60 and more than 60 of approximately 2: 1. Neurocryptococcosis was associated in more than 50% of patients with: AIDS (57.5%); previous hospitalization (52.5%) related to chemo and corticoid therapy, transplantation, surgeries for resection of neoplasias; and in less than 20% with systemic hypertensive cardiovascular disease (17.5%), previous surgery (15%) and tuberculosis (5%). The isolated symptoms present in at least 40% of the patients were: headache (70%), asthenia (50%), fever (45%), vomiting (40%); (30%), dizziness (30%), abdominal pain (27.5%), and seizure (22.5%). The most and least specific abnormalities of CNS involvement were headache; and, vomiting, dizziness and convulsion, respectively. Changes in neurological examination were related to disturbances of consciousness (35%), focal lesion (30%), gait alteration (25%) and behavior disorder (15%). The concomitance of headache, convulsion and vomiting was noted in 5% of the patients; while headache and seizure in 22.5%. The association of headache and vomiting was observed in 40% of patients; but all patients with vomiting and those with focal lesion also had headache. The presence of headache was not described in 35% of patients with altered consciousness at hospital admission. The presumptive diagnosis of neurocryptococcosis should be performed independently of the clinical neurological symptomatology, which highlights the general perception of the patient, including epidemiology, family history, previous history, clinical manifestations, presence of immunosuppression, to start cryptococcosis, and initiate therapy. as soon as possible so that their lethality can be reduced. The limitation of this study is the fact that it was retrospective, in which the control of the recorded data is very limited, and it is impossible to correct the absence of recorded data. (AU)

Body composition and adipokines plasma levels in patients with myasthenia gravis treated with high cumulative glucocorticoid dose.

This study aimed to evaluate changes in body composition, i.e. overweight, obesity, fat accumulation and low lean body mass and plasma levels of adipokines in patients with MG. The study enrolled 80 patients with MG, and 62 controls. Body fat mass and body lean mass was analyzed by dual-energy X-ray absorptiometry technique (DXA). Plasma levels of leptin were analyzed by Luminex® and adiponectin and resistin were analyzed by ELISA. The mean age of patients with MG was 41.9years, with 13.5years of length of illness, and mean cumulative dose of glucocorticoids 38,123mg. Our results showed that the frequency of obesity is higher in MG patients than in controls, and patients with MG presented higher body fat mass, android body adiposity and total body fat than controls. MG patients presented lower levels of resistin and higher levels of leptin in comparison with controls. There were no differences in the plasma levels of adiponectin. Higher total body fat and lower body lean mass were associated with increased severity of MG symptoms. This result points to the relevance of estimation of body composition in planning long-term care of MG patients.

Negative impact of high cumulative glucocorticoid dose on bone metabolism of patients with myasthenia gravis.

This current study aimed to evaluate the frequency of low bone mass, osteopenia, and osteoporosis in patients with myasthenia gravis (MG) and to investigate the possible association between bone mineral density (BMD) and plasma levels of bone metabolism markers. Eighty patients with MG and 62 controls BMD were measured in the right femoral neck and lumbar spine by dual-energy X-ray absorptiometry. Plasma concentrations of osteocalcin, osteopontin, osteoprotegerin, tumor necrosis factor (TNF-α), interleukin (IL)-1ß, IL-6, dickkopf (DKK-1), sclerostin, insulin, leptin, adrenocorticotropic hormone, parathyroid hormone, and fibroblast growth factor (FGF-23) were analyzed by Luminex®. The mean age of patients was 41.9 years, with 13.5 years of length of illness, and a mean cumulative dose of glucocorticoids 38,123 mg. Patients had significant reduction in BMD of the lumbar, the femoral neck, and in the whole body when compared with controls. Fourteen percent MG patients had osteoporosis at the lumbar spine and 2.5% at the femoral neck. In comparison with controls, patients with MG presented lower levels of osteocalcin, adrenocorticotropic hormone, parathyroid hormone, sclerostin, TNF-α, and DKK-1 and higher levels of FGF-23, leptin, and IL-6. There was a significant negative correlation between cumulative glucocorticoid dose and serum calcium, lumbar spine T-score, femoral neck BMD, T-score, and Z-score. After multivariate analysis, higher TNF-α levels increased the likelihood of presenting low bone mass by 2.62. MG patients under corticotherapy presented low BMD and altered levels of bone markers.

Depression and anxiety in a case series of amyotrophic lateral sclerosis: frequency and association with clinical features.

Objective: To investigate the frequency of anxiety and depression and their association with clinical features of amyotrophic lateral sclerosis. Methods: This is a cross-sectional and descriptive study including a consecutive series of patients with sporadic amyotrophic lateral sclerosis according to Awaji's criteria. Patients underwent clinical and psychiatric assessment (anxiety and depression symptoms). Results: We included 76 patients. The men/women ratio was 1.6:1. Participants' mean age at disease onset was 55 years (SD±12.1). Sixty-six patients (86.8%) were able to complete psychiatric evaluation. Clinically significant anxiety was found in 23 patients (34.8%) while clinically significant depression was found in 24 patients (36.4%). When we compared patients with and without depression a significant difference was seen only in the frequency of anxiety symptoms (p<0.001). We did further analysis comparing subgroups of patients classified according to the presence or not of anxiety and or depression, without any significant difference regarding sex, age at onset, initial form, disease duration or functional measures. A positive correlation between anxiety and depressive symptoms was found (p<0.001). Conclusion: Anxiety and depressive symptoms were highly correlated and frequent in patients with amyotrophic lateral sclerosis. In addition, anxiety and depression were not associated with disease duration and presentation, sex, age at onset, and functional score. Objetivo: Investigar a frequência de ansiedade e depressão e sua associação com aspectos clínicos da esclerose lateral amiotrófica. Métodos: Estudo transversal e descritivo de uma série consecutiva de pacientes com esclerose lateral amiotrófica esporádica conforme os critérios de Awaji. Os pacientes foram submetidos à avaliação clínica e psiquiátrica (sintomas depressivos e ansiosos). Resultados: Foram incluídos 76 pacientes. A relação homem/mulher foi de 1,6:1. A média de idade de início dos sintomas foi de 55 anos (DP±12,1). Foram capazes de completar a avaliação psiquiátrica 66 (86,8%) pacientes. Ansiedade clinicamente significativa foi encontrada em 23 pacientes (34,8%), enquanto depressão clinicamente significativa foi encontrada em 24 pacientes (36,4%). Ao comparar os pacientes com e sem depressão, houve diferença significativa apenas na frequência de sintomas de ansiedade (p<0,001). Posteriormente, foram comparados subgrupos de pacientes categorizados em relação à presença ou não de ansiedade e/ou depressão, sem diferença significativa em relação a sexo, idade de início dos sintomas, forma inicial, duração da doença ou na escala funcional. Foi encontrada correlação positiva entre os sintomas de ansiedade e depressão (p<0,001). Conclusão: Sintomas de ansiedade e depressão são frequentes em pacientes com esclerose lateral amiotrófica e estiveram altamente correlacionados. Ansiedade e depressão não foram associadas com duração da doença, forma inicial, sexo, idade de início dos sintomas e pontuação na escala funcional.

Depression and anxiety in a case series of amyotrophic lateral sclerosis: frequency and association with clinical features / Depressão e ansiedade em uma série de casos de esclerose lateral amiotrófica: frequência e associação com aspectos clínicos

ABSTRACT Objective To investigate the frequency of anxiety and depression and their association with clinical features of amyotrophic lateral sclerosis. Methods This is a cross-sectional and descriptive study including a consecutive series of patients with sporadic amyotrophic lateral sclerosis according to Awaji’s criteria. Patients underwent clinical and psychiatric assessment (anxiety and depression symptoms). Results We included 76 patients. The men/women ratio was 1.6:1. Participants’ mean age at disease onset was 55 years (SD±12.1). Sixty-six patients (86.8%) were able to complete psychiatric evaluation. Clinically significant anxiety was found in 23 patients (34.8%) while clinically significant depression was found in 24 patients (36.4%). When we compared patients with and without depression a significant difference was seen only in the frequency of anxiety symptoms (p<0.001). We did further analysis comparing subgroups of patients classified according to the presence or not of anxiety and or depression, without any significant difference regarding sex, age at onset, initial form, disease duration or functional measures. A positive correlation between anxiety and depressive symptoms was found (p<0.001). Conclusion Anxiety and depressive symptoms were highly correlated and frequent in patients with amyotrophic lateral sclerosis. In addition, anxiety and depression were not associated with disease duration and presentation, sex, age at onset, and functional score.

RESUMO Objetivo Investigar a frequência de ansiedade e depressão e sua associação com aspectos clínicos da esclerose lateral amiotrófica. Métodos Estudo transversal e descritivo de uma série consecutiva de pacientes com esclerose lateral amiotrófica esporádica conforme os critérios de Awaji. Os pacientes foram submetidos à avaliação clínica e psiquiátrica (sintomas depressivos e ansiosos). Resultados Foram incluídos 76 pacientes. A relação homem/mulher foi de 1,6:1. A média de idade de início dos sintomas foi de 55 anos (DP±12,1). Foram capazes de completar a avaliação psiquiátrica 66 (86,8%) pacientes. Ansiedade clinicamente significativa foi encontrada em 23 pacientes (34,8%), enquanto depressão clinicamente significativa foi encontrada em 24 pacientes (36,4%). Ao comparar os pacientes com e sem depressão, houve diferença significativa apenas na frequência de sintomas de ansiedade (p<0,001). Posteriormente, foram comparados subgrupos de pacientes categorizados em relação à presença ou não de ansiedade e/ou depressão, sem diferença significativa em relação a sexo, idade de início dos sintomas, forma inicial, duração da doença ou na escala funcional. Foi encontrada correlação positiva entre os sintomas de ansiedade e depressão (p<0,001). Conclusão Sintomas de ansiedade e depressão são frequentes em pacientes com esclerose lateral amiotrófica e estiveram altamente correlacionados. Ansiedade e depressão não foram associadas com duração da doença, forma inicial, sexo, idade de início dos sintomas e pontuação na escala funcional.

Determinants of quality of life in Brazilian patients with myasthenia gravis.

OBJECTIVES: The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. METHODS: This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. RESULTS: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. CONCLUSION: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.

Determinants of quality of life in Brazilian patients with myasthenia gravis

OBJECTIVES: The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. METHODS: This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. RESULTS: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. CONCLUSION: The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.

Cognitive functioning in adolescents with migraine / Funcionamento Cognitivo em Adolescentes com Migrânea

Although migraine is highly prevalent in children and teenagers, it often goes undetected in these patients, resulting in underdiagnosis and inadequate treatment. Several studies have investigated cognitive changes in adults with migraine. However, there are few studies focusing on children and adolescents. Objective : To investigate cognitive performance of adolescents with migraine. Methods : Twenty-eight adolescents diagnosed with migraine and twenty-six individuals without a history of headache were recruited for the study. All participants were evaluated using standardized neuropsychological tests. Results : Adolescents with migraine had worse performance on tests evaluating short- and long-term verbal memory, attention, executive function, and speed of processing information than controls. Conclusion : Cognitive dysfunction is common in adolescents with migraine. Since the cognitive deficits found in adolescents with migraine are similar to those reported in adults with migraine, cognitive impairment seems to persist throughout life.

Apesar de apresentar uma prevalência alta em crianças e adolescentes, nem sempre a migrânea é corretamente identificada nessa faixa etária, o que resulta em tratamentos inadequados e ineficazes. Vários estudos investigaram disfunções cognitivas associadas à migrânea em adultos. No entanto, poucos foram direcionados às crianças e aos adolescentes. Objetivo : Investigar o funcionamento cognitivo de adolescentes com migrânea. Métodos : Participaram do estudo 28 adolescentes diagnosticados com migrânea e 26 controles. Os participantes foram avaliados através de testes neuropsicológicos padronizados. Resultados : Os adolescentes com migrânea apresentaram pior desempenho que controles em testes que avaliaram memória verbal de curto e de longo prazo, atenção, funções executivas, velocidade de processamento. Conclusão : Disfunção cognitiva é comum em adolescentes com migrânea. Os resultados apontam para a persistência dos prejuízos cognitivos ao longo da vida, uma vez que os prejuízos encontrados nos adolescentes são semelhantes aos encontrados em adultos com migrânea.

Amyotrophic lateral sclerosis in Brazil: Case series and review of the Brazilian literature.

Our objective was to systematically analyse the first series of cases of amyotrophic lateral sclerosis (ALS) in Minas Gerais and to review the Brazilian literature about clinical studies in ALS. This was a cross-sectional and descriptive study of a consecutive series of patients with probable or defined sporadic ALS according to the Awaji criteria, followed at two referral centres of Belo Horizonte (South-east Brazil). Patients underwent full clinical assessment. Comparisons of patient subgroups according to disease duration and initial presentation were performed. A systematic review was performed about Brazilian clinical studies in ALS. Results showed that of the 61 enrolled patients the male/female ratio was 1.6:1. The mean age at onset of symptoms was 54.9 years (SD ± 11.4). Mean age at diagnosis was 56.3 years (SD ± 11.1). Regarding the initial form of presentation, 43 cases (70.5%) were spinal, 12 cases (19.7%) were generalized and six cases (9.8%) were bulbar. Eight studies were found in the systematic review. In conclusion, the profile of our sample was similar to other national and international series, except for fewer cases of bulbar ALS in our series. There are few clinical studies of ALS in Brazil. The national data of prevalence and incidence are still uncertain.

Cognitive functioning in adolescents with migraine.

Although migraine is highly prevalent in children and teenagers, it often goes undetected in these patients, resulting in underdiagnosis and inadequate treatment. Several studies have investigated cognitive changes in adults with migraine. However, there are few studies focusing on children and adolescents. OBJECTIVE: To investigate cognitive performance of adolescents with migraine. METHODS: Twenty-eight adolescents diagnosed with migraine and twenty-six individuals without a history of headache were recruited for the study. All participants were evaluated using standardized neuropsychological tests. RESULTS: Adolescents with migraine had worse performance on tests evaluating short- and long-term verbal memory, attention, executive function, and speed of processing information than controls. CONCLUSION: Cognitive dysfunction is common in adolescents with migraine. Since the cognitive deficits found in adolescents with migraine are similar to those reported in adults with migraine, cognitive impairment seems to persist throughout life.

Apesar de apresentar uma prevalência alta em crianças e adolescentes, nem sempre a migrânea é corretamente identificada nessa faixa etária, o que resulta em tratamentos inadequados e ineficazes. Vários estudos investigaram disfunções cognitivas associadas à migrânea em adultos. No entanto, poucos foram direcionados às crianças e aos adolescentes. OBJETIVO: Investigar o funcionamento cognitivo de adolescentes com migrânea. MÉTODOS: Participaram do estudo 28 adolescentes diagnosticados com migrânea e 26 controles. Os participantes foram avaliados através de testes neuropsicológicos padronizados. RESULTADOS: Os adolescentes com migrânea apresentaram pior desempenho que controles em testes que avaliaram memória verbal de curto e de longo prazo, atenção, funções executivas, velocidade de processamento. CONCLUSÃO: Disfunção cognitiva é comum em adolescentes com migrânea. Os resultados apontam para a persistência dos prejuízos cognitivos ao longo da vida, uma vez que os prejuízos encontrados nos adolescentes são semelhantes aos encontrados em adultos com migrânea.