Inspiratory Muscle Weakness is Related to Poor Short-Term Outcomes for Heart Transplantation

Abstract Introduction: In heart transplantation (HT) recipients, several factors are critical to promptly adopting appropriate rehabilitation strategies and may be important to predict outcomes way after surgery. This study aimed to determine preoperative patient-related risk factors that could adversely affect the postoperative clinical course of patients undergoing HT. Methods: Twenty-one hospitalized patients with heart failure undergoing HT were evaluated according to respiratory muscle strength and functional capacity before HT. Mechanical ventilation (MV) time, reintubation rate, and intensive care unit (ICU) length of stay were recorded, and assessed postoperatively. Results: Inspiratory muscle strength as absolute and percentpredicted values were strongly correlated with MV time (r=-0.61 and r=-0.70, respectively, at P<0.001). Concerning ICU length of stay, only maximal inspiratory pressure (MIP) absolute and percent-predicted values were significantly associated. The absolute -MIP- was significantly negatively correlated with ICU length of stay (r=-0.58 at P=0.006) and the percent-predicted MIP was also significantly negatively correlated with ICU length of stay (r=-0.68 at P=0.0007). No associations were observed between preoperative functional capacity, age, sex, and clinical characteristics and MV time and ICU length of stay in the cohort included in this study. Patients with respiratory muscle weakness had a higher prevalence of prolonged MV, reintubation, and delayed ICU length of stay. Conclusion: An impairment of preoperative MIP was associated with poorer short-term outcomes following HT. As such, inspiratory muscle strength is an important clinical preoperative marker in patients undergoing HT.

Functional performance and muscular strength in symptomatic female carriers of Duchenne muscular dystrophy / Desempenho funcional e força muscular em portadoras sintomáticas de distrofia muscular de Duchenne

Abstract Duchenne muscular dystrophy (DMD) usually affects men. However, women are also affected in rare instances. Approximately 8% of female DMD carriers have muscle weakness and cardiomyopathy. The early identification of functional and motor impairments can support clinical decision making. Objective: To investigate the motor and functional impairments of 10 female patients with dystrophinopathy diagnosed with clinical, pathological, genetic and immunohistochemical studies. Methods: A descriptive study of a sample of symptomatic female carriers of DMD mutations. The studied variables were muscular strength and functional performance. Results: The prevalence was 10/118 (8.4%) symptomatic female carriers. Deletions were found in seven patients. The age of onset of symptoms in female carriers of DMD was quite variable. Pseudohypertrophy of calf muscles, muscular weakness, compensatory movements and longer timed performance on functional tasks were observed in most of the cases. Differently from males with DMD, seven female patients showed asymmetrical muscular weakness. The asymmetric presentation of muscle weakness was frequent and affected posture and functionality in some cases. The functional performance presents greater number of compensatory movements. Time of execution of activities was not a good biomarker of functionality for this population, because it does not change in the same proportion as the number of movement compensations. Conclusion: Clinical manifestation of asymmetrical muscle weakness and compensatory movements, or both can be found in female carriers of DMD mutations, which can adversely affect posture and functional performance of these patients.

Resumo A distrofia muscular de Duchenne (DMD) geralmente afeta indivíduos do sexo masculino. No entanto, mulheres também são acometidas em casos raros. Aproximadamente 8% das portadoras de DMD têm fraqueza muscular ou cardiomiopatia. A identificação precoce das alterações funcionais e motoras pode alterar a tomada de decisão clínica. Objetivo: Investigar as deficiências motoras e funcionais de 10 pacientes do sexo feminino com distrofinopatia diagnosticada por estudos clínicos, patológicos, genéticos e imuno-histoquímicos. Método: Estudo descritivo de uma amostra de portadoras sintomáticas de mutações DMD. As variáveis estudadas foram força muscular e desempenho funcional. Resultados: A prevalência foi de 10/118 (8,4%) de portadoras sintomáticas de DMD. Foram encontradas deleções em sete pacientes. A idade de início dos sintomas em portadoras de DMD foi variável. Pseudo-hipertrofia de panturrilhas, movimentos compensatórios, fraqueza muscular e aumento no tempo de execução de tarefas funcionais foram observados na maioria dos casos. Diferentemente dos homens com DMD, sete pacientes apresentaram fraqueza muscular assimétrica. A apresentação assimétrica da fraqueza muscular foi frequente, podendo afetar a postura e a funcionalidade. O desempenho funcional geralmente apresenta aumento no número de movimentos compensatórios. Não podemos sempre considerar o tempo como um bom marcador de funcionalidade para essa população, uma vez que não muda na mesma proporção que o número de compensações em todas essas pacientes. Conclusão: Fraqueza muscular assimétrica e movimentos compensatórios, ou ambos, podem ser encontrados em portadoras sintomáticas de DMD, o que pode afetar a postura e a funcionalidade dessas pacientes.

Serious adverse event: late neurotropic disease associated with yellow fever vaccine / Evento adverso grave: doença neurotrópica tardia associada à vacina da febre amarela

ABSTRACT The yellow fever is a systemic disease that was under control due to the effective campaigns against the vector and promotion of vaccines programs. However, since 1999, outbreaks appeared because of inefficient control of the vector, and led to the need of amplifying the immunization in large scale against the yellow fever virus, and consequently, raising the risk of adverse reactions to the vaccine. We report a case of previously healthy infant, who was referred to our care service, after 3 days with fever, chills, nausea and vomits, he received support therapy and was discharged from the hospital. After 24 hours of supportive measures, he was discharge. The patient returned to our service with general condition decline, strabismus, inability to control of cervical musculature and reduced force of the legs. The patient vaccine had received all vaccines from the calendar, and he was vaccinated for yellow fever 20 days before symptoms. During the hospitalization, liquor was collected, and ceftriaxone and aciclovir were administered. After negative cultures from the liquor, the antibiotics were suspended. The computed tomography of patient's brain showed no alterations. Research for antibodies against yellow fever was requested, being positive for IgM in the liquor, and confirming the neurotropic disease associated with the yellow fever vaccine. On the fifth day of hospitalization, the patient showed improvement on the strabismus, cervical tonus, and musculature force. On the tenth day of hospitalization, patient showed complete improvement, and his laboratory exams no alterations. Subsequently, patient was discharged. The vaccine against yellow fever is safe, efficient and highly recommended, however it is not completely free from serious adverse reactions, including death.

RESUMO A febre amarela é uma doença sistêmica que estava controlada graças às efetivas campanhas de combate ao vetor e aos programas de vacinação. Porém, desde 1999, os surtos reiniciaram-se, devido à ineficácia do controle do vetor, levando à necessidade da imunização em larga escala contra o vírus da febre amarela, gerando aumento do risco de ocorrência de reação adversa à vacina. O presente estudo se propôs a relatar o caso de um lactente previamente saudável, que procurou pronto atendimento, pois, há 3 dias, apresentava febre, calafrios, náusea e vômitos. Em 24 horas após medidas de suporte e alta, evoluiu com queda do estado geral, estrabismo, falta de controle da musculatura cervical e redução da força muscular de membros inferiores. O caderno vacinal encontrava-se completo, tendo recebido vacina contra febre amarela há 20 dias. Durante a internação, foi realizada coleta do liquor, e foram administrados ceftriaxona e aciclovir. Após cultura negativa do liquor, o antibiótico foi suspenso. A tomografia computadorizada de crânio não apresentou alterações. Solicitou-se pesquisa de anticorpos contra o vírus da febre amarela no liquor, sendo positiva para IgM e confirmando a doença neurotrópica associada à vacina da febre amarela. A partir do quinto dia de internação, o paciente evoluiu com melhora do estrabismo, do tônus cervical e da força muscular. No décimo dia de internação, apresentou melhora completa do quadro, sem alterações laboratoriais, recebendo alta. A vacina contra febre amarela é segura, eficaz e fortemente recomendada, porém não está completamente isenta de reações adversas graves, inclusive podendo levar a quadros fatais.

Síndrome de Ehlers-Danlos hipermóvel: um relato de caso / Hypermobile Ehlers-Danlos syndrome: a case report

A síndrome de Ehlers-Danlos é estabelecida por distúrbios hereditários do tecido conjuntivo que tem como manifestações principais a hipermobilidade articular, a hiperextensibilidade da pele e a fragilidade de tecidos, como articulações, ligamentos, pele, vasos sanguíneos e órgãos internos. São reconhecidos 13 subtipos, de acordo com Classificação Internacional de 2017. Dentre estes, abordamos o hipermóvel, cujo diagnóstico é eminentemente clínico, com manifestações sistêmicas distintas. Esse artigo refere-se ao caso de uma paciente diagnosticada com síndrome de Ehlers-Danlos hipermóvel, tendo como intuito a atualização acerca dos novos critérios diagnósticos, assim como o diagnóstico precoce de tal raropatia.

Ehlers-Danlos syndrome is established through hereditary disorders of connective tissue, and has as its manifestations: joint hypermobility, skin hyperextensibility, and fragility of tissues such as joints, ligaments, skin, blood vessels, and internal organs. Thirteen subtypes have been recognized according to the 2017 International Classification. Among these, the hypermobile type, the diagnosis of which is eminently clinical, with distinct systemic manifestations, will be addressed. This article refers to the case of a patient diagnosed with hypermobile Ehlers-Danlos syndrome, with the objective of updating the new diagnostic criteria, as well as the early diagnosis of such a rare disease.

Síndrome de Guillain-Barré ¿enfrentando un nuevo brote? Reporte de 4 casos / Guillain-Barre Syndrome. Report of 4 cases. Is this a new cluster?

El Síndrome de Guillain Barré (SGB) es una enfermedad inflamatoria desmielinizante aguda de probable etiología autoinmune, relacionada con diversos procesos infecciosos. Se caracteriza por debilidad muscular y disminución de los reflejos, pudiendo presentar un patrón clásico simétrico ascendente o con variables. La gravedad y pronóstico son variables, pudiendo comprometer los músculos torácicos derivando en insuficiencia respiratoria. Han sido descritos brotes endémicos asociados a diversos agentes infecciosos. Se presenta el reporte de 4 casos con el patrón más frecuente de SGB en los cuales se hizo el diagnóstico clínico confirmado por estudio del líquido cefalorraquídeo o electromiografía con progresión agresiva, 3 de ellos ameritando el traslado a unidad de terapia intensiva (UTI) para su tratamiento. Presentaron distintos factores de riesgo infecciosos como la suspensión de terapia antiretroviral y síntomas gastrointestinales, principalmente diarrea acuosa previo al debut de la paresia. Se implementó el tratamiento haciendo uso de plasmaféresis en uno de los casos e inmunoglobulina endovenosa en el resto con resultados variables. Se resalta la importancia del diagnóstico oportuno de esta patología ante la presencia de paresia y arreflexia con o sin patrón característico con la finalidad de atender la progresión de los mismos de forma adecuada, mejorar el pronóstico y evitar o disminuir las secuelas de los pacientes(AU)

Guillain Barré Syndrome (GBS) is an acute demyelinating inflammatory disease with probable autoimmune etiology related to diverse infectious processes. It is characterized by muscle weakness and diminished reflexes and may present an ascending symmetrical pattern or with other variables. The severity and prognosis are variable, and the thoracic muscles can be affected, resulting in respiratory failure. Endemic outbreaks associated with various infectious agents have been described. A report of 4 cases is presented in which the clinical diagnosis was confirmed by cerebrospinal fluid study or electromyography, with aggressive progression, 3 of them requiring to be transferred to the intensive care unit. The patients presented different infectious risk factors such as the interruption of anti-retroviral therapy and gastrointestinal symptoms, mainly watery diarrhea prior to the onset of the symptoms. The treatment was implemented using plasmapheresis in one of the cases and intravenous immunoglobulin in the rest with variable results. The importance of the timely diagnosis of this pathology in the presence of paresis and dimished reflexes with or without the characteristic pattern is highlighted in order to address the progression, appropriate management, improve the prognosis and avoid or reduce the sequelae of patients(AU)

Histiocitosis de células de Langerhans con compromiso vertebral / Langerhans cell histiocytosis with vertebral involvement

Resumen: Introducción: La histiocitosis de células de Langerhans (HCL) es un trastorno histiocítico raro y su incidencia exacta se mantiene desconocida; se ha diagnosticado en todos los grupos de edad, pero es más común en los primeros 3 años de vida. Se caracteriza por lesiones únicas o múltiples de tipo osteolítico causadas por proliferación clonal de células histológicamente similares a las células de Langerhans; su presentación clínica es heterogénea. Caso clínico: Presentamos el caso de una paciente de sexo femenino de 7 años, con dificultad para la marcha y debilidad progresiva en los miembros inferiores de 5 días de evolución. A la exploración física presenta hallazgos concordantes con síndrome piramidal e hipoes­ tesias de miembros inferiores. Se realizó resonancia magnética (RM) de columna y tomografía computarizada de cráneo simple, que descartó patología intracraneal . En la RM de columna se detectó vertebra plana con extensión epidural y para­ vertebral, por lo que se inició manejo con esteroides y se indicó descompresión quirúrgica. Se realizó resección parcial y biopsia de la lesión. Debido a los hallazgos histológicos y la presencia de marcadores positivos para CD1a y CD207, se confirmó el diagnóstico de HCL. Conclusiones: La HCL es una enfermedad poco frecuente y de difícil diagnóstico por su presentación heterogénea. El granuloma eosinofílico y la vértebra plana como hallazgos imagenológicos pueden orientar el diagnóstico, aunque siempre se debe confirmar histológicamente.

Abstract: Background: Langerhans cell histiocytosis (LCH) is a rare disease, more common in the first three years of lite. lt is characterized by single ar multiple osteolytic lesions due to clonal proliferation of cells histologically similar to Langerhans cells; its clínical presentation is heterogeneous. Case report: 7-year-old female patient with 5 days of progressive lower extremity weakness and difficulty to walk. Physical exam findings were consistent with pyramidal syndrome and lower extremities hypoesthesia. Magnetic resonance imaging (MRI) of spine and cranial computed tomography (CT) were performed. lntracranial pathology was ruled out. The MRI findings showed vertebra plana with epidural and paravertebral involvement, so treatment with steroids and surgical decompression initiated. Partíal resection and biopsy of the lesion was performed. Due to histological findings and positive CD1a and CD207 markers, diagnosis of LCH was confirmed. Conclusions: LCH is an uncommon disease with a challenging diagnosis due to its heterogeneous clinical presentation. Eosinophilic granuloma and vertebra plana as imaging findings may guide the diagnosis. However, it should always be confirmed with histological evidence.

Alterações motoras em crianças e adolescentes pós-cirurgia de tumor intramedular: estudo retrospectivo / Motor changes in children and adolescents after intramedullary tumor surgery: a retrospective study

Introdução: Os tumores intramedulares são raros e correspondem a menos de 10% das neoplasias do sistema nervoso central. O crescimento tumoral pode comprimir feixes nervosos e resultar em perda da função motora e sensorial. A abordagem cirúrgica é o principal pilar de tratamento e visa à máxima ressecção tumoral com preservação da função. A reabilitação destes pacientes é individualizada se analisarmos os déficits funcionais e prognósticos. Objetivo: Analisar a idade e sexo dos pacientes, tipo e localização do tumor, tratamento cirúrgico e tratamento oncológico dos pacientes com tumor intramedular. Descrever as alterações motoras decorrentes desse tratamento. Método: Estudo retrospectivo realizado no Instituto de Oncologia Pediátrica através de dados de prontuários de janeiro de 2013 a dezembro de 2016 de pacientes com tumor intramedular. Dados analisados: idade ao diagnóstico e no momento cirúrgico, gênero, diagnóstico e localização do tumor, tipo de tratamento oncológico, cirurgia realizada, sequela pré e pós-cirurgia, indicação de coletes ortopédicos e tempo de uso. Não houve necessidade de aplicação do Termo de Consentimento Livre e Esclarecido por ser um estudo retrospectivo. Resultados: Foram selecionados doze prontuários de pacientes. A média de idade dos pacientes ao diagnóstico foi de 10 anos e 8 meses e média de 32 dias até a abordagem cirúrgica. Sete pacientes eram do sexo feminino e cinco do sexo masculino. Todos os pacientes foram submetidos a laminectomias, sendo três submetidos também a protocolo quimioterápico e radioterápico, e dois a protocolo quimioterápico. Dois pacientes foram orientados a usar colar cervical e dois orientados a usar colete ortopédico. Todos pacientes apresentaram alteração da marcha e diminuição de força muscular nos grupos pré e pós-cirúrgico. Conclusão: A idade média dos pacientes ao diagnóstico de tumor intramedular foi inferior a 11 anos, com predomínio do sexo feminino. Os diagnósticos oncológicos e as localizações tumorais foram variados. Sete pacientes evoluíram com deambulação independente após atendimento fisioterápico. A indicação de colares e coletes cervicais precisa ser mais estudada e sistematizada.(AU)

Introduction: Intramedullary tumors are rare and account for less than 10% of neoplasms of the central nervous system. Tumor growth may compress nerve bundles and result in loss of motor and sensory function. The surgical approach is the main pillar of treatment and aims at maximum tumor resection with preservation of function. The rehabilitation of these patients is individualized if we analyze the functional and prognostic deficits. Objective: To analyze the age and sex of the patients, type and location of the tumor, surgical treatment and oncological treatment of patients with intramedullary tumor. Describe the motor changes resulting from this treatment. Method: Retrospective study conducted at the Pediatric Oncology Institute through data from medical records from January 2013 to December 2016 of patients with intramedullary tumor. Data analyzed: age at diagnosis and at the time of surgery, gender, diagnosis and location of the tumor, type of cancer treatment, surgery performed, pre and post-surgery sequelae, indication of orthopedic vests and time of use. There was no need to apply the Free and Informed Consent Term because it is a retrospective study. Results: Twelve patient charts were selected. The mean age of the patients at diagnosis was 10 years and 8 months and mean of 32 days until the surgical approach. Seven patients were female and five were male. All patients underwent laminectomies, three of which were submitted to chemotherapy and radiotherapy, and two to chemotherapy protocol. Two patients were instructed to wear cervical collar and two oriented to wear orthopedic vest. All patients presented gait alteration and decreased muscle strength in the pre and post-surgical groups. Conclusion: The mean age of the patients at the diagnosis of intramedullary tumor was less than 11 years, with a predominance of females. Oncological diagnoses and tumor sites were varied. Seven patients evolved with independent walking after physical therapy. The indication of necklaces and cervical vests needs to be further studied and systematized.(AU)

Acute muscular weakness in children / Fraqueza muscular aguda em crianças

ABSTRACT Acute muscle weakness in children is a pediatric emergency. During the diagnostic approach, it is crucial to obtain a detailed case history, including: onset of weakness, history of associated febrile states, ingestion of toxic substances/toxins, immunizations, and family history. Neurological examination must be meticulous as well. In this review, we describe the most common diseases related to acute muscle weakness, grouped into the site of origin (from the upper motor neuron to the motor unit). Early detection of hyperCKemia may lead to a myositis diagnosis, and hypokalemia points to the diagnosis of periodic paralysis. Ophthalmoparesis, ptosis and bulbar signs are suggestive of myasthenia gravis or botulism. Distal weakness and hyporeflexia are clinical features of Guillain-Barré syndrome, the most frequent cause of acute muscle weakness. If all studies are normal, a psychogenic cause should be considered. Finding the etiology of acute muscle weakness is essential to execute treatment in a timely manner, improving the prognosis of affected children.

RESUMO A fraqueza muscular aguda (FMA) na criança é uma emergência pediátrica. Durante a abordagem diagnóstica, é crucial obter uma anamnese detalhada que inclua: início da fraqueza, história de estados febris associados, ingestão de substâncias tóxicas/toxinas, imunizações e história familiar. O exame neurológico também deve ser acurado. Nesta revisão, nós descrevemos as doeças mais frequentemente associadas com FMA, agrupadas pelo local de origem (desde o neurônio motor superior até a unidade motora). A detecção precoce de aumento de CK pode levar ao diagnóstico de miosite, e a hipopotassemia orienta para o diagnóstico de paralisia periódica. A oftalmoparesia, ptose e sinais bulbares são sugestivos de miastenia gravis ou de botulismo. Fraqueza distal e hiporeflexia são dados clínicos da síndrome de Guillain Barré (SGB), a mais frequente causa de FMA. Se todos os estudos são normais, uma causa psicogênica pode ser considerada. A busca da etiologia da FMA é essencial para que o tratamento seja feito a tempo, melhorando o prognóstico da criança afetada.

Assessment of acute motor deficit in the pediatric emergency room / Avaliação do déficit motor agudo no ambiente de pronto socorro pediátrico

Abstract Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute-onset muscle weakness. Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: "acute," "weakness," "motor deficit," "flaccid paralysis," "child," "pediatric," and "emergency". The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is a fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain-Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented. Conclusions: The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well-being and full health.

Resumo Objetivos: Apresentar uma abordagem clínica, enfatizar a investigação diagnóstica, voltada para crianças e adolescentes no pronto-socorro com fraqueza muscular de surgimento agudo. Fontes: Foi feita uma pesquisa sistemática na base de dados PubMed entre abril e maio de 2017, com os seguintes termos de pesquisa em várias combinações: "agudo", "fraqueza", "déficit motor", "paralisia flácida", "criança", "pediátrico" e "emergência". Os trabalhos escolhidos para esta revisão foram publicados nos últimos dez anos, de 1997 a 2017. Este trabalho aborda a faixa etária pediátrica, até 18 anos. Resumo dos dados: O déficit motor agudo é uma causa razoavelmente comum para crianças e adolescentes procurarem o pronto-socorro. Os pacientes podem ser classificados como com deficiência motora localizada ou difusa e uma descrição precisa das características clínicas é essencial para possibilitar um diagnóstico diferenciado completo. As duas causas mais comuns de paralisia flácida aguda no pronto-socorro pediátrico são síndrome de Guillain-Barré e mielite transversa, independentemente de outras etiologias serem consideradas, como encefalomielite disseminada aguda, mielite infecciosa, miastenia grave, derrame, hemiplegia alternante da infância, paralisia periódica, encefalite do tronco encefálico e fraqueza muscular funcional. Os algoritmos da investigação de fraqueza aguda localizada ou difusa na configuração de emergência também são apresentados. Conclusões: São enfatizadas as habilidades clínicas para obter um histórico completo e fazer um exame físico detalhado. Um manejo diagnóstico e terapêutico organizado, lógico e por etapas é essencial para eventualmente restaurar o bem-estar e a saúde total do paciente.

Clinical and laboratory description of a series of cases of acute viral myositis / Descrição clínico-laboratorial de uma série de casos de Miosite Aguda Viral

ABSTRACT OBJECTIVE: Describe the clinical and laboratory profile, follow-up, and outcome of a series of cases of acute viral myositis. METHOD: A retrospective analysis of suspected cases under observation in the emergency department was performed, including outpatient follow-up with the recording of respiratory infection and musculoskeletal symptoms, measurement of muscle enzymes, creatine phosphokinase (CPK), lactate dehydrogenase (LDH), transaminases (AST and ALT), blood count, C-reactive protein, and erythrocyte sedimentation rate in the acute phase and during follow-up until normalization. RESULTS: Between 2000 and 2009, 42 suspected cases were identified and 35 (27 boys) were included. The median age was 7 years and the diagnosis was reported in 89% in the first emergency visit. The observed respiratory symptoms were cough (31%), rhinorrhea (23%), and fever (63%), with a mean duration of 4.3 days. Musculoskeletal symptoms were localized pain in the calves (80%), limited ambulation (57%), gait abnormality (40%), and muscle weakness in the lower limbs (71%), with a mean duration of 3.6 days. There was significant increase in CPK enzymes (5507 ± 9180 U/L), LDH (827 ± 598 U/L), and AST (199 ± 245 U/L), with a tendency to leukopenia (4590 ± 1420) leukocytes/mm3. The complete recovery of laboratory parameters was observed in 30 days (median), and laboratory and clinical recurrence was documented in one case after 10 months. CONCLUSION: Typical symptoms with increased muscle enzymes after diagnosis of influenza and self-limited course of the disease were the clues to the diagnosis. The increase in muscle enzymes indicate transient myotropic activity related to seasonal influenza, which should be considered, regardless of the viral identification, possibly associated with influenza virus or other respiratory viruses.

RESUMO OBJETIVO: Descrever o perfil clínico-laboratorial, o acompanhamento e o desfecho de uma série de casos de miosite aguda viral. MÉTODO: Foi conduzida uma análise retrospectiva de casos suspeitos, em observação em unidade de emergência, e seguimento ambulatorial com o registro de sintomas de infecção respiratória, sintomas músculo-esqueléticos, determinação de enzimas musculares, creatina-fosfoquinase (CPK), desidrogenase lática (DHL), transaminases (AST e ALT), hemograma, proteína C reativa e velocidade de hemossedimentação, na fase aguda e no acompanhamento, até a normalização. RESULTADOS: Entre 2000 e 2009, 42 casos suspeitos foram identificados e 35 (27 meninos) foram incluídos. A mediana de idade foi de sete anos e o diagnóstico relatado em 89%, na primeira visita de emergência. Os sintomas respiratórios observados foram: tosse (31%), coriza (23%) e febre (63%), com duração média de 4,3 dias. Os sintomas músculo-esqueléticos foram: dor localizada nas panturrilhas (80%), deambulação limitada (57%), marcha anormal (40%) e fraqueza muscular nos membros inferiores (71%), com duração média de 3,6 dias. Observou-se elevação importante das enzimas CPK (5.507 ± 9.180) U/l, DHL (827 ± 598) U/l e AST (199 ± 245) U/l e tendência a leucopenia (4.590 ± 1.420) leucócitos/mm3. A recuperação completa dos parâmetros laboratoriais foi observada em 30 dias (mediana) e a recaída clínica e laboratorial em um caso após 10 meses. CONCLUSÃO: Os sintomas típicos com enzimas musculares elevadas após diagnóstico de influenza e o curso autolimitado foram os indícios para o diagnóstico. A elevação de enzimas musculares indica a atividade miotrópica transitória relacionada à influenza sazonal que deve ser considerada, a despeito da identificação viral, possivelmente associada com o vírus influenza ou outros vírus respiratórios.

Variante faringo-cérvico-braquial del síndrome de Guillain-Barré

El síndrome de Guillain-Barré es una polineuropatía desmielinizante inflamatoria aguda, existiendo variantes locorregionales poco frecuentes como la faringo-cérvico-braquial. Presentamos el caso de un varón de 63 años de edad que acudió a urgencias por debilidad de extremidades superiores y a nivel cervical, con incapacidad para deglutir y masticar, y una insuficiencia respiratoria aguda rápidamente progresiva por debilidad de músculos respiratorios, todo ello secundario a la variante faringo-cérvico-braquial del síndrome de Guillain-Barré. Esta variante, aunque poco frecuente, presenta un patrón clínico y unos criterios diagnósticos bien definidos, importantes de reconocer para poder así iniciar de forma precoz el tratamiento y mejorar el pronóstico, no siempre favorable, de estos pacientes.

Guillain-Barré syndrome is an acute inflammatory demyelinating polyneuropathy. Infrequent loco-regional variants, like the pharyngeal-cervical-brachial, have been described. We report the case of a 63-year-old male admitted to the emergency department with cervical and upper limb weakness, inability to swallow and chew, he also presented a rapidly progressive acute respiratory failure due to weakness of the respiratory muscles secondary to the pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. This variant, although unusual, presents a well-defined clinical pattern and diagnostic criteria, which is important in order to start an early treatment to improve the prognosis, not always favorable, to these patients.

Strength deficits of the shoulder complex during isokinetic testing in people with chronic stroke

OBJECTIVES: To examine the strength deficits of the shoulder complex after stroke and to characterize the pattern of weakness according to type of movement and type of isokinetic parameter. METHOD: Twelve chronic stroke survivors and 12 age-matched healthy controls had their shoulder strength measured using a Biodex isokinetic dynamometer. Concentric measures of peak torque and work during shoulder movements were obtained in random order at speeds of 60°/s for both groups and sides. Type of movement was defined as scapulothoracic (protraction and retraction), glenohumeral (shoulder internal and external rotation) or combined (shoulder flexion and extension). Type of isokinetic parameter was defined as maximum (peak torque) or sustained (work). Strength deficits were calculated using the control group as reference. RESULTS: The average strength deficit for the paretic upper limb was 52% for peak torque and 56% for work. Decreases observed in the non-paretic shoulder were 21% and 22%, respectively. Strength deficit of the scapulothoracic muscles was similar to the glenohumeral muscles, with a mean difference of 6% (95% CI -5 to 17). Ability to sustain torque throughout a given range of motion was decreased as much as the peak torque, with a mean difference of 4% (95% CI -2 to 10). CONCLUSIONS: The findings suggest that people after stroke might benefit from strengthening exercises directed at the paretic scapulothoracic muscles in addition to exercises of arm elevation. Clinicians should also prescribe different exercises to improve the ability to generate force and the ability to sustain the torque during a specific range of motion. .

Guillain-Barre syndrome following spinal anaesthesia

Guillain-Barre Syndrome [GBS] is the most common disease resulting in acute diffuse flaccid paralysis. It is an autoimmune disease that can occur at any age. The clinical course is characterized by weakness in the arms and legs, areflexia and the progression of muscle weakness from the lower limbs to the upper limbs. The most common causes of GBS include infections, vaccinations, surgery and some medicines. We present the case of a 48 years old male patient, who developed GBS after undergoing surgery for renal calculus, under spinal anaesthesia. In this case report, we presented a rather rare case of GBS occurring following spinal anaesthesia

Prevalência da fragilidade entre os pacientes com doença renal crônica em tratamento conservador e em diálise / Prevalence of frailty in patients in chronic kidney disease on conservative treatment and on dialysis

INTRODUÇÃO: Fragilidade é um estado de vulnerabilidade fisiológica do paciente que se associa ao maior número de hospitalização e óbito. OBJETIVOS: Avaliar a prevalência de fragilidade e os fatores a ela associados em pacientes com doença renal crônica (DRC) em tratamento conservador (TC), hemodiálise (HD) e diálise peritoneal (DP). MÉTODOS: A fragilidade foi avaliada em 146 pacientes (86 em TC, 37 em HD e 23 em DP) e caracterizada como fraqueza muscular e exaustão - pelos domínios do aspecto físico e da vitalidade, respectivamente, avaliados pelo questionário SF-36 de qualidade de vida; como inatividade física caso a resposta fosse "nunca" ou "quase nunca" - quando questionado sobre a prática de atividade física; e como perda de peso não intencional (> 4,5 kg por ano). Os pacientes foram divididos em três grupos: não frágeis (NF), pré-frágeis (PF) e frágeis (F). As variáveis demográficas, clínicas e laboratoriais foram extraídas dos prontuários dos pacientes. RESULTADOS: A fragilidade foi caracterizada em 36% dos pacientes em TC, 37,8% em HD e 47,8% em DP. Foi diagnosticada em 36,8% dos pacientes com idade entre 20 e 40 anos e 40,3% daqueles entre 41 e 60 anos. A fragilidade associou-se significativamente ao uso de vitamina D (r = 0.16; p = 0.03), hemoglobina (r = -0.14; p = - 0.02) e paratormônio intacto (r = 0.16; p = 0.03). CONCLUSÕES: A fragilidade é frequente entre os pacientes com DRC em tratamento conservador e dialítico, mesmo naqueles não idosos. Nos pacientes estudados, o fenótipo de fragilidade se associou com o não uso de vitamina D, menores níveis séricos de hemoglobina e níveis mais elevados de paratormônio.

INTRODUCTION: Frailty is a physiological vulnerability status of the patient which is associated with the increased number of hospitalization and death. OBJECTIVES: To evaluate the prevalence of frailty and its associated factors in patients with chronic kidney disease (CKD) on conservative treatment (CT), hemodialysis (HD) and peritoneal dialysis (PD). METHODS: Frailty was assessed in 146 patients (86 CT, 37 HD and 23 PD) and characterized as muscle weakness and exhaustion - by the physical aspect and vitality domains, respectively, evaluated in the SF-36 quality of life instrument; physical inactivity - if he or she answered "never" or "hardly ever" when asked about physical activity; and as unintentional weight loss (> 4.5 kg per year). Patients were divided into three groups: non-fragile (NF), pre-fragile (PF) and fragile (F). The demographic, clinical and laboratory data were extracted from patient charts. RESULTS: Frailty was diagnosed in 36% of patients on CT, 37.8% in HD and 47.8% in PD. It was characterized in 36.8% of patients aged between 20 and 40 years and 40.3% of those between 41 and 60 years. Frailty was significantly associated with the use of vitamin D (r = 0.16; p = 0.03), hemoglobin (r = -0.14; p = - 0.02) and intact parathyroid hormone (r = 0.16; p = 0.03). CONCLUSIONS: Frailty is common among patients with CKD on conservative treatment and dialysis, even in those who are not elderly. In the patients studied, the phenotype of frailty was associated with no usage of vitamin D, lower serum levels of hemoglobin and higher levels of parathyroid hormone.

Werdnig Hoffman in heterozygote twins

Spinal Muscular Atrophy [SMA] consists of three types of disease including Werdnig Hoffman [SMA type1] which is an autosomal recessive degenerative motor neuron disease. These patients have abnormally in motor function of the muscles and will die in the first few years of life. The genetic locuses of all three types of SMA are on the chromosome numbers and a deletion in locus 5q11 -q13 will result in a variety of this disease. Case Report: heterozygous twin infants [boy and girl] were born from relative parents admitted to the hospital, one in three days after another, with upper respiratory tract infection, respiratory distress and coughing. They were hypotonic and had tongue fasciculation. They were intubated and ventilated in the PICU. SMA was suspected because of the general muscular weakness; therefore, biopsy and neurophysiologic studies were performed. Quadriceps muscle biopsy showed fascicular atrophy of muscle fibers and in genetic analysis of SMN1 gene in twin homozygous deletion of SMN1 gene at exon 7 was found. They became ventilator dependent and suffered respiratory failure and two weeks after their admissions in hospital, and with three day interval, died. It seems prudent to perform genetic assessments before having children in the parents who are close relatives especially after one affected child

Persistence of Orientia tsutsugamushi in Humans

We investigated the persistence of viable Orientia tsutsugamushi in patients who had recovered from scrub typhus. Blood specimens were available from six patients with scrub typhus who were at 1 to 18 months after the onset of the illness. The EDTA-treated blood specimens were inoculated into ECV304 cells, and cultures were maintained for 7 months. Sequencing of the 56-kDa type-specific antigen gene of O. tsutsugamushi was performed to ascertain the homology of isolates. O. tsutsugamushi was isolated from all six patients, and nucleotide sequences of isolates serially collected from each patient were identical in all five patients in whom nucleotide sequences were compared. One patient relapsed 2 days after completion of antibiotic therapy; two patients complained of weakness for 1 to 2.5 months after the illness; one patient underwent coronary angioplasty 6 months later; and one patient suffered from a transient ischemic attack 8 months later. This finding suggests that O. tsutsugamushi causes chronic latent infection, which may be associated with certain clinical illnesses, preceded by scrub typhus. Antibiotic therapy abates the symptoms of scrub typhus, but does not eradicate O. tsutsugamushi from the human body.

Leptospirosis presenting as ascending progressive leg weakness and complicating with acute pancreatitis

Leptospirosis is a spirochetal bacterial infection of great public health importance. It has a broad spectrum of clinical manifestations which goes from subclinical infection and self-limited anicteric febrile illness (80-90 percent of all cases) to icteric leptospiropirosis known as Weil's disease. This is a severe disease characterized by hemorrhage, acute renal failure and jaundice. It is uncommon for leptospirosis to present itself as a primary neurological disease. Additionally, acute pancreatitis is an unusual gastrointestinal manifestation. We report a case of leptospirosis presenting as ascending progressive leg weakness and complicating with acute pancreatitis in an adult patient treated at Hospital Universitário, Universidade Federal de Santa Catarina. The diagnosis was confirmed through ELISA-IgM antibody testing positive for leptospirosis. After antibiotic therapy and support treatment for a few weeks, total resolution of severe manifestations was achieved. Rare and unusual presentations of leptospirosis should be kept in mind in relevant epidemiological scenario.

Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis / Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

O Lúpus Eritematoso Sistêmico (LES) e a Miastenia Gravis (MG) são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA) test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.