Children's Multisystem inflammatory syndrome with myopathy

Abstract This report describes a case of multisystem inflammatory syndrome in a child that evolved with a pattern of toxic shock syndrome with coronary artery ectasia and neurological involvement, documented by magnetic resonance imaging, with changes in the corpus callosum and myopathy in the pelvic girdle and paravertebral musculature.

Cystic lesion of pectoralis minor muscle: learning from mistakes

Hydatidosis is a frequent infestation in large endemic areas, caused by helminths. Primary localization within the muscle or bone tissues is rare. We report the case of a 52-year-old woman with a cystic lesion located in the right pectoralis minor muscle, who was initially diagnosed with cystic lymphangioma by imaging examination. She was submitted for surgical treatment; in block resection of the tumor along with the involved muscle was performed. The histopathological diagnosis was of hydatid cyst. The contribution of the ancillary lab tests is analyzed for a precise preoperative diagnostic approach. This case well illustrates that the most likely is not always what it appears to be. Facing of a cystic lesion in the lungs, liver or muscle, clinicians should always think on hydatid disease, particularly in endemic areas.

McArdle's disease: an underestimated or underdiagnosed myopathy in rheumatologic practice? Cases series and literature review / Doença de mcardle: uma miopatias subestimada e subdiagnosticada na pratica reumatológica? série clínica e revisão de literatura

OBJECTIVE: McArdle's disease is a metabolic myopathy that manifests with varied clinical conditions and is often confounded with other diagnoses. Herein, the authors report a case series and carry out a literature review. METHODS: A cross-sectional single-center study evaluating 12 patients with McArdle's disease was conducted. RESULTS: Mean age at onset of symptoms was 28.0±17.4 years, while age at disease diagnosis was 39.0±14.8 years. History of intolerance to physical exercises was observed in 10 cases; muscle weakness in 9, second wind phenomenon in only 1 case. The presence of cramps, fatigue and myalgia was observed in 12, 11 and 9 of the cases respectively. Median creatine phosphokinase level was 5951U/L. Most of the patients (83.3%) were initially diagnosed with another condition (polymyositis, inclusion body myositis, fibromyalgia and/or muscular dystrophy), and approximately half had received glucocorticoids and/or immunosuppressants prior to definitive diagnosis. All patients underwent muscular biopsy, which revealed the presence of subsarcolemmal vacuoles characterized by glycogen deposits, and negative histochemical reaction for the myophosphorylase enzyme. CONCLUSION: The present study reinforces the presence of clinical variability among patients and shows that McArdle's disease should be considered one of the differential diagnoses of inflammatory myopathies and other rheumatic diseases.

OBJETIVO: A doença de McArdle é uma miopatia metabólica que se manifesta com condições clínicas variadas e muitas vezes é confundida com outros diagnósticos. Os autores relatam uma série de casos e realizam uma revisão de literatura. MÉTODOS: Estudo transversal de um único centro em que foram avaliados 12 pacientes com doença de McArdle. RESULTADOS: A média de idade no início dos sintomas foi de 28,0±17,4 anos, enquanto a idade no diagnóstico da doença foi de 39,0±14,8 anos. História de intolerância ao exercício físico foi observada em 10 dos casos; fraqueza muscular em 9; fenômeno do "second wind" em apenas 1 caso. A presença de câimbras, fadiga e mialgia foi observada, respectivamente, em 12, 11 e 9 dos casos. O nível mediano de creatinafosfoquinase foi de 5951U/L. Oito pacientes foram inicialmente diagnosticados com outra condição (polimiosite, miosite de corpos de inclusão, fibromialgia e/ou distrofia muscular), e aproximadamente metade havia recebido glicocorticoides e/ou imunossupressores antes do diagnóstico definitivo. Todos os pacientes foram submetidos à biópsia muscular, que revelou a presença de vacúolos subsarcolêmicos caracterizados por depósitos de glicogênio e reação histoquímica negativa para a enzima miofosforilase. CONCLUSÕES: O presente estudo reforça a presença de variabilidade clínica entre pacientes e mostra que a doença de McArdle deve ser considerada um dos diagnósticos diferenciais de miopatias inflamatórias e outras doenças reumáticas.

Equinococosis quística músculo-esquelética primaria de evolución crónica / Primary musculo-skeletal echinococcosis of chronic evolution

Resumen La equinococosis quística es una zoonosis parasitaria crónica de alta prevalencia en Chile. Se presenta el caso clínico de un varón de 66 años, proveniente de la Región del Maule, con una equinococosis quística músculo-esquelética. Consultó por dolor, aumento de volumen y una fístula en muslo izquierdo, con salida de líquido cristalino. En el estudio imagenológico se identificaron múltiples lesiones quísticas en el ala sacra, hueso ilíaco y tejidos blandos de zona inguinal y muslo izquierdo. La serología Elisa IgG para Equinococcus granulosus fue positiva. Se realizó la resección quirúrgica de las lesiones musculares y se inició terapia antiparasitaria combinada con albendazol y praziquantel, con buena respuesta clínica; sin embargo, al suspender la terapia, por iniciativa del paciente, se reiniciaron los síntomas.

Cystic echinococcosis is a chronic parasitic zoonosis of high prevalence in Chile. We report a clinical case of a 66-year-old man, domiciled in an urban area of the Maule Region, who presents skeletal muscle cystic echinococcosis. Consultation for pain, volume increase and left thigh fistula that gives out crystalline fluid. In the study with imaging techniques, multiple cystic lesions are identified in the sacral wing, iliac bone, soft tissues of the groin and left thigh. No cysts were evident in other organs. Serology Elisa IgG was positive Echinococcus granulosus. Surgical resection of soft tissue injuries. Combined antiparasitic therapy with albendazole and praziquantel was started, with good clinical response. Upon discontinuation of antiparasitic therapy at the initiative of the patient, symptoms are reinitiated.

Miopatias com padrão distal: descrição clínica de pacientes do Estado da Bahia / Distal myopathies: clinical description of patients from the State of Bahia

INTRODUÇÃO: As miopatias são doenças cuja etiologia decorre de alterações estruturais e/ou funcionais no músculo esquelético. As miopatias distais são doenças musculares primárias em que fraqueza e, frequentemente atrofia, tem início nas mãos, antebraços, pés e segmento distal das pernas. Apesar de terem sido divididas como um grupo restrito de doenças, outras miopatias podem se manifestar com um padrão distal, como a miopatia nemalínica e as distrofias musculares cintura-membros. Devido à escassez de trabalhos que descrevem clinicamente as miopatias distais, este trabalho visou contribuir com essa caracterização. METODOLOGIA: Os pacientes foram selecionados no ambulatório de doenças neuromusculares do Hospital Universitário Professor Edgar Santos, em seguida avaliados clinicamente, através de exame físico e também com exames complementares: eletroneuromiografia, exames laboratoriais, estudo molecular e histopatológico. RESULTADOS: Quinze pacientes com padrão distal foram analisados, sendo 40% do sexo feminino, média de idade de 29,8 anos, seis (40|%) pacientes naturais da capital, Salvador-Bahia. Quanto ao padrão de distribuição de fraqueza, sete apresentavam padrão distal, enquanto oito, padrão distal-proximal. Os pacientes foram agrupados de acordo com a idade de início dos sintomas, sendo 11 iniciados na infância e adolescência (T em homozigose), um com sarcoglicanopatia (mutação c.229C>T em homozigose) e um com miopatia nemalínica (histopatológico com presença de corpos nemalínicos). DISCUSSÃO: Os achados identificados nos pacientes com diagnósticos firmados foram compatíveis com o que é visto na literatura, como apresentação clínica e mutações identificadas previamente. Destaca-se o componente distal pronunciado da paciente com sarcoglicanopatia, considerado incomum. Além disso, a descrição da ressonância magnética realizada nos indivíduos demonstrou um padrão típico. Na maior parte dos pacientes não se chegou a um diagnóstico etiológico, a despeito da investigação realizada com os exames complementares e clínicos. CONCLUSÃO: O presente estudo caracterizou uma amostra de pacientes com miopatias distais, corroborando que essas doenças se manifestam clinicamente de forma heterogênea. A caracterização e divisão entre grupos visa tornar mais fácil a investigação, devendo ser feita com exames complementares, considerados imprescindíveis para se estabelecer o diagnóstico etiológico dessas doenças

INTRODUCTION: Myopathies are diseases which etiology results from structural and/or functional changes in skeletal muscle. Distal myopathies are a group of muscular pathologies in which weakness and atrophy begins and predominates in distal limbs, like hands and feet. Although it has been divided as a restrict group of diseases, other myopathies can manifest with that pattern of weakness, such nemaline myopathy and limb-girdle muscular dystrophies. Due to the scarcity of studies that described clinically the distal myopathies, this study focuses on clinical characterization of myopathies with distal pattern of weakness. METHODOLOGY: The patients were selected in the outpatient clinic for neuromuscular diseases at Professor Edgar Santos University Hospital. Those subjects were clinically evaluated through physical examination, laboratory tests, electroneuromyography, magnetic resonance (MRI) and histopathological study. RESULTS: Fifteen patients with distal pattern were analyzed, being 40% female, mean age 29.8 years, six (40 %) patients were born in the capital, Salvador-Bahia. As for the pattern of weakness distribution, seven had an exclusive distal pattern, while eight had a distal-proximal pattern. Patients were grouped according to the age of onset of symptoms, of which 11 were initiated in childhood and adolescence ( T in homozygous in exon 53 in another and one patient were diagnosed by biopsy), one with sarcoglicanopathy (mutation c.229C> T in homozygous) and one with nemaline myopathy (histopathological with the presence of nemalinic bodies). DISCUSSION: The findings identified in patients with established diagnoses were compatible with what is seen in the literature, such as clinical presentation and previously identified mutations. We highlight the pronounced distal component of the patient with sarcoglicanopathy, considered to be uncommon. In addition, the description of MRI performed in the individuals demonstrated a typical pattern. Most of the patients were not diagnosed, despite the research done with the complementary and clinical exams. CONCLUSION: The present study characterized a sample of patients with distal myopathies, corroborating that these diseases manifest themselves clinically heterogeneously. The characterization and division between groups aims to make the investigation easier, and should be done with complementary tests, considered essential to establish the etiological diagnosis of these diseases

Evaluation of Cases of Abdominal Wall Endometriosis at Universidade Estadual de Campinas in a period of 10 Years / Avaliação dos casos de endometriose de parede abdominal na Universidade Estadual de Campinas em um período de 10 anos

Abstract Purpose To determine the clinical and epidemiological characteristics of abdominal wall endometriosis (AWE), as well as the rate and recurrence factors for the disease. Methods A retrospective study of 52 women with AWE was performed at Universidade Estadual de Campinas from 2004 to 2014. Of the 231 surgeries performed for the diagnosis of endometriosis, 52 women were found to have abdominal wall endometriosis (AWE). The frequencies, means and standard deviations of the clinical characteristics of these women were calculated, as well as the recurrence rate of AWE. To determine the risk factors for disease recurrence, Fisher's exact test was used. Results The mean age of the patients was 30.71 ± 5.91 years. The main clinical manifestations were pain (98%) and sensation of a mass (36.5%).We observed that 94% of these women had undergone at least 1 cesarean section, and 73% had used medication for the postoperative control of endometriosis. The lesion was most commonly located in the cesarean section scar (65%). The recurrence rate of the disease was of 26.9%. All 14 women who had relapsed had surgical margins compromised in the previous surgery. There was no correlation between recurrent AWE and a previous cesarean section (p = 0.18), previous laparotomy (p = 0.11), previous laparoscopy (p = 0.12) and postoperative hormone therapy (p = 0.51). Conclusion Women with previous cesarean sections with local pain or lumps should be investigated for AWE. The recurrence of AWE is high, especially when the first surgery is not appropriate and leaves compromised surgical margins.

Resumo Objetivos Determinar as características clínicas e epidemiológicas da endometriose de parede, bem como sua taxa de recorrência e os fatores que levam a ela. Métodos Estudo retrospectivo, em que se avaliaram 52 mulheres com endometriose de parede na Universidade Estadual de Campinas no período de 2004 a 2014. Entre as 231 cirurgias para diagnosticar endometriose, foram encontradas 52 mulheres que apresentavam endometriose de parede. Foram calculadas as frequências, a média e o desvio padrão das características clínicas destas mulheres, bem como a taxa de recorrência da endometriose de parede. Para determinar os fatores de risco de recorrência, foi utilizado o teste exato de Fisher. Resultados A idade média das mulheres foi de 30.71 ± 5,91 anos. As principais manifestações clínicas foram dor (98%) e sensação do nódulo (36,5%). Foi observado que 94% dessas mulheres tinham pelo menos uma cesárea, e 73% destas fizeram uso de medicação para controle da endometriose no pós-operatório. A localizaçãomais frequente da lesão foi na cicatriz da cesárea (65%). A taxa de recorrência da doença foi de 26,9%. Todas as 14mulheres que tiveramrecidiva tinhammargens cirúrgicas comprometidas na cirurgia prévia. Não houve correlação entre a endometriose de parede recorrente e a cesariana prévia (p = 0,18), a laparotomia prévia (p = 0,11), a laparoscopia prévia (p = 0,12) e receber terapia hormonal no pós-operatório (p = 0,51). Conclusão Mulheres com antecedente de cesárea anterior com dor local ou nódulo devem ser investigadas com relação à endometriose de parede. A recorrência do endometrioma de parede é alta, principalmente quando a primeira cirurgia não é adequada, e deixa margens cirúrgicas comprometidas.

Hernia del músculo tibial anterior: a propósito de un caso / Tibial anterior muscle hernia: one case report

Objetivo: Presentación de un caso y revisión de la literatura. Caso clínico: Varón de 59 años que acude a la consulta de cirugía general por tumoración en miembro inferior derecho. La exploración física revela una tumoración no dolorosa de 3 cm sugestiva de lipoma. Se realiza ecografía que informa de la existencia de una hernia del músculo tibial anterior. Dado que el paciente se encuentra asintomático, se decide tratamiento rehabilitador. Conclusiones: Las hernias musculares, habitualmente localizadas en los miembros inferiores, suponen una entidad a tener en cuenta en el diagnóstico diferencial de tumores de partes blandas. Su diagnóstico es clínico con confirmación ecográfica y su tratamiento habitualmente es conservador, aunque en casos sintomáticos puede requerir cirugía.

Objective: Presentation of a case report and review of literature. Case report: A 59-years old male came to the Outpatient Clinic complaining of a painless lump in right lower limb. Physical examination revealed a 3 cm diameter tender lump, suggestive of lipoma. Ultrasonography revealed a hernia in the tibial anterior muscle. Given that the patient was asymptomatic, rehabilitation treatment was decided. Conclusions: Muscular hernias, usually located in lower limbs, must be considered in the differential diagnosis of soft tissue lumps. Diagnosis is usually confirmed with ultrasonography and conservative treatment is indicated, though symptomatic cases might require surgery.

Miositis en pacientes pediátricos con inmunoglobulina M antidengue positiva / Myositis in pediatric patients with positive anti-dengue M immunoglobulin

INTRODUCCIÓN: la miositis aguda benigna infantil es un proceso poco frecuente, transitorio y autolimitado, que afecta predominantemente a la edad escolar tras una infección viral. OBJETIVO: divulgar esta forma de presentación de una virosis y contribuir a su diagnóstico. MÉTODOS: se estudiaron 13 pacientes, ingresados en el Hospital Pediátrico Docente del Cerro, por síndrome febril durante el año 2013 con inmunoglobulina M positiva antidengue, que presentaron impotencia funcional y concentraciones de creatininfosfokinasa superiores a 190 µl. Se evaluaron elementos demográficos, clínicos y niveles enzimáticos, y se aplicó el estadígrafo promedio. RESULTADOS: los signos y síntomas más comunes en todos los pacientes resultaron ser la fuerza muscular disminuida, el dolor intenso a la presión de los músculos gastrocnemios y la flexión dorsal de ambos pies. El dolor retro-ocular y el rash se presentaron indistintamente en ambos sexos. El promedio de edad fue de 11 años. Las cifras de la frecuencia cardiaca, respiratoria y tensión arterial fueron normales. La duración de los síntomas clínicos tuvo una media aritmética de 4 días. El conteo de leucocitos alcanzó promedio de 6 000 x 109/L, plaquetas 170 000 x 109/L, el hematocrito 37 vol/% y creatininfosfokinasa 217 µl. CONCLUSIONES: se concluye que el dolor muscular intenso con impotencia funcional en la extremidades inferiores, acompañado de cifras de cratinfosfokinasa elevadas en pacientes con inmunoglobulina M positiva antidengue, confirma el diagnóstico de miositis viral en el curso de esta arbovirosis. Se comenta que en Cuba no se ha efectuado algún reporte sobre esa entidad, por lo cual es oportuno realizar la presente comunicación que contribuya a su divulgación.

INTRODUCTION: benign acute childhood myositis is a infrequent, transient and self-limited process that mainly affects children at school age after viral infections. OBJECTIVE: to disseminate information on this form of presentation of a type of virosis and to contribute to its correct diagnosis. METHODS: thirteen patients admitted to the teaching pediatric hospital of Cerro with febrile syndrome during 2013 were studied. They showed positive anti-dengue M immunoglobuline, functional impotence and creatinin-phosphokinase over 190 µl. Several demographic and clinical elements as well as enzyme levels were assessed and the average statistic was applied. RESULTS: most frequent signs and symptoms in all the patients were reduced muscle strength, intense pain to gastrocnemy muscle pressure and dorsal flexion of both feet. The retroocular pain and rash occurred regardless of sex. The average age was 11 years. The heart and respiratory rate and blood pressure figures were all normal. The duration of clinical systems had an arithmetic mean of 4 days. Leukocyte count reached averages of 6 000 x 109/l; platelets showed 170 000 x 109/L; hematocrit reached 37 vol/% and creatinine-phosphokinase was 217 µl. CONCLUSIONS: intense muscular pain with functional impotence at the lower limbs accompanied by high creatinin-phosphokinase figures in patients with positive antidengue M immunoglobulin confirms the diagnosis of viral myositis in the course of this arbovirosis. It is commented that there has been no report on this disease in Cuba so far, so it is timely to provide the present communication in order to contribute to dissemination of information about this entity.

Hydatid cysts in muscles: clinical manifestations, diagnosis, and management of this atypical presentation

ABSTRACTINTRODUCTION: Hydatid cysts are rarely detected in muscle tissue (0.7-0.9%), even in endemic countries. The aim of this study was to present information regarding the clinical manifestations, diagnosis, and management of muscle echinococcosis.METHODS: Twenty-two patients with hydatid cysts in the muscle were followed from January 2006 through December 2014.RESULTS: Twenty-four sites of muscle involvement were observed in the 22 patients. Fifteen (68%) of our patients were women, while seven (32%) were men. The mean age was 28.1 ± 15.4 (6-61) years. The most frequent locations were the thigh (27.2%) and the paravertebral region (13.6%). Most patients reported a painless slow-growing mass with normal overlying skin. Most (90.2%) cases were treated by surgical excision and fine-needle aspiration.CONCLUSIONS: Primary muscle hydatid cyst should be considered in the differential diagnosis in cystic masses of the muscular system without pain and localized enlargement of soft tissue, especially in endemic areas. Hydatid cyst should be investigated using serological tests and imaging modalities. If possible, total surgical excision of hydatid cyst in the muscle should be performed.

Sarcopenia en pacientes con y sin insuficiencia renal crónica: diagnóstico, evaluación y tratamiento / Sarcopenia in patients with and without chronic renal insufficiency: diagnosis, evaluation and treatment

Se define sarcopenia como la pérdida de masa y función musculares, no sólo por disminución del tamaño sino también del número de fibras musculares. Altamente prevalente en el adulto mayor, aparece también en pacientes con enfermedades crónicas. En la insuficiencia renal crónica (IRC) contribuyen a su aparición la enfermedad crónica per se, la edad avanzada, el sedentarismo habitual, sumado a múltiples factores que deterioran el estado nutricional, tales como reducción de la ingesta asociada o no a drogas anorexígenas, inflamación crónica, déficit de hormonas anabólicas, bajos niveles de vitamina D, resistencia insulínica y disminución de gelsolina (proteína clave en el ensamblaje y desensamblaje de filamentos de actina). La presencia de sarcopenia correlaciona con mayor mortalidad, discapacidad y aumento del riesgo de caídas. El diagnóstico se basa en medir la fuerza muscular y el rendimiento físico; para lo primero se utiliza el dinamómetro, y para lo segundo la medida de la velocidad de la marcha (registra el tiempo necesario para caminar una distancia determinada) y el test ôTime Up and Goõ (evalúa el tiempo para levantarse, recorrer 3 metros y volver a sentarse). En pacientes con IRC, una adecuada ingesta proteica, sumado a actividad física (particularmente ejercicios de resistencia) mejoran el rendimiento físico, la aptitud respiratoria y la sobrevida en general, y reducen la mortalidad cardiovascular. Asimismo, el ejercicio aumenta elcontenido muscular de IGF-1, y del ARNm para factor de crecimiento tipo insulina II, la capacidad oxidativa muscular y el número de células satélites necesarias para regenerar las fibras musculares.

Sarcopenia is defined as the loss of muscle mass and function, not only due to muscle fiber decrease in size but also in number. Highly prevalent in older adults, it also appears in patients with chronic diseases. In the chronic renal failure (CRF), the facts that contribute to its appearance are: chronic disease per se, advanced age, sedentary lifestyle, added to multiple factors which deteriorate the nutritional status such as reduction of in-take associated or not to anorexic drugs, chronic inflammation, anabolic hormone deficit, vitamin D low levels, insulin resistance and gelsolin decrease ( key protein in the assembly and disassembly of actin filaments). Presence of sarcopenia correlates with greater mortality, disability and falls risk increase. Diagnosis is based on measuring muscle strength and physical performance, for the first one a dynamometer is used, and for the second one: walking speed measurement (records the needed period of time to walk a determined distance) and the test ôTime Up and Goõ (which evaluates the needed period of time to stand up, walk 3 meters and sit down again). In patients with CRF, an appropriate protein ingestion, added to physical activity, (specially resistance exercises) improve physical performance, respiratory aptitude and survival in general, and reduce cardiovascular mortality. Additionally, exercise increases IGF-1 muscle content, as well as the mRNA for insulin-like growth factor type II, muscle oxidative capacity and the number of required satellite cells to regenerate muscle fibers.

Utilidad de la resonancia magnética en el diagnóstico de las enfermedades musculares hereditarias / Utility of magnetic resonance imaging in the diagnosis of hereditary muscular diseases

Hereditary muscular diseases are rare disorders, due to a genetic defect that causes an alteration in the structure or function of the muscle fibers. They may present at any stage of life and their definitive diagnosis usually requires muscle biopsy. While the most frequent hereditary myopathies have a relatively characteristic clinical presentation, there is a substantial part thereof in which the symptoms are non-specific and the definitive diagnosis may take a long time. Magnetic Resonance Imaging (MRI) has earned a place in the diagnostic process of this last group of myopathies, confirming the presence of muscle involvement and raising diagnostic approaches based on its distribution, information that guides the immunohistochemical and/or genetic study necessary for the definitive diagnosis. In this article we review the basic study protocols with MRI of the myopathies and their interpretation, also showing some cases of these diseases.

Las enfermedades musculares hereditarias son patologías raras, debidas a un defecto genético que causa una alteración en la estructura o funcionamiento de las fibras musculares. Pueden debutar en cualquier etapa de la vida y su diagnóstico definitivo suele requerir de biopsia muscular. Si bien las miopatías hereditarias más frecuentes tienen una presentación clínica relativamente característica, existe una parte importante de ellas en que los síntomas son poco específicos y su diagnóstico definitivo puede tomar largo tiempo. La Resonancia Magnética (RM) ha ganado un espacio en el proceso diagnóstico de este último grupo de miopatías, confirmando la presencia del compromiso muscular y planteando aproximaciones diagnósticas en base a su distribución, información que acota el estudio inmunohistoquímico y/o genético necesario para el diagnóstico definitivo.En el presente artículo revisaremos los protocolos de estudio básico con RM de las miopatías y su interpretación, mostrando también algunos casos de estas enfermedades.

Imaging in the Diagnosis of Symptomatic Forearm Muscle Herniation.

Symptomatic muscle herniations are an unusual cause of upper extremity pain that is rarely reported in the literature. Out of 18 reported cases of upper extremity herniations, only 3 were caused by strenuous exertion6. Dynamic ultrasound and Dynamic MRI test are the very good tool for diagnosis of muscle herniation, FNAC and biopsy are rarely needed. This article describes a successful repair of a 22yr old manual worker’s ventral forearm herniation with polypropylene mesh. Prevalence Muscle herniation in an extremity is a well-known cause of pain, even though there have been extremely few documented cases. In a 2009 report published by the “Journal of Hand and Microsurgery,” only 200 cases of herniated muscles of the extremities had been reported since the mid-1800s, and only 17 cases of muscle herniation in the upper limb have been described10. Characteristics A herniated muscle in the forearm can cause mild to severe localized pain, affect grip, cause nerve pain or have no physical symptoms at all. Causes of documented cases include sporting or occupational activities, or an unrelated primary medical condition. Patients usually have a swollen mass that increases in size when the affected muscle is engaged and decreases when the muscles are relaxed. One differential diagnosis for a herniated forearm muscle is a tumor. Muscle herniation in the forearm typically affects males in their adolescent or young-adult years1. We report a case of a disappearing forearm nodule that appeared with muscle contraction. This is characteristic of a transfascial muscle hernia. Ultrasound and MRI are the key to identifying an area of fascial alteration. Treatment alternatives of this unusual condition are discussed.

Evaluación por imágenes del diafragma en el niño / Image evaluation of diaphragm in children

The anatomy, embriology andfunctions of the neonate 's diaphragm, as well as its anatomical and functional oddities were reviewed. And, in a deeper way, imaging studies were also reviewed; these have an important role in functional and anatomic evaluation of the diaphragm, each one with its own advantages and limitations. Chest X-rays allow an anatomic two dimensional evaluation of the diaphragm and constitutes the first approach in the study of diaphragm pathology in children; hence, the normal anatomy and the most common pathological signs are reviewed. Digestive tube studies using contrast media still are the best choice for diagnosis of hiatal hernia and of herniation through the foramen of Morgagni, where the colon is ascended. Ultrasound use is highlighted for the evaluation of diaphragmatic motility, as well as some of its advantages over fluoroscopy, which is and has been the method of choice in the diagnosis of diaphragmatic paralysis in children. Multiplanar images are the most complete method for the anatomic evaluation of the diaphragm, since they show its spatial orientation and allow the detailed evaluation of those pahologies where the anatomy is altered, such as diaphragmatic hernias, trauma and tumors.

Revisamos la embriología, anatomía y funciones del diafragma, sus particularidades anatómicas y funcionales en los neonatos y, en forma más profunda, los estudios por imágenes, que en la actualidad tienen un importante rol en su evaluación anatómica y funcional, cada uno de ellos con sus ventajas y limitaciones. La radiografía de tóraxpermite una evaluación anatómica en dos planos del diafragma y constituye la primera aproximación en el estudio de la patología del diafragma en los niños, por lo que se describe la anatomía normal y los signos de las patologías más frecuentes. Los estudios contrastados del tubo digestivo siguen siendo de elección para el estudio de las hernias hiatales y hernias de Morgagni en donde está ascendido el colon. Destacamos el uso del ultrasonido en la evaluación de la motilidad diafragmática, y algunas de sus ventajas sobre la fluoroscopia, que es y ha sido el método de elección en el diagnóstico de la parálisis diafragmática en los niños. Las imágenes multiplanares son un método más completo en la evaluación anatómica del diafragma, muestran mejor su orientación espacial y permiten la evaluación detallada de las patologías donde la anatomía está alterada, como hernias diafragmáticas congénitas, trauma y tumores.

Presentación atípica de piomiositis tropical difusa de psoas por Staphylococcus aureus meticilino resistente / Atypical presentation of diffuse tropical pyomiositis of the psoas due to methicillin resistant Staphylococcus aureus

La piomiositis tropical difusa primaria es una enfermedad de presentación infrecuente en nuestro medio, con pocos casos asociados a Staphylococcus aureus meticilino resistente, adquirido en la comunidad (MRSA-AC). Se presenta el caso de un paciente de 70 años, con tratamiento irregular para diabetes mellitus tipo 2, que fue hospitalizado por presentar un cuadro de diez días de evolución, con dolor lumbar irradiado a miembro inferior izquierdo, fiebre y flexión forzada de la cadera derecha por dolor a la movilización. El diagnóstico de piomiositis difusa de ambos psoas se realizó con resonancia magnética. Del cultivo de una colección paravertebral posterior se aisló Staphylococcus aureus resistente a oxacilina, penicilina y dicloxacilina.

Diffuse tropical primary pyomyositis is an infrequent entity in our country, with few cases associated to communityacquired Methicillin- resistant Staphylococcus aureus. There are no reported cases of Community-Acquired Methicillin- Resistant Staphylococcus aureus (CA- MRSA) in Peru. We present the case of a 70 year old male with a previous diagnosis of type 2 diabetes mellitus, receiving irregular treatment, who was admitted to the hospital with a history of 10 days of low back pain radiating to the left leg, fever and forced flexion of the right hip due to pain during movement. The diagnosis of diffuse pyomyositis of both psoas muscles was performed with MRI and culture of a posterior paravertebral collection, from which Staphylococcus aureus resistant to oxacillin, penicillin and dicloxacillin was isolated.

Evolución de los signos cerebelosos, la paratonía y las sincinesias en el niño / Evolution of cerebellar signs, the paratonia and the child synkinesias

Objetivo: estudiar a través del examen neurológico evolutivo, la maduración de los signos cerebelosos, la paratonía y las sincinecias en el niño. Método: se realizó un estudio de tipo transversal, examinándose niños de 4 a 12 años de edad, de ambos sexos, de escuelas públicas de Montevideo, entre octubre 2006-mayo 2008. Resultados: se examinaron 330 niños, 178 varones y 152 niñas. La paratonía en maniobra de caída del brazo, estuvo presente en el 70% a los 4 años y en el 17% a los 11 años. A los 7 años el 100% de los niños lograron el equilibrio estático en un pie y el 92.5% el equilibrio en el salto en un pie. La marcha punta-talón la lograron el 96.2% de los niños de 6 años de edad. El 79% de los niños de 7 años lograron la metría en la maniobra índice-nariz. La diadococinesia en la maniobra palma-dorso fue lograda en el 100% de los niños de 6 años de edad y la maniobra de marionetas en el 100% de los niños de 9 años. Las sincinesias de imitación estuvieron presentes en el 80% a los 4 años y menos del 20% a los 11 años. La maniobra de oposición de dedos al pulgar la lograron el 92,5% de los niños de 8 años de edad. Conclusiones: se constata que el desarrollo motriz presenta etapas secuencialmente ordenadas, donde los cambios más significativos en la maduración de los sistemas motores ocurre entre los 5 y 6 años de edad.

Miopatía necrotizante autoinmune (NAM): a propósito de un caso clínico / Necrotizing autoimmune myopathy (NAM): a propos of a clinical case

La miopatía inducida por estatinas es una reacción adversa que limita el uso de estos fármacos. Si bien su incidencia es baja, puede asociarse a cuadros severos e invalidantes. Habitualmente la miopatía es autolimitada y mejora con la suspensión del hipolipemiante, pero se han descrito casos en que el daño es persistente. en muchos de estos casos, la biopsia ha permitido demostrar una nueva entidad, denominada Miopatía Necrotizante Autoinmune (NAM) por estatinas, la cual se relaciona a una sobreexpresión del complejo mayor de histocompatibilidad tipo I y a la presencia de anticuerpos anti-HMGCoA reductasa. La NAM inducida por estatinas es una condición que responde al tratamiento esteroidal e inmunosupresor, pero puede dejar importantes secuelas. Por tal razón, su detección precoz y adecuado tratamiento resultan fundamentales. Presentamos el caso clínico de una mujer con esta entidad que refleja la dificultad diagnóstica y terapéutica.

Statins induced myotoxicity, constitutes sometimes a major barries to the use of these drugs. Although a low incidece, may be associated with severe disease and disability. Usually this myopathy is self-limited and improves with the removal of lipid-lowering agent, but has been reported patients with persistent disease. In many of this cases, the muscle biopsy has demonstrated a new entity called autoimmune necrotizing myopathy (NAM) for statins, which is characterized by an up-regulation of major histocompatibility complex type I (MHC I) and the presence of anti-HMGCoA reductase antibodies. NAM caused by statins in difficult to manage and can cause important damage. by this reason, early detection and treatment is crucial. We report a case of a woman with this condition, which reflects the difficulty in diagnosis and therapy.

Recurrent diabetic myonecrosis: A rare complication of a common disease.

Spontaneous aseptic diabetic muscle infarction (DMI) is one of the rare complications of diabetes. We report a case of type 2 diabetes mellitus with advanced microvascular complications presenting with severe muscular pain. She was diagnosed as DMI on the basis of clinical presentation, radiological and histopathological investigations. She was managed conservatively. During 18 months of follow up, she had good improvement but subsequently other muscle groups were involved suggesting recurrent DMI.