Association between muscle strength and risk factors for metabolic syndrome in children and adolescents: a systematic review.

The aim of this systematic review was to identify and summarize evidence for the association between muscle strength (MS) and metabolic syndrome (MetS), and MS and combinations of risk factors for MetS in children and adolescents. Five databases (Medline/PubMed, EBSCO, Scielo, Scopus, and Web of Knowledge) were searched up to November 2019 with complementary reference list searches. Inclusion criteria were studies that investigated the relationship between MS and MetS or MS and combinations of risk factors for MetS in children and adolescents (≤19 years of age). Risk of bias was assessed using standard procedures. From the total of 15,599 articles initially identified, 13 articles were included, representing 11,641 children and adolescents. Higher MS values were associated with lower risk for MetS or combinations of risk factors for MetS (n=11/13 studies). Of the total of included studies, about 23.1% (03/13) were longitudinal and all included studies were classified as having a moderate risk of bias. This review provides preliminary evidence for a beneficial relationship between MS and MetS among children and adolescents. Additionally, although the body of evidence points to the beneficial relationship between higher MS and lower risk for combination of factors for MetS in children and adolescents, this relationship is inconclusive.

Avances en el empleo del ultrasonido muscular en miopatías inflamatorias / Advances in the use of muscle ultrasound in inflammatory myopathies

Aunque los métodos de imágenes aún no se han introducido en los criterios de clasificación de la miositis, el uso de imágenes musculares en la evaluación de las miopatías inflamatorias idiopáticas (MII) ha crecido a lo largo de los años. Las diferentes técnicas de imagen han demostrado ser útiles, pero la RM sigue siendo el estándar de oro para la imagen muscular. Sin embargo, el alto costo y las contraindicaciones en algunos pacientes, hacen considerar otros métodos como el ultrasonido muscular. Esta revisión tiene el objetivo de ofrecer una visión general de las diferentes técnicas de ultrasonido que se han estudiado y proporcionar información a los reumatólogos sobre el papel actual del ultrasonido para diagnosticar las MII. Existen diversos factores que pueden influir en la medición de los parámetros musculares: estandarizar la configuración de la máquina, el plano de visión y la posición del paciente, ya que pueden afectar la medición de la intensidad del eco y el grosor muscular. En el caso de las miopatías inflamatorias influyen otros factores en los resultados de la imagen: sexo, edad, escala de grises de la imagen, cambios en la calidad muscular, intensidad del eco, grosor, tamaño y ecogenicidad muscular. La ecografía muscular es una herramienta próxima en la evaluación de los trastornos neuromusculares y las miopatías inflamatorias. Es fácilmente aplicable en diversos entornos clínicos, no tiene contraindicaciones y proporciona una alternativa rentable a otras modalidades de imágenes como la resonancia magnética(AU)

Although imaging methods have not yet been introduced into the myositis classification criteria, the use of muscle imaging in the evaluation of IIMs has grown over the years. Different imaging techniques have proven helpful, but MRI remains the gold standard for muscle imaging. In this review, the objective is to provide an overview of the different ultrasound techniques that have been studied and to provide information to rheumatologists about the current role of ultrasound in the field of IIM. Development: There are multiple factors that can influence the measurement of muscle parameters that must be considered. First, it is important to standardize the machine configuration, the plane of vision and the position of the patient, as these can affect the measurement of echo intensity and muscle thickness. Conventionally, a linear ultrasound probe is used with sufficient frequency (at least 6-12 MHz) to obtain images of the peripheral skeletal muscle. Magnetic resonance imaging remains the gold standard for muscle imaging. However, the role as a diagnostic tool in the field of IIMs has grown over the years, and the promising results of new advanced imaging techniques suggest that it has not yet reached its full potential(AU)

Síndrome de Hoffmann, manifestación de hipotiroidismo: presentación de un caso / Hoffmann syndrome, manifestation of hypothyroidism: presentation of a case

Fundamento: el síndrome de Hoffmann es definido como la combinación de hipotiroidismo con miopatía, rigidez, calambres e hipertrofia muscular. Dicha forma de miopatía tiroidea es rara y por lo general acompaña a los pacientes con hipotiroidismo severo y de larga evolución. Objetivo: describir un caso con características clínicas, hormonales y musculares de un síndrome de Hoffmann, como manifestación excepcional del hipotiroidismo. Caso clínico: paciente femenina, de 16 años de edad comienza a sentir molestias musculares dadas por dolores y fatiga, las cuales se incrementaron de manera progresiva, las mialgias se tornaron intensas al punto que no le permitían realizar esfuerzo físico alguno, con astenia marcada y luego se agregaron contracciones musculares dolorosas, lo que la motivó asistir a consulta médica. Asociadas a estas manifestaciones se encontraron niveles muy elevados de enzimas musculares. Se comprueba aumento de volumen de la glándula tiroides, así como síntomas y signos sugestivos de hipotiroidismo, el cual se confirma tras dosificaciones de la tirotropina, tiroxina y triyodotironina. Conclusiones: el caso que se presentó es característico del síndrome de Hoffmann, cuya historia clínica detallada y meticulosa evidenció la presentación del hipotiroidismo(AU)

Background: Hoffmann syndrome is defined as the combination of hypothyroidism with myopathy, rigidity, cramps and muscle hypertrophy. This form of thyroid myopathy is rare and usually accompanies patients with severe and long-evolving hypothyroidism.Objective: to describe a case with clinic, hormonal and muscle characteristics of a Hoffmann syndrome, as an exceptional manifestation of hyperthyroidism. Clinical case:16-year-old, female patient begins to feel muscular discomfort due to pain and fatigue, which gradually increased, the myalgia became intense to the point that did not allow any physical effort, with marked asthenia and later contractions were added painful muscles, motivating her attendance to medical consultation. Associated with these manifestations were very high levels of muscle enzymes. Increased thyroid volume and symptoms and signs suggestive of hypothyroidism are confirmed, which is confirmed after dosages of thyrotropin, thyroxine and triiodothyronine. Conclusions: the presented case constitutes a characteristic example of the Hoffmann syndrome, which thanks to a detailed and meticulous clinical history showed the presentation of hypothyroidism(AU)

Safety of statin drugs in patients with dyslipidemia and stable systemic autoimmune myopathies.

Recent studies have shown a high prevalence of dyslipidemia in patients with systemic autoimmune myopathies (SAM). However, little is known about the safety of the use of statins in these patients, and this gap in research motivated the accomplishment of the present study. In a retrospective cohort study conducted from 2004 to 2018, 250 patients with SAM were evaluated, and 24 patients had stable forms of SAM (16 dermatomyositis, 1 polymyositis and 7 antisynthetase syndrome) but had dyslipidemia and had received statins. Patients with clinically amyopathic dermatomyositis, immune-mediated necrotizing myopathy, dermatomyositis, or polymyositis induced by statins were excluded. The mean age of the patients was 50.6 years, and they were predominantly women. The median duration of the disease was 5.0 years. Twelve patients received simvastatin (10-60 mg/day), and 11 patients received atorvastatin (20-40 mg/day), and 1 patient received atorvastatin (10 mg/day) which was later replaced by simvastatin (20 mg/day). The median time of exposure to the statin was 22.5 months. The follow-up appointments showed that the patients' lipid profiles had improved and that there had been no recurrences of disease activity or clinical intercurrences. Despite the small sampling, the data showed that the use of statins in patients with SAM was safe. New studies with a larger sample and patients with different degrees of disease activity are necessary to corroborate the results of the present study.

Postural alterations as a risk factor for temporomandibular disorders.

The aims of this study were to estimate frequency and assess postural alterations as a risk factor for temporomandibular disorders (TMD). Patients aged 10 to 15years (N=243, mean age 12. 6) seeking comprehensive dental care were analyzed according to RDC/ TMD. For static postural assessment, photographs were taken and analyzed by a physiotherapist following Kendall's model. Patients were divided into: A: without TMD (n=133); B: with muscle disorders (n=61); C: with disk displacement (N=49). No difference in age wasobserved between groups (p=0. 95). Significant association was observed between muscular TMD and alterations in spinal curves, head posture, and lower limbs: OR: 3. 40, 2. 44 and 2. 22 respectively. The most frequent types were hyperlordosis 23. 30 and 32. 78%; forward head posture 39. 85 and 52. 45%; and genu valgum 33. 08 and 45. 90% in A and B respectively. Alterations in head posture, vertebral curves and lower limbs could be considered risk factors for muscular TMD. The most frequent postural alterations were lumbar hyperlordosis, forward head posture and genu valgus.

Los objetivos de este estudio fueron estimar la frecuencia y evaluar las alteraciones posturales como factor de riesgo para trastornos temporomandibulares (TTM). Se evaluaron pacientes de 10 a 15 años (N= 243, edad media 12. 6) que concurrieron para atención odontológica integral utilizandolos protocolos CDI/TTM años. La evaluación postural estática, se j realizó mediante fotografías analizadas por un fisioterapeuta siguiendo el modelo de Kendall. Los pacientes se dividieron en f diferentes grupos según el diagnóstico: A: sin TTM (n = 133); B: con trastornos musculares (n = 61) y C: con desplazamiento del disco (n = 49). No se observaron diferencias entre los grupos en la edad (p = 0, 95). Se observó asociación significativa entre TTM muscular y alteraciones en las curvas espinales, la postura de la cabeza y los miembros inferiores: OR: 3, 40, 2, 44 y 2, 22 respectivamente. Los tipos más frecuentes fueron hiper-lordosis lumbar 23, 30 y 32, 78%; anteversión cefálica 39. 85 y 52. 45%; y genu valgum 33, 08 y 45, 90% en A y B, respectivamente. Las alteraciones en la postura de la cabeza, las curvas vertebrales y los miembros inferiores podrían considerarse factores de riesgo para los TTM musculares. Las alteraciones posturales más frecuentes fueron hiperlordosis lumbar, anteversión cefálica y genu valgo.

Estudio electrofisiológico exploratorio de la posible relación entre hipovitaminosis D y disfunción muscular / 3 EXPLORATORY ELECTROPHYSIOLOGIC STUDY OF THE POSSIBLE RELATIONSHIP BETWEEN VITAMIN D DEFICIENCY AND MUSCULAR DYSFUNCTION

Introducción: La prevalencia de hipovitaminosis D es alta, afectando a jóvenes y adultos. Además de los trastornos esqueléticos originados por este déficit, se evidenció la presencia de otros trastornos, debido a la acción de la vitamina D en otros órganos y sistemas. La reducción de los niveles séricos de esta hormona se correlaciona con la presencia de un sindrome miopático que afecta a los músculos proximales de los miembros. Objetivo: Evaluar retrospectivamente los hallazgos electromiográficos de un grupo de pacientes que consultaron por debilidad muscular y/o déficit de fuerza proximal en quienes se descartaron otras causas de miopatía. Materiales y métodos: Se estudiaron 22 pacientes, con una edad media de 51 años (24-64), con un cuadro clínico compatible con miopatía, con hipovitaminosis D. Se descartaron otras causas de miopatía. Se analizaron los datos electromiográficos obtenidos durante el reposo y los hallazgos cuantitativos realizados en los músculos bíceps braquial y vasto interno en forma cruzada mediante el multianálisis de los Potenciales de unidad motora (PUM) durante el esfuerzo voluntario leve. Se compararon los siguientes parámetros: amplitudes, duraciones, número de fases y el índice "thickness" de los PUM de los pacientes evaluados, con los datos publicados por la literatura; se relacionó cada uno de esos parámetros con los niveles séricos de vitamina D. Resultados: En ninguno de los músculos evaluados se evidenció la presencia de actividad espontánea durante el reposo. Se halló una diferencia significativa (p<0.05) en todos los parámetros evaluados: amplitudes, duraciones, número de fases e índice "thickness". Ninguno de estos parámetros alterados fue correlacionado con los niveles séricos de vitamina D. Conclusión: La hipovitaminosis D se asocia con debilidad muscular proximal que se manifiesta en el examen físico neuromuscular y se confirma mediante el examen electrofisiológico utilizando el paradigma multi-MUAP de electromiografía cuantitativa. No se asocia con actividad espontánea en en reposo. Es de vital importancia su reconocimiento dado que se trata de un desorden reversible con la terapia adecuada

Introduction: the prevalence of Vitamin D deficiency is high, affecting young people and also adults. In addition to the skeletal disorders caused by this deficit, the presence of other disorders was evidenced, due to the action of Vitamin D in other organs and systems. The reduction of serum levels of this hormone correlates with the presence of a myopathic syndrome that affects the proximal muscles of the limbs. Objective: To retrospectively evaluate the electromyographic findings of a group of patients who consulted due to muscular weakness and / or proximal force deficit in whom other causes of myopathy were ruled out. Materials and methods: 22 patients were studied, with an average age of 51 years (24-64), with a clinical picture compatible with myopathy, with hypovitaminosis D. Other causes of myopathy were ruled out. We analyzed the electromyographic data obtained during rest and the quantitative findings made in the biceps brachialis and vastus medialis in a crossed way by means of the multianalysis of the Potentials of the motor unit (PUM) during the mild voluntary effort. The following parameters were compared: amplitudes, durations, number of phases and the thickness index of the PUM of the evaluated patients, with the data published by the literature; each of these parameters was related to serum vitamin D levels. Results: In none of the evaluated muscles was evidentiated the presence of spontaneous activity during rest. A significant difference (p <0.05) was found in all parameters evaluated: amplitudes, durations, number of phases and thickness index. None of these altered parameters was correlated with serum vitamin D levels. Conclusion: Hypovitaminosis D is associated with proximal muscle weakness that manifests itself in the neuromuscular physical examination and is confirmed by electrophysiological examination using the multi-MUAP quantitative electromyography paradigm. It is not associated with spontaneous activity at rest. Its recognition is of vital importance given that it is a reversible disorder with the appropriate therapy

[Eyelid retraction of neurologic origin: Report of three cases]. / Retracción palpebral de causa neurológica: Presentación de tres pacientes, interpretación patogénica.

Eyelid retraction, has received limited attention and it has passively been interpreted as the result of an overactive levator palpebrae superioris muscle secondary to midbrain injury. However, eyelid retractions can occur in other neurological diseases, not directly related with the midbrain. We report three patients who developed eyelid retraction. One patient had a bilateral eyelid retraction, related with Creutzfeldt-Jakob disease (CJD). Another patient had a unilateral right eyelid retraction associated with a thalamic-mesencephalic infarct. The third patient had a bilateral pontine infarction on magnetic resonance imaging. In the patient with CJD, eyelid retraction did not subside. Among patients with infarctions, the retraction persisted after focal symptoms had subsided, showing an evolution that was apparently independent of the basic process. The analysis of these patients allows us to conclude that the pathogenesis of eyelid retraction includes supranuclear mechanisms in both the development and maintenance of the phenomenon. Unilateral or bilateral eyelid retraction does not alter the normal function of eyelid, which ever had normal close eye blink. In these reported cases, a hyperactivity of levator palpebrae superioris muscle was clinically ruled out.

Retracción palpebral de causa neurológica: presentación de tres pacientes, interpretación patogénica / Eyelid retraction of neurologic origin: report of three cases

Eyelid retraction, has received limited attention and it has passively been interpreted as the result of an overactive levator palpebrae superioris muscle secondary to midbrain injury. However, eyelid retractions can occur in other neurological diseases, not directly related with the midbrain. We report three patients who developed eyelid retraction. One patient had a bilateral eyelid retraction, related with Creutzfeldt-Jakob disease (CJD). Another patient had a unilateral right eyelid retraction associated with a thalamic-mesencephalic infarct. The third patient had a bilateral pontine infarction on magnetic resonance imaging. In the patient with CJD, eyelid retraction did not subside. Among patients with infarctions, the retraction persisted after focal symptoms had subsided, showing an evolution that was apparently independent of the basic process. The analysis of these patients allows us to conclude that the pathogenesis of eyelid retraction includes supranuclear mechanisms in both the development and maintenance of the phenomenon. Unilateral or bilateral eyelid retraction does not alter the normal function of eyelid, which ever had normal close eye blink. In these reported cases, a hyperactivity of levator palpebrae superioris muscle was clinically ruled out.

Beyond muscle destruction: a systematic review of rhabdomyolysis for clinical practice.

BACKGROUND: Rhabdomyolysis is a clinical syndrome that comprises destruction of skeletal muscle with outflow of intracellular muscle content into the bloodstream. There is a great heterogeneity in the literature regarding definition, epidemiology, and treatment. The aim of this systematic literature review was to summarize the current state of knowledge regarding the epidemiologic data, definition, and management of rhabdomyolysis. METHODS: A systematic search was conducted using the keywords "rhabdomyolysis" and "crush syndrome" covering all articles from January 2006 to December 2015 in three databases (MEDLINE, SCOPUS, and ScienceDirect). The search was divided into two steps: first, all articles that included data regarding definition, pathophysiology, and diagnosis were identified, excluding only case reports; then articles of original research with humans that reported epidemiological data (e.g., risk factors, common etiologies, and mortality) or treatment of rhabdomyolysis were identified. Information was summarized and organized based on these topics. RESULTS: The search generated 5632 articles. After screening titles and abstracts, 164 articles were retrieved and read: 56 articles met the final inclusion criteria; 23 were reviews (narrative or systematic); 16 were original articles containing epidemiological data; and six contained treatment specifications for patients with rhabdomyolysis. CONCLUSION: Most studies defined rhabdomyolysis based on creatine kinase values five times above the upper limit of normal. Etiologies differ among the adult and pediatric populations and no randomized controlled trials have been done to compare intravenous fluid therapy alone versus intravenous fluid therapy with bicarbonate and/or mannitol.

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.

Aerobic Exercise and Pharmacological Therapies for Skeletal Myopathy in Heart Failure: Similarities and Differences.

Skeletal myopathy has been identified as a major comorbidity of heart failure (HF) affecting up to 20% of ambulatory patients leading to shortness of breath, early fatigue, and exercise intolerance. Neurohumoral blockade, through the inhibition of renin angiotensin aldosterone system (RAS) and ß-adrenergic receptor blockade (ß-blockers), is a mandatory pharmacological therapy of HF since it reduces symptoms, mortality, and sudden death. However, the effect of these drugs on skeletal myopathy needs to be clarified, since exercise intolerance remains in HF patients optimized with ß-blockers and inhibitors of RAS. Aerobic exercise training (AET) is efficient in counteracting skeletal myopathy and in improving functional capacity and quality of life. Indeed, AET has beneficial effects on failing heart itself despite being of less magnitude compared with neurohumoral blockade. In this way, AET should be implemented in the care standards, together with pharmacological therapies. Since both neurohumoral inhibition and AET have a direct and/or indirect impact on skeletal muscle, this review aims to provide an overview of the isolated effects of these therapeutic approaches in counteracting skeletal myopathy in HF. The similarities and dissimilarities of neurohumoral inhibition and AET therapies are also discussed to identify potential advantageous effects of these combined therapies for treating HF.

Reduced ovarian reserve in patients with adult polymyositis.

Polymyositis (PM) affects female gender during reproductive age; however, there is no study assessing ovarian reserve in these patients to evaluate ovarian reserve markers in PM. Eight female patients with PM (Bohan and Peter criteria, 1975) with aged 18-40 years, followed at our tertiary centre from March 2011 to May 2014, were invited to participate. They were age-matched with 16 healthy individuals (control group). All were evaluated at early follicular phase of menstrual cycle. Follicle stimulating hormone (FSH), estradiol, inhibin B, anti-Müllerian hormone (AMH) serum levels (ELISA) and sonographic antral follicle count (AFC) were determined. PM patients and controls had comparable mean age (31.4 ± 6.5 vs. 30.7 ± 6.2 years, P = 0.946), ethnicity and socioeconomic class (P > 0.05). PM mean age of onset was 27.3 ± 6.5 years and disease duration of 6.5 ± 4.1 years. Menstrual cycles were alike in both groups with a similar frequency of age at menarche, gynaecological age, duration and length of menstrual cycle (P > 0.05). The median serum level of AMH was significantly lower in PM compared to controls [0.7(0.3-3.4) vs. 3.1(1.4-4.0), P = 0.021]. AMH levels ≤1 ng/mL (50 vs. 6.3 %, P = 0.024) and very low AFC (37.5 vs. 6.3 %, P = 0.037) were significantly in PM patients versus controls. The other hormones (FSH, inhibin B and estradiol levels) were similar between both groups (P > 0.05). The present study was the first to identify subclinical ovarian dysfunction in PM patients during reproductive ages. Further study is necessary to assess the possible role of PM-related factors that may influence the ovarian function of these patients.

Adolescent idiopathic scoliosis without limb weakness: a differential diagnosis of core myopathy?

BACKGROUND: Core myopathies are a clinically and genetically heterogeneous group of congenital myopathies with the common defined histopathological feature of focally reduced oxidative activity on muscle biopsy. It has a low incidence, however, recent articles show broad clinical spectrum, suggesting that the real incidence should be considerably larger than previously described. Due to the important association between scoliosis and paravertebral muscle imbalance, numerous authors study, by biopsy of the spinal rotator muscles, potential changes that may elucidate the etiology of adolescent idiopathic scoliosis. CASE PRESENTATION: Two patients have been followed at Spine Group of Department of Orthopedics at Federal University of São Paulo, with an initial diagnosis of idiopathic scoliosis. Both patients had clinical and radiological findings compatible with it. The patients authorized, through the Term of Consent, intraoperative biopsy of muscle multifidus from the apex of the thoracic curve on concave and convex sides. After muscle biopsy was performed a histopathological analysis. As regard to the histopathological features: in both patients were identified, the presence of core structures in extensive areas with reduced oxidative activity running along the muscle fiber. CONCLUSIONS: All patients with 'idiopathic' scoliosis deserve a careful neurological evaluation, even if they have minimal muscle symptoms in the extremities. The frequent occurrence of scoliosis in patients with CORE Myopathies, supports the thesis that the change in the paravertebral muscle fiber must be the underlying pathogenic factor in scoliosis and may help us understand the onset and progression of curves in patients previously diagnosed with idiopathic scoliosis.

Aerobic exercise training as therapy for cardiac and cancer cachexia.

Aerobic exercise training (AET) induces several skeletal muscle changes, improving aerobic exercise capacity and health. Conversely, to the positive effects of AET, the cachexia syndrome is characterized by skeletal muscle wasting. Cachexia is a multifactorial disorderassociated with other chronic diseases such as heart failure and cancer. In these diseases, an overactivation of ubiquitin-proteasome and autophagy systems associated with a reduction in protein synthesis culminates in severe skeletal muscle wasting and, in the last instance, patient's death. In contrast, AET may recycle and enhance many protein expression and enzyme activities, counteracting metabolism impairment and muscle atrophy. Therefore, the aim of the current review was to discuss the supposed therapeutic effects of AET on skeletal muscle wasting in both cardiac and cancer cachexia.

"Milk of calcium": a rare presentation of calcinosis.

Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs. During surgery, we observed the presence of whitish fluid collection suggestive of "milk of calcium".

"Milk of calcium": uma apresentação rara de calcinose / "Milk of calcium": a rare presentation of calcinosis

Algumas doenças reumáticas, como dermatomiosite juvenil (DMJ), lúpus eritematoso sistêmico juvenil (LESJ) e esclerose sistêmica (ES), podem apresentar depósitos de cálcio nos tecidos subcutâneo e muscular, lesões conhecidas como calcinoses. Extensas coleções líquidas de cálcio referidas como milk of calcium são formas raras de calcinoses presentes na DMJ. Descrevemos um paciente de 15 anos de idade, com diagnóstico de síndrome de sobreposição ou overlap (esclerodermatomiosite e LESJ), cuja ressonância magnética (RM) evidenciou coleções líquidas perimusculares em membros inferiores e que, durante procedimento cirúrgico, foi observada a presença de coleção líquida esbranquiçada sugestiva de milk of calcium.

Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs. During surgery, we observed the presence of whitish fluid collection suggestive of "milk of calcium".

[Acute muscle weakness: differential diagnoses]. / Debilidad muscular aguda: diagnósticos diferenciales.

Acute muscle weakness, a common disorder in pediatrics, can occur from impairment of any part of the motor unit, including the upper motor neuron, lower motor neuron, peripheral nerve, neuromuscular junction or muscle. It usually manifests itself as an acute or hyperacute motor disorder of progressive or rapidly progressive course. Acute muscle weakness is a neuromuscular emergency, especially if it affects the respiratory or oropharyngeal musculature. The location of the motor weakness and associated neurological signs and symptoms usually indicate the location of the lesion. The onset, speed and clinical evolution, as well as other data from the patient's history, suggest the pathophysiological differential diagnosis. Successful treatment depends on the immediate and correct differential diagnosis. This paper presents the main differential diagnosis of main neuromuscular diseases that cause acute muscle weakness in children.

TITLE: Debilidad muscular aguda: diagnosticos diferenciales.La debilidad muscular aguda, un trastorno frecuente en pediatria, puede ocurrir a partir de la afectacion de cualquier parte de la unidad motora, incluyendo la neurona motora superior, la neurona motora inferior, el nervio periferico, la union neuromuscular o los musculos. Se manifiesta en general como un trastorno motor agudo de curso progresivo o rapidamente progresivo. La debilidad muscular aguda es una urgencia neuromuscular en especial si afecta a la musculatura respiratoria u orofaringea. La localizacion de la debilidad, las señales y los sintomas asociados suelen permitir la ubicacion de la lesion. La instalacion, la velocidad de la evolucion y otros datos de la historia sugieren un diagnostico fisiopatologico diferencial. El exito en el tratamiento depende de saber establecer un diagnostico diferencial rapido y correcto. En este articulo, se presenta el diagnostico diferencial de las principales patologias neuromusculares que causan la debilidad muscular aguda en la infancia.