Miositis viral aguda en un paciente pediátrico con Covid-19: a propósito de un caso / Acute viral myositis in a pediatric patient with Covid-19: about a case

Se presenta el reporte de un caso de miositis aguda en un niño de 4 años de edad con COVID-19. El paciente manifestó fiebre y dolor en ambas pantorrillas. Con sospecha de miositis se realizó análisis de CPK, encontrando valores de 4460 UI/L. Asimismo se realizó hisopado nasofaríngeo para SARS-CoV 2, confirmando la infección. El paciente recibió hiperhidratación, presentando resolución de su cuadro clínico en menos de 5 días

This case report describes a 4 year old child with COVID-19. He presented with fever and pain in both calves. Under the suspicion of myositis a CPK analysis was performed, which showed CPK: 4460 UI/L. Nasopharyngeal RT - PCR was also performed, which was positive. As a treatment, the patient received hyperhydration, achieving full recovery after five days

Miopatía necrotizante autoinmune: Presentación de un caso clínico / Autoimmune necrotizing myositis: Presentation of a clinical case

Las miopatías inflamatorias (MI) son un grupo heterogéneo de enfermedades musculares de rara ocurrencia, caracterizadas por inflamación de los distintos componentes del tejido muscular, ya sea de forma aislada o, más comúnmente, en el contexto de una afección sistémica. Las miopatías necrotizantes inmunomediadas (MNIM) constituyen un subtipo de miopatía inflamatoria caracterizada por debilidad muscular proximal, necrosis de miofibrillas con mínimo infiltrado celular inflamatorio en la biopsia muscular e infrecuente compromiso extramuscular asociado1. Si bien existen similitudes clínicas e histopatológicas, el espectro de las miopatías inflamatorias es considerablemente variable. Por este motivo, es fundamental realizar estudios complementarios para la identificación correcta del subtipo de MI a fin de determinar su pronóstico e implementar un adecuado tratamiento. Se presenta el caso de una paciente de 29 años, sin antecedentes personales y heredofamiliares de enfermedad autoinmune ni antecedentes patológicos relevantes, que consulta a la Guardia Médica de nuestra Institución por un cuadro de dolor e impotencia funcional en los cuatro miembros, con debilidad muscular a predominio de cintura escapular y en menor medida pelviana, acompañado de astenia, tendencia al sueño e hiporreactividad.

Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement1. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis. The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological background. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

Long-term rituximab treatment of refractory idiopathic inflammatory myopathy: A report of 3 cases / 北京大学学报(医学版)

Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.

Neumonía y miositis aguda benigna de la infancia por Mycoplasma pneumoniae asociado a encefalitis: reporte de caso / Pneumonia and benign acute myositis of childhood due to Mycoplasma pneu-moniae associated with encephalitis: a case report

Una de las principales causas de neumonía en niños entre 5 y 15 años es el Mycoplasma pneumoniae, una bacteria que causa manifestaciones clínicas atípicas como la miositis y encefalitis. Reportamos un caso de una niña de cinco años que presentó limitación funcional en extremidades inferiores precedida por una infección respiratoria superior. Posteriormente, se complicó con neumonía y encefalitis. Se administraron antibióticos y antivirales debido al deterioro clínico del paciente. La serología de inmunoglobulinas para Mycoplasma pneumoniae fue positiva; mientras que los demás estudios virales fueron negativos. El curso clínico fue favorable con disminución progresiva de la dificultad respiratoria, trastorno del sensorio y mejoría en la limitación funcional en las extremidades inferiores a los 15 días de tratamiento.

One of the leading causes of pneumonia in children between 5 to 15 years is Mycoplasma pneumoniae, a bacterium that causes atypical clinical manifestations such as myositis and encephalitis. We report a 5-year-old girl who presented functional limitations of the lower extremities preceded by an upper respiratory infection. Later on, she developed pneumonia and encephalitis. Antibiotics and antivirals were administered due to the clinical deterioration of the patient. IgM serology for Mycoplasma pneumoniae was positive, while the other viral studies were negative. The clinical course was favorable with a progressive decrease in respiratory distress, sensorial disorder, and improvement in the functional limitations of the lower limbs after 15 days of treatment.

Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo / Idiopathic inflammatory myopathies: a review

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.

Topical action of Buriti oil (Mauritia flexuosa L) in myositis induced in rats

Abstract Purpose: To analyze the topical effects of Buriti oil (Mauritia flexuosa L.) in induced myositis in rats. Methods: Thirty six male rats divided into three groups: Control group (C), induced myositis group (MI) and induced myositis group reated with Mauritia flexuosa L. (MT). After inducing myositis with 1% acetic acid, was topically applied 0.5 ml of Mauritia flexuosa L.extract on the posterior region of the right gastrocnemius muscle in animals belonging to group MT, for 7 and 14 days. Results: The neutrophil number there was statistically significant difference, after 7 and 14 days, between groups C and MI (p <0.001) (p<0.01). The group MT there was a significant difference in relation to MI group in both experimental times with (p<0.001). The number of fibroblasts in the 14 days showed that when comparing the groups M and MT the differences were also significant (p<0.001). As for the DLL, in 7 days, there was a significant difference between group C and MI group (p <0.001). When considering the MT group, there was a significant difference in relation to the MI group (p <0.001). Conclusion: The extract of Mauritia flexuosa L. leaves lessened acute and chronic inflammation, increased fibroblast proliferation and reduced macroscopically edema.

Clinical applications of immunoglobulin in neuromuscular diseases: focus on inflammatory myopathies / Aplicações clínicas de imunoglobulina em doenças neuromusculares com foco em miopatias inflamatórias

During recent years, an increasing number of neuromuscular diseases have been recognized either to be caused primarily by autoimmune mechanisms, or to have important autoimmune components. The involved pathophysiological mechanisms and clinical manifestations have been better recognized and many of these disorders are potentially treatable by immunosuppression or by immunomodulation with intravenous immunoglobulin (IVIg). IVIg has been tried in a variety of immune-mediated neurological diseases, being target of widespread use in central and peripheral nervous systems diseases. Objective To give an overview of the main topics regarding the mechanism of action and different therapeutic uses of IVIg in neurological practice, mainly in neuromuscular diseases. .

Nos últimos anos, um número progressivo de doenças neuromusculares passaram a ser reconhecidas tanto por ser causadas por mecanismos autoimunes ou por envolver importantes componentes autoimunes. Os mecanismos fisiopatológicos e as manifestações clínicas envolvidos têm sido mais bem reconhecidos e muitas de tais doenças são potencialmente tratáveis por imunossupressão ou imunomodulação com imunoglobulina intravenosa (IVIg). IVIg vem sendo utilizada em uma variedade de doenças neurológicas imunomediadas, sendo alvo de amplo uso em doenças dos sistemas nervosos central e periférico. Objetivo Oferecer uma visão global sobre os principais tópicos relacionados aos mecanismos de ação e aos diferentes usos terapêuticos da IVIg na prática neurológica, principalmente em doenças neuromusculares. .

Miopatía inflamatoria con compromiso inicial de músculos respiratorios y artritis reumatoidea

Las miopatías inflamatorias constituyen un grupo heterogéneo de enfermedades musculares adquiridas de presentación subaguda, crónica y a veces aguda. Las entidades clínicas más frecuentes son la dermatomiositis, la polimiositis, la miositis necrotizante autoinmune y la miositis por cuerpos de inclusión. Suelen presentarse con debilidad muscular con predominio proximal y simétrica, pero rara vez comprometen los músculos respiratorios. Presentamos el caso de una mujer de 39 años con miopatía inflamatoria inespecífica que presentó insuficiencia respiratoria secundaria a hipoventilación alveolar por debilidad muscular y requirió asistencia respiratoria mecánica. Respondió favorablemente y de forma rápida tras el tratamiento instaurado con inmunosupresores (corticoides y metotrexato) e inmunoglobulina humana endovenosa. Se utilizó ventilación no invasiva como alternativa a la intubación orotraqueal con adecuada tolerancia.

Inflammatory myopathies comprise a heterogeneous group of subacute, chronic and sometimes acute acquired muscle diseases. The most common inflammatory myopathies seen in practice can be separated into four distinct subsets: polymyositis, dermatomyositis, necrotizing autoimmune myositis and inclusion body myositis. These disorders present as proximal and symmetric muscle weakness but rarely respiratory muscles may also be affected. We report the case of a 39 year-old female with inflammatory myopathy with acute respiratory failure due to alveolar hypoventilation secondary to respiratory muscle dysfunction that required mechanical ventilation. The treatment with steroids, methotrexate and intravenous immune globulin was successful as well as the implementation of non-invasive ventilation as an alternative to endotracheal intubation.

Primary tuberculous myositis: a rare clinical entity.

Summary: Primary tuberculous myositis without underlying pathology has been sparingly reported in medical literature. We report a case of primary tuberculous myositis of left upper arm in a seven-year-old boy. He presented with gradually increasing swelling on the medial aspect of the left arm. Ziehl Neelsen staining of pus collected revealed acid fast bacilli morphologically resembling Mycobacterium tuberculosis and the same was grown on the culture. Histopathological findings were consistent with tuberculosis. The results were confirmed by Genotype MTBDRpluse line probe assay. He was treated with standard four-drug regimen to which he responded well with complete resolution of the lesion.

Enfermedad pulmonar intersticial como manifestación inicial de síndrome antisintetasa / Interstitial lung disease as initial manifestation of anti-synthetase syndrome

Se reporta el caso de un paciente que presentó un síndrome antisintetasa en el cual la miopatía fue precedida por EPID grave de instalación rápida y buena respuesta al manejo inmunosupresor con corticosteroides, ciclofosfamida y azatioprina. El cuadro clínico inicial con fiebre, infiltrados pulmonares, SDRA y ausencia de miopatía fue muy sugestivo de infección.Palabras clave: miopatía inflamatoria, enfermedad pulmonar intersticial, síndrome antisintetasa.

We report a patient with antisintetase síndrome with rapid and progressive interstitial difuse pulmonary disease preceding the muscular weakness. The patient was successfully treated with steroids, cyclophosphamide and azathioprine. The initial compromise: fever, dispnoea and pulmonary infiltrates without miopathy was misdiagnosed as pneumonia.Key words: inflamatory miopathy, interstitial pulmonary disease, antisintetase syndrome.

Mlasa syndrome: a case report

Mitochondrial myopathy, lactic acidosis, and siderobiastic anemia [MLASA] syndrome is a rare autosomal recessive disorder of oxidative phosphorylation and iron metabolism. The association between myopathy and siderobiastic anemia was initially reported in 1974. Here we report an 8.5 year old boy with normal cognitive function, suffering from chronic progressive weakness in his lower extremities, inability to walk and palor, Microcytic siderobiastic anemia, mild lactic acidosis and inflammatory myopathy [myositis] in muscle biopsy was detected and treated; the response to corticosteroid therapy and rehabilitation was excellent and the patient was ambulatory after four months

Caracteríticas de las miopatías inflamatorias idiopáticas en la población mexicana / Idiopathic inflammatory myophaty: characteristics in the Mexican population

La mayoría de las descripciones epidemiológicas y clínicas respecto a las miopatías inflamatorias idiopáticas (MII) corresponden a población anglosajona y existen muy pocos estudios sobre las características de este grupo de padecimientos en la población mestiza mexicana. En este estudio se analizaron las características de los pacientes con diagnóstico de MII atendidos en el Servicio de Reumatología del Centro Médico Nacional 20 de Noviembre del Instituto de Seguridad y Servicio Social para Trabajadores del Estado de enero 1996 a julio de 1997. Fueron incluidos 42 pacientes con diagnóstico de MII, 34 del sexo femenino y ocho del masculino, todos de raza mestiza. La MII se clasificó como polimiositis en 12 casos, dermatomiositis en 22, juvenil en seis y asociada a otra enfermedad de tejido conectivo en dos. La edad promedio de los grupos adultos fue 33.3 años y en el grupo juvenil fue de siete años. Un gran porcentaje de pacientes (84 por ciento) refirió síntomas inespecíficos previos a la aparición de debilidad muscular o alteraciones cutáneas típicas. La evolución del padecimiento fue limitada en 15 enfermos, con exacerbaciones y remisiones en 23 y progresiva en cuatro. Se encontró mayor frecuencia de MII tipo II que lo reportado en población anglosajona, que se caracterizó además por inicio a edad temprana, evolución más agresiva y peor pronóstico en relación con los otros tipos. Del total de pacientes, sólo el 19 por ciento remitió con esteroides, el resto requirió de la adición de uno o más inmunosupresores. Se observó relación estadísticamente significativa entre el menor tiempo de evolución al iniciar el tratamiento y la obtención de remisión

Tetraparesia fláccida / Flaccid tetraparesis

In this paper we review a clinical case of a man of 66 years old admitted to the medicine service of the Clinical Hospital of the University of Chile, with a clinical setting of flaccid tetraparesis associated with myalgias, dysphagia, dysphonia, reddish-violaceous eruption on the upper eyelids, periungual erythema and erythema distributed over the anterior neck and chest. We take this case to review the cases of flaccid tetraparesis that can be due to systemic, neuromuscular or psychiatric diseases. The clinical approach begins with the anamnesis: drugs consumption, or systemic diseases; continues with the physical examination orientated to stablish compromise of first or second motoneuron. Finally laboratory test as muscle enzymes, endocronologic test, and others like electromyography and muscle biopsy. We also review the actual tratments and the outcome and prognosis with each of them

Enfermedad invasiva por Strptococcus ß-hemolítico grupo A: experiencia de una unidad de cuidados intensivos polivalente / Invasive group A streptococcus B-hemolytic disease: experience of an intensive care unit polyvalent

Se presentan 4 casos de síndrome de shock tóxico asociados a infección por Streptococcus beta-hemolítico grupo A (Streptococcus pyogenes). Todos presentaron hipotensión y disfunción orgánica. Un caso se presentó en forma típica con fascitis necrotizante. Otro paciente tuvo una forma eritrodérmica con posterior descamación asociado a faringitis no invasiva. Una sepsis puerperal con neumonía cavitada y empiema; y un niño con faringitis y bacteremia. Se discute clínica, teorías fisiopatológicas, epidemiología y tratamiento

Piomiositis de interaductores de cadera

La piomiositis es una infección del músculo esquelético, considerada no muy frecuente en áreas templadas pero sí en zonas tropicales. Sin embargo, en nuestra institución es una patología infecciosa poco usual, por tal razón, presentamos el caso de un paciente escolar hospitalizado en el servicio de infectología de la Clínica Infantil de Colsubsidio entre el 4 y 23 de septiembre de 1994 y en seguimiento posterior ambulatorio por infectología y ortopedia, en quien se llegó al diagnóstico de piomiositis de interaductores de cadera izquierda como foco primario; asociado luego a una osteomielitis de tibia derecha debidos a Staphylococcus aureus. El paciente tuvo una evolución favorable después del drenaje quirúrgico. No fue fácil establecer el diagnóstico definitivo sino después de cinco días de un gran consumo de recursos humanos, técnicos y económicos, así como de una gran expectativa del personal médico, paramédico, de la familia y del mismo paciente con el fin de tomar una conducta apropiada y oportuna que favoreciera el pronóstico del paciente. Nuestro objetivo es el de ilustrar con un caso una patología infecciosa no muy frecuente en nuestra institución, a pesar de estar en un área tropical, pero que debe considerarse en el diagnóstico diferencial del estudio del paciente con cuadro febril asociado a mialgias o limitación funcional sin un foco claro y el cual, tratado oportuna y adecuadamente, puede evitar complicaciones tardías y pronóstico reservado

Piomiosite tropical em pacientes hígidos em regiäo de clima temperado: relato de dois casos / Tropical pyomyositis in healthy patients in a temperate region: report of 2 cases

Piomiosite, infecçäo bacteriana dos músculos estriados, é uma doença comum nos trópicos, pouco descrita em climas temperados, apresenta-se em 90 por cento dos casos no momento do diagnóstico, com edema importante e sensibilidade muscular na área afetada e que é causada, na grande maioria dos casos, por Staphylococcus aureus. A doença atinge principalmente pacientes jovens saudáveis do sexo masculino. Neste relato, a condiçäo foi diagnosticada em dois jovens do sexo masculino sem antecedentes mórbidos e que tiveram rápida progressäo para sepse e choque séptico. Ambos receberam suporte hemodinâmico e tratamento cirúrgico e clínico adequados, mas um dos casos evoluiu para óbito. O diagnóstico foi confirmado por tomografia computadorizada das áreas afetadas e o organismo isolado nos abscessos foi o esperado (Staphylococcus).O tratamento preconizado e, nestes casos, realizado foi a drenagem dos abscessos e uso adequado de antibióticos, além do suporte hemodinâmico através de monitorizaçäo do transporte de oxigênio e adequado suporte nutricional.