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)

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

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.

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.

Poland's syndrome with unusual hand and chest anomalies: A rare case report.

Poland’s syndrome is a rare congenital anomaly consisting of a unilateral absence of the pectoralis major, ipsilateral muscle, hand anomaly and occasionally associated other malformations of the chest wall and breast. Many structural and functional abnormalities have been described in association with this syndrome. We report an incidentally diagnosed case in a 27-year-old male patient who presented to us with symbrachydactyly. In addition to this, anterior depression of 2nd, 3rd and 4th ribs and bifid (forked) 5th rib was present on radiological investigations. The body of sternum was short and deformed on the right side with absence of xiphoid process. All middle phalanges were absent on right hand. It is a rare variant of Poland’s syndrome.

"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".

Trastornos musculo-esqueléticos de hombros en trabajadores de la Empresa Manufacturera S.A., Cortés, Honduras. Enero-Diciembre 2011 / Musculoskeletal disorders in workers shoulder Manufacturing Company SA, Cortes, Honduras. January to December 2011

Resalta la importancia de un programa de prevención de lesiones musculo esqueléticas de hombro en la población trabajadora, el cual debe hacer énfasis en la higiene postural, las condiciones ergonómicas, capacitaciones constantes sobre riesgo, los factores organizacionales, rotación de los trabajadores de sus puestos de trabajo y el programa de pausas activas

Como manejar os efeitos colaterais dos hipolipemiantes? / How to manage the side effects of lipid-lowering medications?

Os hipolipemiantes são medicações amplamente utilizadas no tratamento das dislipidemias, com impacto na redução da morbi-mortalidade cardiovascular. Contudo, não são fármacos isentos de efeitos colaterais. Os principais efeitos indesejáveis discutidos neste artigo são a miopatia e a hepatoxicidade. Os autores enfatizam a necessidade de cuidadosa anamnese antes de iniciar a medicação hipolipemiante, ressaltando: antecedentes clínicos, alterações metabólicas presentes, uso atual de fármacos, eventuais reações de intolerância medicamentosa, além do uso de drogas ilícitas. Faz-se necessária a estabilização dos desvios metabólicos antes da administração do hipolipemiante, por exemplo, diabetes mellitus e hipotireoidismo. Ressaltam, ainda, a importância de valorizar as queixas dos pacientes, seu diagnóstico diferencial, bem como a fármaco-vigilância em relação à interação de drogas. Deve haver monitoração clínica e laboratorial cuidadosa durante o tratamento, principalmente nos pacientes de maior risco. Com o uso cada vez mais disseminado destas medicações, além de combinações de medicações diversas, são de suma importância o reconhecimento e o correto manejo destes efeitos colaterais.

The lipid-lowering medications are widely used in the treatment of dyslipidemia and impact in reducing cardiovascular morbidity and mortality. However, these drugs are not free from side effects. The main side effects discussed in this article are myopathy and hepatotoxicity. The authors emphasize the need for careful anamnesis before starting lipid-lowering medication, considering: medical history, metabolic abnormalities, drugs currently in use, drug intolerance, and the use of illicit drugs. It is necessary to stabilize the metabolic alterations before the administration of lipid-lowering medication, for example, diabetes mellitus and hypothyroidism. It is very important to valorize patients' complaints, their differential diagnosis, as well as drug-surveillance for drug interaction. Clinical and laboratory monitoring during treatment is especially important in high risk patients. With the increasingly widespread use of these medications, and various combinations of drugs, it is extremely important to correctly recognize and manage these side effects.

Etiopathogenesis of disk displacement of the temporomandibular joint: A review of the mechanisms.

Disk displacement of the temporomandibular joint (TMJ) is a clinically important condition, showing a high prevalence in both patient and non-patient populations. Despite its clinical importance, there is incomplete understanding of the etiopathogenic mechanisms leading to disk displacement. A number of possible risk factors have been identified. This article analyzes the etiopathogenesis from both the clinical and the biomechanical viewpoints and also reviews the literature on the association between disk displacement and the main risk factors (i.e., trauma, altered disk shape and/or dynamic properties, occlusal abnormalities, steepness of the articular eminence, hyperactivity of the lateral pterygoid muscle, joint hypermobility, etc.). According to our interpretation of available data, an impairment of joint lubrication may be a common finding in cases of disk displacement, thus suggesting the need for future studies addressing both local and systemic neuroendocrine aspects influencing the friction coefficient of the TMJ. A full comprehension of the etiopathogenesis of disk displacement is far from being achieved, and clinicians must take into account this consideration when treating patients with temporomandibular disorders.

Clinico-Radiologic Findings of Entrapped Inferior Oblique Muscle in a Fracture of the Orbital Floor

A 51-year old man presented with vertical and torsional diplopia after reduction of a blowout fracture at another hospital one year ago. He had no anormalies of head position and 14 prism diopters (PD) right hypertropia (RHT) in the primary position. In upgaze no vertical deviation was found, and hyperdeviation on downgaze was 35PD. Bielschowsky head tilt test showed a negative response. Distinct superior oblique (SO) and inferior rectus (IR) underaction of the right eye was noted but IO overaction was mild on the ocular version test. Double Maddox rod test (DMRT) revealed 10-degree extorsion, but fundus extorsion was minimal in the right eye.Thin-section coronal CT scan showed that there was no fracture line on the anterior orbital floor, but a fracture remained on the posterior orbital floor. Also, the anterior part of the right inferior oblique muscle was vertically reoriented and the medial portion of the inferior oblique muscle was not traced on the coronal CT scan. The patient underwent 14 mm right IO recession and 3 mm right IR resection. One month after the surgery, his vertical and torsional diplopia were eliminated in the primary position.

Successful resuscitation of prolonged hyperkalemic cardiac arrest following succinylcholine administration in possibly undiagnosed myopathy

We report a 3 years old male with undiagnosed myopathy, who developed cardiac arrest secondary to hyperkalemia following succinylcholine administration.The patient underwent prolonged cardiopulmonary resuscitation [CPR], which ended successfully after applying external pace maker. He was discharged home eleven days after the cardiac arrest for follow up of his myopathy

Síndrome lumbociático de etiología distinta a hernia discal / Etiology of lumbosciatic syndrome different from lumbar herniated disk

La causa más frecuente de lumbociática es la hernia de disco y la patología asociada de estenosis foraminal, espondilolistesis y entesopatía de la articulación facetaria, incluyendo quistes sinoviales. Existen una serie de condiciones que pueden presentar un cuadro clínico similar, y el problema es detectar estas causas infrecuentes en un universo muy grande de pacientes con patología discal. Esto crea una situación potencialmente peligrosa, en la cual se podría interpretar la sintomatología secundaria, por ejemplo a un tumor, como producida por una hernia discal, por otro lado asintomática. En base a una historia clínica cuidadosa y al uso racional de los exámenes complementarios, se puede sospechar aquellos casos que pudieran albergar esta patología de baja incidencia, pero de gran importancia clínica. Se revisan las causas más importantes que pueden provocar un síndrome lumbociático y que deben incluirse en el diagnóstico diferencial, como el síndrome piriforme, tumores intradurales y del nervio ciático y fístulas durales.

The most frequent etiology of sciatic pain is herniation of the nucleus pulposus and associated entesopathic diseases, including synovial cysts. There are several conditions that can present with a similar clinical picture, and the clinician is confronted with the problem of detecting this infrequent occurrences. This creates a potentially dangerous condition of thinking that an asymptomatic disc herniation is causing the symptoms that are originated higher by a tumor for example. With a careful history and judicious use of ancillary examinations, specially NMR, most of the cases can be suspected. The principal causes of non-discal sciatica are reviewed, including piriform syndrome, tumors of the spine and sciatic nerve, and dural fistulae.

Amiloidose manifestando-se com pseudo-hipertrofia muscular / Amyloidosis presenting as muscle pseudohypertrophy

Amiloidose tipo AL é uma doença rara causada pela deposição extracelular de fragmentos de cadeias leves monoclonais em órgãos e tecidos. Pode apresentar-se com uma ampla variedade de sinais e sintomas, e o acometimento cutâneo-muscular, simulando pseudo-hipertrofia muscular, é um achado muito raro. São descritos dois casos que apresentaram tal manifestação. CASO 1 - Mulher, 61 anos, há quatro meses com história de mialgia e aumento da massa muscular nas cinturas pélvica, escapular e região cervical. Não havia alterações significativas ao exame físico, exceto aparente hipertrofia muscular difusa e discreta macroglossia. CASO 2 - Homem, 51 anos, há dois anos com cansaço e espessamento cutâneo progressivo do dorso, pescoço e braços. Em outros serviços levantou suspeitas diagnósticas de esclerodermia ou de escleredema de Buschke; desde fevereiro de 2007 passou a ser acompanhado nesse serviço e referia, havia cerca de um ano, disfagia para sólidos, disartria e dificuldade para movimentar a língua. Chamava atenção em seu exame o porte físico atlético com musculatura torácica proeminente, porém referia não fazer exercícios físicos. Em ambos os casos, a biópsia cutânea foi realizada com identificação do depósito amilóide por meio da coloração de vermelho congo.

AL amyloidosis is a rare disease secondary to extracellular deposition of light chains fragments in organs and tissues. It can cause a wide variety of signs and symptoms, being the muscular pseudohypertrophy form a very rare finding. CASE 1 - a 61-year-old female had a history of myalgia and increase of muscular mass on pelvic and scapular girdle and cervical region. Besides the generalized muscular hypertrophy and discrete macroglossia, the rest of physical examination was normal. CASE 2 - a 51-year-old male complained of tiredness and progressive cutaneous thickening on his thorax, neck and arms for the last two years. Initially, he was misdiagnosed with either scleredema of Buschke or scleroderma. In February 2007 he was referred to our service, reporting symptoms of dysphagia and difficulty to move his tongue. On physical examination, besides the skin thickness, there was an evident muscle hypertrophy out of proportion the reported exercise practice. In both cases, subcutaneous biopsy was undertaken which revealed amyloid deposit by congo red dye.

Pseudo-obstrucción intestinal por miopatía visceral esporádica / Intestinal pseudo-obstruction due to sporadic visceral myopathy

We report an unusual case of a patient with sporadic visceral myopathy and involvement of the entire gastrointestinal and urinary tract. Visceral myopathy is a form of chronic idiophatic intestinal pseudo-obstruction characterized by vacuolar degeneration, atrophy and fibrosis of the intestinal propia muscle layer without inflammatory cells. It can be found in childhood and adolescence affecting the gastrointestinal and urinary visceral muscle. The familial occurrence can be found in about 30% of cases and the mode of transmission is autosomal recessive in most families. It is crucial to exclude secondary forms of chronic intestinal pseudo-obstruction and to obtain full thickness intestinal biopsy for the diagnosis. Surgical treatment is only beneficial in cases with isolated segmental involvement of the gastrointestinal tract.

Se presenta el caso infrecuente de un paciente con miopatía visceral esporádica y afectación de la totalidad del tracto gastrointestinal y de la vía urinaria. La miopatía visceral es una forma de pseudo-obstrucción intestinal crónica idiopática caracterizada por degeneración vacuolar, atrofia y fibrosis de la capa muscular propia de la pared intestinal, sin células inflamatorias. Se puede presentar en niños y adolescentesafectando la musculatura visceral digestiva y urinaria. La manifestación familiar se encuentra en aproximadamente el 30% de los casos y se transmite de forma autosómica recesiva en la mayoría de las familias. Esfundamental descartar causas secundarias de pseudo-obstrucción intestinal crónica y la realización de biopsia de todo el espesor de la pared gastrointestinal para poder arribar al diagnóstico. El tratamiento quirúrgico sólo es eficaz en los que tienen afectación de porciones aisladas del tubo digestivo.

Amyloidotic muscle pseudohypertrophy: case report

The authors report one case of amyloidosis associated with muscular pseudohypertrophy in a 46-year-old woman, who developed weakness, macroglossia and muscle hypertrophy associated with primary systemic amyloidosis. Electromyography showed a myopathic pattern and bilateral carpal tunnel syndrome. The muscle biopsy presented with a type I and II fiber hypertrophy and infiltration of amyloid material in the interstitious space and artery walls. She underwent bone marrow transplantation with stabilization and subjective improvement of the clinical picture