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Objective: To summarize the clinical, thigh magnetic resonance (tMRI) and electromyographic (EMG) characteristics in patients with immune-mediated necrotizing myopathy (IMNM). Methods: A total of 32 IMNM patients who were admitted to the Department of Neurology from April 2019 to April 2021 were enrolled at the First Medical Centre of Chinese PLA General Hospital. According to the type of antibody, the patients were divided into anti-SRP antibody positive (SRP + ) group, anti-HMGCR antibody positive (HMGCR + ) group and seronegative (SN) group. The gender, age, course of disease, myositis antibodies, extramuscular manifestations, EMG were collected and analyzed among three groups. The characteristics of skeletal muscle were assessed by tMRI inflammatory edema and fat infiltration scores. Analysis of variance, Kruskal-Wallis test and Chi-square test were used to compare the differences in different clinical characteristics and tMRI scores among the three groups. When there was a statistical difference among the three groups, the comparison between the two groups was corrected by the Bonferroni method. Result: (1) Of the 32 patients, 20 were females (62.5%).The median age of onset was 47±14 years, 25 (78.1%) patients had an acute or subacute course.There were 17 (53.1%) with SRP + , 8 (25.0%) with HMGCR + , and 7 (21.9%) with MSAs (myositis specific antibodies) negative. Anti-Ro52 antibody was the most common combined antibody (12/32, 37.5%), among which 10 were in SRP + group.(2) The CK of all patients were elevated, median was 5 948 (4 229, 7 664) U/L. There was no statistical difference of MMT scores among three groups. The proximal limb score was lower than distal limb ( P <0.01). The axial muscle score was lower than the distal limb score ( P <0.05).(3) Extramuscular manifestations of HMGCR + group were lower than those of the other two groups (12.5% vs. 71.4% and 76.5%, P <0.017). Rash (60.0% vs.14.3%, P <0.05) and interstitial pulmonary diseases (70.0% vs. 14.3%, P <0.05) were more common in patients with anti-SRP coexistence with anti-Ro52 than those with isolated anti-SRP. Connective tissue disease was more common in SN group (57.1% vs. 11.8% and 0, P <0.017).(4) tMRI showed fascial edema of SN group was more obvious than that of the other two groups ( P <0.017). There was no statistical difference in the degree of fat infiltration and inflammatory edema among three groups, but SRP + group had more cases of early fat infiltration.(5) Myotonic potentials (25.0% vs. 0 and 0, P <0.017) and compound repetitive discharges (CRDs) (50.0% vs. 5.9% and 0, P <0.017) were common in HMGCR + group. Proteomic analysis found significantly different expressed proteins in skeletal muscle of patients with myotonic potentials or CRDs were associated with cytoskeleton, cell junction and extracellular matrix. Conclusion: IMNM with pure anti-SRP antibody positive and anti-HMGCR positive were mainly affected by skeletal muscles. Those who were co-positive for anti-SRP antibody and anti-Ro52 antibody had more extramuscular manifestations, which might be a special subtype of SRP + group. This study proposed for the first time that myofascial inflammatory edema is an early sign of SN-IMNM injury. EMG of HMGCR + group were more prone to myotonia potential and CRDs.

To emphasise that hydatid cyst disease can present as a soft tissue mass even in an unusual site like the thigh, we report the case of a 21-year male patient who presented to Surgical Unit IV, Civil Hospital, Karachi, with a slow growing cystic swelling on the medial aspect of the left thigh. Based on serology and imaging, a diagnosis of solitary subcutaneous hydatid cyst thigh was made and cystopericystectomy was performed. Histopathology confirmed the diagnosis. Up to three months postoperatively, there was no recurrence. In the absence of visceral organ involvement, this is the first reported case of primary subcutaneous hydatid disease of the thigh in Pakistan.

Background: Collagen VI-related myopathies are autosomal dominant and recessive hereditary myopathies, mainly including Ullrich congenital muscular dystrophy (UCMD) and Bethlem myopathy (BM). Muscle magnetic resonance imaging (MRI) has been widely used to diagnosis muscular disorders. The purpose of this study was to evaluate the diagnostic value of thigh muscles MRI for collagen VI-related myopathies., Methods: Eleven patients with collagen VI gene mutation-related myopathies were enrolled in this study. MRI of the thigh muscles was performed in all patients with collagen VI gene mutation-related myopathies and in 361 patients with other neuromuscular disorders (disease controls). T1-weighted images were used to assess fatty infiltration of the muscles using a modified Mercuri's scale. We assessed the sensitivity and specificity of the MRI features of collagen VI-related myopathies. The relationship between fatty infiltration of muscles and specific collagen VI gene mutations was also investigated., Results: Eleven patients with collagen VI gene mutation-related myopathies included six UCMD patients and five BM patients. There was no significant difference between UCMD and BM patients in the fatty infiltration of each thigh muscle except sartorius (P = 0.033); therefore, we combined the UCMD and BM data. Mean fatty infiltration scores were 3.1 and 3.0 in adductor magnus and gluteus maximus, while the scores were 1.3, 1.3, and 1.5 in gracilis, adductor longus, and sartorius, respectively. A "target" sign in rectus femoris (RF) was present in seven cases, and a "sandwich" sign in vastus lateralis (VL) was present in ten cases. The "target" and "sandwich" signs had sensitivities of 63.6% and 90.9% and specificities of 97.3% and 96.9% for the diagnosis of collagen VI-related myopathies, respectively. Fatty infiltration scores were 2.0-3.0 in seven patients with mutations in the triple-helical domain, and 1.0-1.5 in three of four patients with mutations in the N- or C-domain of the collagen VI genes., Conclusions: The "target" sign in RF and "sandwich" sign in VL are common MRI features and are useful for the diagnosis of collagen VI-related myopathies. The severity of fatty infiltration of muscles may have a relationship with the mutation location of collagen VI gene.

Quantitative magnetization transfer (qMT) imaging in skeletal muscle may be confounded by intramuscular adipose components, low signal-to-noise ratios (SNRs), and voluntary and involuntary motion artifacts. Collectively, these issues could create bias and error in parameter fitting. In this study, technical considerations related to these factors were systematically investigated, and solutions were proposed. First, numerical simulations indicate that the presence of an additional fat component significantly underestimates the pool size ratio (F). Therefore, fat-signal suppression (or water-selective excitation) is recommended for qMT imaging of skeletal muscle. Second, to minimize the effect of motion and muscle contraction artifacts in datasets collected with a conventional 14-point sampling scheme, a rapid two-parameter model was adapted from previous studies in the brain and spinal cord. The consecutive pair of sampling points with highest accuracy and precision for estimating F was determined with numerical simulations. Its performance with respect to SNR and incorrect parameter assumptions was systematically evaluated. QMT data fitting was performed in healthy control subjects and polymyositis patients, using both the two- and five-parameter models. The experimental results were consistent with the predictions from the numerical simulations. These data support the use of the two-parameter modeling approach for qMT imaging of skeletal muscle as a means to reduce total imaging time and/or permit additional signal averaging., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: We describe the clinical and muscle MRI changes in 2 siblings with neutral lipid storage disease with myopathy (NLSDM) carrying the mutation c.187+1G>A., Methods: Peripheral blood smears, genetic tests, and muscle biopsies were performed. Thigh MRI was performed to observe fatty replacement, muscle edema, and muscle bulk from axial sections., Results: Both siblings had similar fatty infiltration and edema. T1-weighted images of the gluteus maximus, adductor magnus, semitendinosus, and semimembranosus revealed marked and diffuse fatty infiltration. There was asymmetric involvement in biceps femoris and quadriceps. There was extensive fatty infiltration in the quadriceps, except for the rectus femoris. Gracilis and sartorius were relatively spared. Thigh muscle volume was decreased, while the gracilis and sartorius appeared to show compensatory hypertrophy., Conclusions: Compared with previous reports in NLSDM, MRI changes in this myopathy tended to be more severe. Asymmetry and relatively selective fatty infiltration were characteristics., (© 2014 Wiley Periodicals, Inc.)

Purpose: The aim of this study was to determine the effectiveness of using Tc-sestamibi thigh SPECT/CT imaging for evaluating myopathy in cerebrotendinous xanthomatosis (CTX)., Patients and Methods: Four genetically proven CTX patients (Family I, Cases I-1 and I-2; Family II, Cases II-1 and II-2) were included. They all underwent muscle biopsies for histopathologic and ultrastructural studies. Immunohistochemical staining for vinculin expression was also performed. Tc-sestamibi thigh SPECT/CT imaging was conducted on all 4 CTX patients, and both visual interpretation and muscle-to-background (M/B) ratio count were applied for assessment. Correlation analysis of the imaging findings and results of the ultrastructural and immunohistochemical studies was done., Results: In the Tc-sestamibi thigh SPECT/CT imaging study, all 4 CTX cases had abnormal scores of visual interpretation and M/B ratios. The ultrastructural features of the skeletal muscle of the 4 CTX cases showed mitochondrial and membrane system abnormalities, with increased depositions of metabolites. They also had abnormal increases in vinculin expression after immunohistochemical staining of the skeletal muscle., Conclusions: This is the first report on the use of Tc-sestamibi thigh SPECT/CT imaging to assess the mitochondrial status of CTX. The imaging findings may have a correlation with the ultrastructural and immunohistochemical findings on skeletal muscle. Although the Tc-sestamibi thigh SPECT/CT imaging is not specific for CTX, this noninvasive in vivo assessment can be an important tool for the detection and follow-up study of skeletal muscle involvement in CTX.

Diabetic muscle infarction (DMI) is a rare complication of long-standing diabetes mellitus. This is the first case of DMI reported by cardiologists. A 49-year-old patient with a history of diabetes and hypertension for only two years was admitted to the cardiac ward due to pain in the left thigh with pitting edema in both lower extremities. Magnetic resonance imaging finally confirmed the presence of DMI in the left thigh, which was improved by treatment with anticoagulants, analgesics and rest. However, the typical clinical symptoms of DMI were unrecognizable at the start of treatment, which may be attributed to a lack of awareness of this rare condition among non-endocrinologist physicians.

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.

Enhance your diagnostic skills with this "test yourself" monthly column, which features a radiograph and challenges you to make a diagnosis.

Calcific myonecrosis is a rare complication of limb trauma, that may occur after many years. It is characterized by dystrophic calcification that develops in the late period following compartment syndrome usually in the lower limb. We present a 66-year-old man who developed calcific myonecrosis 35 years after surgical intervention for compartment syndrome secondary to a gunshot injury to the left thigh. He presented with pain and swelling in the left leg. On physical examination, there was a well-defined and immobile mass lesion in the anterolateral part of the left crus, soft in consistency and 20 x 8 x 6 cm in size, showing no relation with the joint. Radiographic evaluation showed linear calcifications in the left crus without osseous pathology. At surgery, all the fibers in the anterior tibial compartment were calcified and there was no attachment to the bone. The patient underwent an excisional biopsy. Histopathological evaluation of the specimens revealed calcific myonecrosis. At the end of one-year follow-up, the patient was symptomless, without any recurrence.

The purpose of this study is to demonstrate and discuss the radiological features of four patients with muscular hydatid disease and to evaluate the results of percutaneous treatment in these patients. Four patients (three female and one male) with six muscular hydatid cysts underwent percutaneous treatment and were followed up. The mean age of patients was 35 years (range: 12-60 years). Type I (n = 2), type II (n = 1), and type III (n = 3) hydatid cysts were observed in the thigh (n = 3) and gluteal (n = 1) region on radiologic examination. All interventions were performed under sonographic and fluoroscopic guidance. According to the type of the cyst, the procedure was carried out by either a "catheterization technique with hypertonic saline and alcohol" or a "modified catheterization technique." The mean cathaterization time was 13.7 days, ranging from 1 to 54 days. The dimensions of the residual cavity were noted at every sonographic control, and an average of 96.1% volume reduction was obtained in six cysts of four patients. No sign of viability was observed during the follow-up period. Cavity infection and cellulitis were observed as complications, which resolved after medical therapy. Percutaneous treatment is a safe and effective procedure in patients with soft-tissue hydatid cysts and should be considered as a serious alternative to surgery.

We report an exceptional case of tuberculosis in an 8-year-old girl. The focus of infection was situated in the anterior muscle compartment of the left thigh. Diagnosis was established on the basis of histological findings. A 12-month antituberculosis regimen was given. Outcome was good with no general or functional sequelae. Based on this case and data reported in the literature, we discuss the routes of dissemination of the Koch bacillus explaining this unusual sporadic localization.

Knowledge of variations in normal human thigh musculature is important for the interpretation of various clinical and biomedical investigations. In this study, cross-sections from whole thighs of 34 cadavers were analyzed qualitatively and morphometrically. Sections were cut from right and left limbs at three levels in the region often used for muscle biopsy. Measurements were made of limb circumferences and cross-sectional areas of the three vasti and of the femur, and of the extent of overlap and fusion between the vasti on the lateral aspect of the thigh. Limb circumference proved to be a good predictor of total cross-sectional area of the quadriceps muscle. Large individual variations were found for most measurements but especially for the amount of fusion between the muscle bellies of vastus lateralis and vastus intermedius. Significant differences were found for most variables between the three levels, but there were few differences between sides except for the cross-sectional area of vastus lateralis which was greater on the right at the most distal level. These variations in size and form of thigh musculature should be considered when interpreting data obtained from muscle biopsy, imaging, biomechanical, electromyographic, and functional studies of the human thigh. Review of the literature on variability of quadriceps muscle and its clinical relevance showed a diversity of approaches but insufficient definitive data suitable for interpreting functional outcomes in response to exercise or following surgery. Further work is suggested combining current anatomical datasets with advanced imaging techniques capable of determining muscle fiber orientation and fiber type composition, and including 3D reconstruction., (Copyright 2002 Wiley-Liss, Inc.)

We report a patient who developed a focal myopathy with prominent contractures of his thigh muscles induced by chronic heroin injection. Muscle biopsy and magnetic resonance imaging (MRI) were indicative of a fibrotic and inflammatory process. Treatment with steroids, D-penicillamine, and physical therapy led to full functional recovery within 6 months. Sequential electromyographic (EMG) studies and muscle biopsies showed evidence of regenerating muscle fibers and absence of inflammatory cells. We conclude that heroin-induced focal myopathy is reversible and that combination therapy with D-penicillamine and steroids may be useful., (Copyright 2000 John Wiley & Sons, Inc.)

We have developed an experimental model to monitor inflammatory lesions in muscle and soft-tissues during the different stages of the disease by means of Magnetic Resonance Imaging (MRI). MRI of mice legs infected with Candida albicans was performed by standard two-dimensional spin echo and fast spin echo (RARE) using customized coils. The MRI findings were compared with pathologic examinations at the initial acute and established acute inflammatory stages, which provided accurate and detailed information on the evolution of the processes involved. The yeast caused inflammation within the first hours post-inoculation, appearing on T2-weighted images as an inhomogeneous mass with increased signal intensity. The presence of fungal hyphae was observed as hypointense signal areas in both T2 and T1 weighted images, with histologic confirmation. Areas of decreased signal intensity on T2 weighted images were apparent on the last experimental day and were attributed to the granulation tissue located within the capsule surrounding the abscess. The close correlation found between MRI and histopathology suggests that MRI is an ideal radiologic technique for monitoring the clinical and therapeutic follow-up of fungal infections in muscle and soft tissues.

A 67-year-old woman presented with rapidly enlarging right anterior thigh mass. Clinical impression was of a primary sarcoma, but the histologic analysis of an incisional biopsy specimen showed adenocarcinoma with clear cell differentiation. Initially thought to be a metastasis, the malignant tumor appears to have arisen from extraabdominal endometriosis of the right groin. This case shows clinical, radiographic, and histologic findings that may help the orthopaedic surgeon recognize an unusual complication of extrapelvic endometriosis.

Magnetic resonance imaging (MRI) of thigh and leg muscles was performed in a patient with alcoholic myopathy showing myalgia, hypercreatine kinasemia, and hypermyoglobinemia. High signal intensities in both T1- and T2-weighted images were widely distributed in the affected muscle groups, which most likely reflected lipid accumulation. Although he had hypermyoglobinemia, MRI and muscle biopsy did not show findings of rhabdomyolysis, such as necrosis, regeneration, and edema. We suggest that the high signal intensities in this case may have indicated "prerhabdomyolysis" related to alcohol abuse and that muscle MRI is useful in the evaluation of alcoholic myopathy, mainly predicting the onset of rhabdomyolysis.

We report the case of a fatal myofascial necrosis caused by an imipenem-resistant Aeromonas hydrophila in a patient with a history of aplastic anemia. He presented with fever and left thigh tenderness. The CT scan was consistent with cellulitis and, after cultures were obtained, empirical treatment with imipenem and amikacin was started. Two days later, necrotic bullae appeared on his thigh, and cultures showed Aeromonas hydrophila which was imipenem-resistant. Although surgical debridement was performed and ciprofloxacin was initiated, the patient died.

We report about a 48 year old female patient with a tumor of the soft-tissue of the right upper leg. The diagnostic results showed an echinococcus cyst of the muscle adductor longus. It is reported that in 0.5-5% of the cases echinococcus cysts could be located in muscles. But mostly this is not the primary location.

A rare case of popliteal cyst due to echinococcus is reported. The implications of the hydatid localisation in this site in regard to differential diagnosis are discussed.

Myxomas of the skeletal muscle are uncommon mesenchymal tumours. About one hundred such tumours have been reported in the world literature so far. In the present paper, we report a case of myxoma which occurred in the thigh muscles, with brief review of pertinent literature.

A patient with the clinical picture of quadriceps myopathy was shown to have an underlying myositis. Review of the literature showed myositis to be a rare cause of the 'quadriceps syndrome' but it is important to identify this group because they are potentially treatable with corticosteroids.

An outbreak of the asymmetric hindquarter syndrome was investigated in a Western Australian piggery. The condition was manifested as asymmetry of the hindquarter, particularly the semitendinosis, semimembranosis and biceps femoris muscles. The gait of affected animals was not noticeably altered, despite an obvious disparity in size between normal and affected limbs. Histologically, muscular degenerative and dystrophic changes were seen but were not present in all undersized muscles and were occasionally present in macroscopically normal muscles. All 7 affected pigs whose breeding history could be traced were sired by the same boar. The condition adversely affected conformation and resulted in down-grading of carcases.