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Spontaneous abdominal wall hematomas are relatively rare and mainly attributed to anticoagulation and severe cough. Despite the high incidence of anticoagulation-related bleeding complications, there are no reports of spontaneous abdominal wall hematomas during extracorporeal membrane oxygenation (ECMO). We report a case of a spontaneous rectus sheath hematoma caused by alternation of the lateral semi-prone position during ECMO in a 76-year-old female patient with severe acute respiratory distress syndrome. Unfractionated heparin 12,000-14,000 units/day was administered for anticoagulation during ECMO. From Day 6 of ECMO, the patient who was under deep sedation was alternately placed in the left and right lateral semi-prone positions every 4 h, for approximately 20 h per day. On Day 12 of ECMO, the patient developed hypotension with anemia and a palpable mass in the right lower abdomen. Abdominal ultrasonographic imaging revealed a huge echo-free space centered in the right lower abdomen. Emergency contrast-enhanced computed tomography (CT) scanning showed extravasation from the superior and inferior epigastric arteries as well as a rectus sheath hematoma. Despite no apparent contrast leakage, an inferior epigastric artery embolization was undertaken because the patient was on ECMO. On Day 13 after ECMO initiation, ECMO and anticoagulation were discontinued. On CT scanning a week later, the hematoma had reduced. In conclusion, spontaneous abdominal wall hematoma is a rare and important complication that might occur during ECMO. Thus, careful physical examination should be routinely conducted when the patient is semi-prone during ECMO.

It is well recognized that poor muscle function and poor physical performance are strong predictors of clinically relevant adverse events in older people. Given the large number of approaches to measure muscle function and physical performance, clinicians often struggle to choose a tool that is appropriate and validated for the population of older people they deal with. In this paper, an overview of different methods available and applicable in clinical settings is proposed. This paper is based on literature reviews performed by members of the European Society for Clinical and Economic Aspects of Osteoporosis and Osteoarthritis (ESCEO) working group on frailty and sarcopenia. Face-to-face meetings were organized afterwards where the whole group could amend and discuss the recommendations further. Several characteristics should be considered when choosing a tool: (1) purpose of the assessment (intervention, screening, diagnosis); (2) patient characteristics (population, settings, functional ability, etc.); (3) psychometric properties of the tool (test-retest reliability, inter-rater reliability, responsiveness, floor and ceiling effects, etc.); (4) applicability of the tool in clinical settings (overall cost, time required for the examination, level of training, equipment, patient acceptance, etc.); (5) prognostic reliability for relevant clinical outcomes. Based on these criteria and the available evidence, the expert group advises the use of grip strength to measure muscle strength and the use of 4-m gait speed or the Short Physical Performance Battery test to measure physical performance in daily practice. The tools proposed are relevant for the assessment of muscle weakness and physical performance. Subjects with low values should receive additional diagnostic workups to achieve a full diagnosis of the underlying condition responsible (sarcopenia, frailty or other).

Fatty acid β-oxidation (FAO) disorders have a wide variety of symptoms, not usually evident between episodes of acute decompensations. Cardiac involvement is frequent, and severe ventricular arrhythmias are suspected of causing sudden death. Expanded newborn screening (ENS) for these disorders, hopefully, contribute to prevent potentially acute life-threatening events. In order to characterize acute decompensations observed in FAO-deficient cases identified by ENS, a retrospective analysis was performed, covering a period of 9 years. Demographic data, number/type of acute decompensations, treatment, and follow-up were considered. Eighty-three clinical charts, including 66 medium-chain acyl-CoA dehydrogenase deficiency (MCADD), 5 carnitine-uptake deficiency (CUD), 3 carnitine palmitoyltransferase I and II (CPT I/II) deficiency, 5 very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), and 4 multiple acyl-CoA dehydrogenase deficiency (MADD) cases were reviewed. Nineteen patients had acute decompensations (1 CPT I, 1 CPT II, 3 MADD, 14 MCADD). Six patients developed symptoms previously to ENS diagnosis. Severe clinical manifestations included multiple organ failure, liver failure, heart failure, and sudden death. Long-chain FAO disorders had the highest number of decompensations per patient.Conclusion: Despite earlier diagnosis by ENS, sudden deaths were not avoided and acute decompensations with severe clinical manifestations still occur as well. What is Known: • Severe ventricular arrhythmias are suspected to cause unexpected death in FAO disorders. • Neonatal screening intends to reduce the incidence of severe metabolic crisis and death. What is New: • Acute severe decompensations occurred in FAO disorders diagnosed through neonatal screening. • Sudden deaths were not avoided by starting treatment precociously.

Myopathies and mitochondrial diseases pose a major challenge in diagnosis due to the multitude of different entities and - in the case of mitochondriopathies - the possible involvement of multiple organs. Furthermore, there is broad clinical variability within particular diseases; patients with hereditary myopathy, for example, can show great phenotypic variability despite identical genetic defects. In addition to environmental factors, gender-specific influences, and the degree of heteroplasmy in mitochondrial diseases, the existence of disease-modifying genes has long been assumed as an explanation. In recent years, risk genes, which can influence the course of disease, have been identified for some myopathies and mitochondrial diseases. The precise role of these disease-modifying genes in the pathogenesis of the diseases is largely unexplained and requires further research.

Intensive care unit acquired weakness (ICUAW) is a frequent and severe complication of intensive care management. Within ICUAW critical illness polyneuropathy (CIP) and myopathy (CIM) can be differentiated. The major symptom of ICUAW is progressive quadriparesis, which makes weaning from the respirator more difficult, can appear early after admission to an ICU and can often be detected several months after discharge from the ICU. The pathophysiology of ICUAW is multifactorial and complex. Potential therapeutic approaches are the early and sufficient therapy of mulitorgan dysfunction, optimal control of glucose levels as well as early and intensive physiotherapy. This review article discusses the data on incidence, pathophysiology, diagnostic approaches and prognosis of ICUAW.

Pearson marrow-pancreas syndrome (PS) is a rare mitochondrial disorder. Impaired mitochondrial respiratory chain complexes (MRCC) differ among individuals and organs, which accounts for variable clinical pictures. A subset of PS patients develop 3-methylglutaconic aciduria (3-MGA-uria), but the characteristic symptoms and impaired MRCC remain unknown. Our patient, a girl, developed pancytopenia, hyperlactatemia, steatorrhea, insulin-dependent diabetes mellitus, liver dysfunction, Fanconi syndrome, and 3-MGA-uria. She died from cerebral hemorrhage at 3 years of age. We identified a novel 5.4-kbp deletion of mitochondrial DNA. The enzymatic activities of MRCC I and IV were markedly reduced in the liver and muscle and mildly reduced in skin fibroblasts and the heart. To date, urine organic acid analysis has been performed on 29 PS patients, including our case. Eight patients had 3-MGA-uria, while only one patient did not. The remaining 20 patients were not reported to have 3-MGA-uria. In this paper, we included these 20 patients as PS patients without 3-MGA-uria. PS patients with and without 3-MGA-uria have similar manifestations. Only a few studies have examined the enzymatic activities of MRCC., Conclusion: No clinical characteristics distinguish between PS patients with and without 3-MGA-uria. The correlation between 3-MGA-uria and the enzymatic activities of MRCC remains to be elucidated., What Is Known: • The clinical characteristics of patients with Pearson marrow-pancreas syndrome and 3-methylglutaconic aciduria remain unknown., What Is New: • No clinical characteristics distinguish between Pearson marrow-pancreas syndrome patients with and without 3-methylglutaconic aciduria.

Calcium hydroxyapatite crystal deposition is a common disorder, which sometimes causes acute pain as calcifications dissolve and migrate into adjacent soft tissue. Intraosseous calcium penetration has also been described. We illustrate the appearance of these lesions using a series of 35 cases compiled by members of the French Society of Musculoskeletal Imaging (Société d'Imagerie Musculo-Squelettique, SIMS). The first group in our series (7 cases) involved calcification-related cortical erosions of the humeral and femoral diaphyses, in particular at the pectoralis major and gluteus maximus insertions. A second group (28 cases) involved the presence of calcium material in subcortical areas. The most common site was the greater tubercle of the humerus, accompanying a calcifying tendinopathy of the supraspinatus. In addition, an extensive intramedullary diffusion of calcium deposits was observed in four of these cases, associated with cortical erosion in one case and subcortical lesions in three cases. Cortical erosions and intraosseous migration of calcifications associated with calcific tendinitis may be confused with neoplasm or infection. It is important to recognize atypical presentations of hydroxyapatite deposition to avoid unnecessary investigation or surgery.

Castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum. It is exceptionally uncommon for Castleman disease to present in the extremities. We report a rare case of extranodal Castleman disease presenting as a muscular forearm mass. We compare our case with the seven other reported cases in which Castleman disease presented as an isolated soft tissue mass in the extremities.

Herpes zoster is a common disorder characterized by a painful rash along a dermatome caused by reactivation of the varicella zoster virus (VZV). Muscle denervation injury from motor involvement is an uncommon phenomenon. Discordant distribution of the skin rash and motor nerve involvement, presenting as a skin rash in one body part and muscle weakness or pain from nerve involvement in another body part is an even more uncommonly reported finding. We present an unusual case of muscle denervation injury resulting from motor involvement of a peripheral nerve by VZV diagnosed by magnetic resonance imaging with cutaneous manifestations in a different dermatomal distribution. To the best of our knowledge, there has been no similar case reported in the English radiology literature. We suggest that whenever a radiologist notices MRI findings suggesting denervation injury and a cause not readily identified, VZV-related denervation injury should be included in the differential diagnosis, especially in an older immunocompromised patient.

Objective: Bent spine syndrome (BSS), defined as an abnormal forward flexion of the trunk resolving in supine position, is usually related to parkinsonism, but can also be encountered in myopathies. This study evaluates whole-body muscle MRI (WB-mMRI) as a tool for detecting underlying myopathy in non-extrapyramidal BSS., Materials and Methods: Forty-three patients (90 % women; 53-86 years old) with a non-extrapyramidal BSS were prospectively included. All underwent a 1.5-T WB-mMRI and a nerve conduction study. Muscle biopsy was performed if a myopathy could not be eliminated based on clinical examination and all tests. Systematic MRI interpretation focused on peripheral and axial muscle injury; spinal posture and incidental findings were also reported., Results: WB-mMRI was completed for all patients, with 13 muscle biopsies ultimately needed and myopathy revealed as the final etiological diagnosis in five cases (12 %). All biopsy-proven myopathies were detected by the WB-mMRI. Relevant incidental MRI findings were made in seven patients., Conclusions: This study supports WB-mMRI as a sensitive and feasible tool for detecting myopathy in BSS patients. Associated with electroneuromyography, it can better indicate when a muscle biopsy is needed and guide it when required. Rigorous radiological interpretation is mandatory, so as not to miss incidental findings of clinical consequence.

The deltoid is a fascinating muscle with a significant role in shoulder function. It is comprised of three distinct portions (anterior or clavicular, middle or acromial, and posterior or spinal) and acts mainly as an abductor of the shoulder and stabilizer of the humeral head. Deltoid tears are not infrequently associated with large or massive rotator cuff tears and may further jeopardize shoulder function. A variety of other pathologies may affect the deltoid muscle including enthesitis, calcific tendinitis, myositis, infection, tumors, and chronic avulsion injury. Contracture of the deltoid following repeated intramuscular injections could present with progressive abduction deformity and winging of the scapula. The deltoid muscle and its innervating axillary nerve may be injured during shoulder surgery, which may have disastrous functional consequences. Axillary neuropathies leading to deltoid muscle dysfunction include traumatic injuries, quadrilateral space and Parsonage-Turner syndromes, and cause denervation of the deltoid muscle. Finally, abnormalities of the deltoid may originate from nearby pathologies of subdeltoid bursa, acromion, and distal clavicle.

Aim: Diastasis of rectus abdominis is a condition defined as a separation between this muscle and the linea alba, which leads to weakness in the abdominal muscles. Later, such weakness will influence the biomechanical posture causing back pain in women. In the present study, our aim was to assess the accuracy of clinical examination in measuring diastasis of rectus abdominis in the postpartum period., Method: Puerperal women (n=106) were selected for this study, including caesarean delivery (62 %) and vaginal delivery (38 %). Diastasis of rectus abdominis was measured in the postpartum period using clinical examination and ultrasonography, at four levels of the anterior sheath of the rectus abdominis, in the region above the umbilical scar., Results: At each level of measurement, the results were statistically analyzed using the Student's t and Pearson tests (confidence interval 95 %), Bland-Altman plot, and Kappa test. The Student t test showed no significant difference between clinical examination and ultrasonography. The Pearson test showed correlation with positive coefficients; moderate correlation (r > 0.5) in the first levels (3, 6, and 9 cm) and a strong correlation (r > 0.75) in the last level (12 cm). In the Kappa test, 65 % of diagnoses given in the clinical examination were confirmed by ultrasonography., Conclusions: These results show a good agreement between both forms of examination, allowing clinical examination to be used in the diagnosis of rectus muscle diastasis, when ultrasonography is not available.

Unlabelled: Kearns-Sayre syndrome (KSS) is a rare mitochondrial DNA deletion syndrome defined as the presence of ophthalmoplegia, pigmentary retinopathy, onset less than age 20 years, and one of the following: cardiac conduction defects, cerebellar syndrome, or cerebrospinal fluid protein above 100 mg/dl. KSS may affect many organ systems causing endocrinopathies, encephalomyopathy, sensorineural hearing loss, and renal tubulopathy. Clinical presentation at diagnosis is quite heterogeneous and, usually, few organs are affected with progression to generalized disease early in adulthood. We present the case of a boy with KSS presenting at the age of 5 years with myopathy, Addison's disease, primary hypoparathyroidism, and Fanconi syndrome. The proper replacement treatment along with the administration of mitochondrial metabolism-improving agents had a brief ameliorating effect, but gradual severe multisystemic deterioration was inevitable over the next 5 years., Conclusion: This report highlights the fact that in case of simultaneous presentation of polyendocrinopathies and renal disease early in childhood, KSS should be considered.

Objectives: Nonspecific clinical presentations often lead to misdiagnosis of focal cysticercal myositis. This report emphasizes the role of ultrasonography and magnetic resonance imaging (MRI) in the diagnosis of solitary intramuscular cysticercosis., Materials and Methods: Six patients with persistent post-traumatic isolated muscular swelling were treated with analgesic and antibiotics, but the swelling did not subside. Radiographs showed soft tissue swelling with no bony abnormalities. Laboratory markers were inconclusive. Ultrasonographic and magnetic resonance images (MRI) showed typical features of intramuscular cysticercosis. Clinical, radiological, and fundoscopic evaluation of brain and eyes could not isolate any cysticercosis focus in these organs. Patients were treated with 3 weeks albendazole therapy., Results: The identifying sonographic features of intramuscular cysticercosis, as evident from this case series, included an intramuscular elliptical or oval anechoic lesion with echogenic intralesional focus likely to be scolex. Magnetic resonance images showed orientation of the cyst along the direction of muscle fibers with T2W hyperintense signal and post-contrast perilesional enhancement. All patients responded to medical treatment., Conclusion: Cysticercosis may manifest as isolated muscular swelling without neurological or ocular involvement. Clinicians should be aware of this clinical condition to avoid misdiagnosis. Ultrasonography and magnetic resonance imaging are good diagnostic aids to establish soft tissue cysticercosis.

Individuals with a history of non-specific low back pain (LBP) while in a quiescent pain period demonstrate altered automatic postural responses (APRs) characterized by reduced trunk torque contributions and increased co-activation of trunk musculature. However, it is unknown whether these changes preceded or resulted from pain. To further delineate the relationship between cyclic pain recurrence and APRs, we quantified postural responses following multi-directional support surface translations, in individuals with non-specific LBP, following an active pain episode. Sixteen subjects with and 16 without LBP stood on two force plates that were translated unexpectedly in 12 directions. Net joint torques of the ankles, knees (sagittal only), hips, and trunk, in the frontal and sagittal planes, were quantified and the activation of 12 muscles of the lower limb unilaterally and the dorsal and ventral trunk, bilaterally, were recorded using surface electromyography (EMG). Peaks and latencies to peak joint torques, rates of torque development (slopes), and integrated EMGs characterizing baseline and active muscle contributions were analyzed for group by perturbation direction (torques) and group by perturbation by epoch interaction (EMG) effects. In general, the LBP cohort demonstrated APRs that were of similar torque magnitude and rate but peaked earlier compared to individuals without LBP. Individuals with LBP also demonstrated increased muscle activity following perturbation directions in which the muscle was acting as a prime mover and reduced muscle activity in opposing directions, proximally and distally, with some proximal asymmetries. These altered postural responses may reflect increased muscle spindle sensitivity. Given that these motor alterations are demonstrated proximally and distally, they likely reflect the influence of central nervous system processing in this cohort.

Leuprorelin is a luteinizing hormone-releasing hormone (LH-RH) agonist that is used as an agent of androgen deprivation in some patients with prostate cancer. When administered in depot form, local granulomatous reactions may occur at the injection site, which may mimic masses and which are associated with treatment failure. We present a patient who, over a period of 5 years, developed multiple intramuscular gluteal masses while receiving leuprorelin therapy via intramuscular depot injections; biopsy of one of the masses showed the specific histologic features of leuprorelin granuloma. To our knowledge, this entity has not been described in the radiology literature. Awareness of this entity is necessary to suggest the correct diagnosis in patients with a history of leuprorelin depot injections.

A frequent cause of weaning failure and the resultant long-term artificial ventilation is the generalized weakness syndrome in the sense of critical illness polyneuropathy or polymyopathy. However, hardly any information is presently available regarding the necessary intensity of the diagnostic workup for reaching or excluding a diagnosis with certainty in the neurological examination or regarding the additional diagnostic value of electrophysiological studies in patients receiving long-term acute care suspected of having critical illness polyneuropathy and polymyopathy. Therefore, the goal of this investigation was to address these questions. A total of 280 patients with complicated weaning were included in the study. All patients underwent clinical examination by a specialist in neurology and electrophysiological workup performed by another specialist. Among the patients studied, the greatest possible certainty of the diagnosis (positive predictive value) of the clinical examination was 97.9% [95% confidence interval (CI) 69.4-99.9] and the best certainty of excluding the diagnosis (negative predictive value) was 88.9% (95% CI 82.7-93.0). Thus, in difficult-to-wean patients who were considered to probably have the diagnosis of critical illness polyneuropathy or polymyopathy as assessed by a specialist, little additional information is gained from an electrophysiological study, which is hence dispensable in these cases.

Background: Critical Illness Myopathy and Neuropathy (CRIMYN) frequently coexist with severe sepsis and is associated with prolonged weaning from mechanical ventilation and prolonged ICU length of stay. We aimed to classify different levels as well as patterns of impairment with regard to electrophysiological disturbances in CRIMYN patients by cluster analysis., Methods: A total of 30 patients with sepsis/SIRS were studied prospectively. Motor and sensory conduction studies were performed from six motor and four sensory nerves on a weekly basis from admission until discharge and finally after 6 months. A control group of 63 healthy persons was examined simultaneously using the same criteria. Different patterns of electrophysiological disturbances were classified by cluster analysis based on differences to reference values of 20 parameters, compound muscle action potential (CMAP), sensory nerve action potential (SNAP) and motor and sensor conduction velocity (NCV)., Results: Four different clusters were identified: cluster 1 showing normal values for CMAP, SNAP and NCV in all nerves (3 patients and all test persons), cluster 2 showing pathological values for CMAP in the lower extremities and the other parameters were normal (5 patients), cluster 3 showing moderately pathological values for CMAP, SNAP and sensory NCV in upper and lower extremities and motor NCV in lower extremities (12 patients) and cluster 4 showing severe disturbances of CMAP, SNAP and NCV in upper and lower extremities (10 patients)., Conclusion: A total of four different clusters of electrophysiological impairment can be identified in patients with sepsis/SIRS, which enables further differentiation of the severity of neuromuscular disturbances in sepsis-associated organ failure. This might be useful as a prognostic parameter and can be correlated with additional clinical and paraclinical parameters related to sepsis.

Lipid storage myopathies are a clinically and genetically heterogeneous group of muscle diseases characterized by an accumulation of lipid in skeletal muscle. Currently four different groups of lipid storage myopathies are described: primary carnitine deficiency (PCD), multiple acyl-CoA dehydrogenase deficiency, primary and secondary coenzyme Q10 deficiency and neutral lipid storage diseases. It might be due to their rareness and considerable clinical variability that these disorders are frequently disregarded in neurological differential diagnosis. This article provides a synopsis of several new aspects of pathophysiology, symptoms, diagnostic tools and current therapeutic approaches of lipid storage myopathies.

Objective: To determine if magnetic resonance imaging (MR imaging) is useful in the diagnostic workup of muscular dystrophies and idiopathic inflammatory myopathies for describing the topography of muscle involvement., Materials and Methods: MR imaging was performed in 31 patients: 8 with dystrophic myotony types 1 (n = 4) or 2 (n = 4); 11 with limb-girdle muscular dystrophy, including dysferlinopathy, calpainopathy, sarcoglycanopathy, and dystrophy associated with fukutin-related protein mutation; 3 with Becker muscular dystrophy; and 9 with idiopathic inflammatory myopathies, including polymyositis, dermatomyositis, and sporadic inclusion body myositis., Results: Analysis of T1 images enabled us to describe the most affected muscles and the muscles usually spared for each muscular disease. In particular, examination of pelvis, thigh, and leg muscles demonstrated significant differences between the muscular diseases. On STIR images, hyperintensities were present in 62% of our patients with muscular dystrophies., Conclusion: A specific pattern of muscular involvement was established for each muscular disease. Hyperintensities observed on STIR images precede fatty degeneration and are not specific for inflammatory myopathies.

Introduction: Blunt force injuries in martial arts occur frequently but isolated hematoma of muscles in the extremities is rare. Even minor trauma of the lower extremities due to throwing techniques in judo and other forms of Asian martial arts can lead to major pathologies., Methods: A 9-year-old girl presented with an unclear swelling and soreness of the calf muscle. The patient could not remember an obvious traumatic event. She was admitted 4 days later because of increased swelling, pain and erythema. The parents reported a minor trauma at judo training 1 week ago. Further investigation was performed with MRI and confirmed a massive hematoma much greater than previously shown by sonography., Results: Histologic and microbiologic evaluation demonstrated florid inflammation and proof of Staphylococcus aureus was found intra-operatively., Discussion: The diagnosis of blunt force injuries due to martial arts is difficult in childhood because often children do not remember a traumatic event. Therefore, it is important to obtain a thorough history from caregivers. Because sonography depends highly on the experience of the investigator MRI is considered to be a better diagnostic modality to diagnose and guide treatment in this age group.

Introduction: Despite a high complication rate, subjective and objective results of reversed shoulder arthroplasty for severe rotator cuff lesions associated with osteoarthritis, fracture sequelae, or revision of hemiarthroplasty are favorable. However, whether the changes in biomechanics of the joint may lead to structural changes in the remaining rotator cuff and the deltoid muscle, and may thereby alter the clinical result, has rarely been described. This study investigates the context between postoperative fatty infiltration of the remaining rotator cuff and the deltoid muscle, and the clinical outcome after reversed shoulder arthroplasty., Methods: Twenty-three patients with an average age of 73.1 +/- 5.6 years were evaluated at an average of 26 +/- 16.8 months. Evaluation included assessment of Constant Score, disabilities of the arm, shoulder and hand score, radiographic evaluation with measurement of the humeral lengthening and medialization of the center of rotation, and magnetic resonance imaging or computed tomography to classify changes in the rotator cuff and the deltoid muscle. Results There was a significant correlation between fatty infiltration of the deltoid muscle and the clinical score results. Patients with a longer follow-up showed a higher amount of degenerative changes of the deltoid than patients with a shorter follow-up. Degenerative changes of the rotator cuff showed no significant correlation to clinical results. The amount of arm lengthening, medialization of the center of rotation, and scapula notching showed no correlation to clinical results or degenerative changes of the deltoid and the rotator cuff., Conclusions: These data show that degeneration of the deltoid muscle is of impact for clinical outcome. Although other factors like disuse or pain as a reason for muscular changes cannot be fully excluded, this degeneration might occur after time due to biomechanical changes after reversed arthroplasty. However, prospective studies with longer follow-up periods and repetitive assessment of muscle quality are necessary to further support these results.

Haemophilia disorders are characterised by a blood coagulation anomaly leading to prolonged and excessive bleeding. Imaging provides an essential role in the investigation of both the musculoskeletal and the non-musculoskeletal complications of haemophilia. Our institution is home to a large tertiary referral centre for haemophilia treatment. Using our broad experience, we present a multi-modality pictorial review of the musculoskeletal manifestations of haemophilia, including haemophilic arthropathy, intra-muscular haemorrhage and haemophilic pseudotumour. The main imaging features of haemophilic arthropathy are described, including synovial hypertrophy, haemosiderin deposition, sub-chondral cyst formation and loss of joint space.

Introduction: Calcific myonecrosis is a rare condition characterized by a calcified lesion. Because this lesion sometimes presents as a huge mass with unique radiographic characteristics, it can be confused with soft tissue sarcoma. The appropriate treatment is still controversial. We report here three cases diagnosed as calcific myonecrosis of the lower leg and discuss its clinical presentation, diagnosis and options for treatment., Patients and Methods: Three patients presented as expanding masses in the anterior or lateral compartment muscle and occurred more than 10 years after an initial injury. Plain radiographs showed a large soft tissue mass with extensive amorphous calcifications. Computed tomograms revealed a low-density tumor with scattered calcifications. Magnetic resonance imaging showed a bright, high-signal area on T2-weighted imaging consistent with fluid. Two patients underwent excisions for histopathological evaluation and one was treated conservatively because of the absence of disability., Results: Two operative patients had no complication and the returned to work without evidence of deterioration of the disease. In a patient treated conservatively, the mass was not enlarged and was still painless at 2-year follow-up., Conclusion: The key points for diagnosis were (1) amorphous liner calcification, (2) anterior or lateral compartment, (3) previous injury (several decades) of the tibia or femur, and (4) compartment syndrome. Although calcific myonecrosis is not commonly encountered, it must be considered in the differential diagnosis of an expanding intramuscular mass. In asymptomatic masses where the diagnosis is recognized, observation may be possible and unnecessary aggressive surgical intervention should be avoided.

Myofibrillar myopathies (MFMs) are rare inherited or sporadic progressive neuromuscular disorders with considerable clinical and genetic heterogeneity. In the current study, we have analyzed histopathological and immunohistochemical characteristics in genetically identified MFMs. We performed a morphological and morphometrical study in a cohort of 24 genetically identified MFM patients (12 desmin, 6 alphaB-crystallin, 4 ZASP, 2 myotilin), and an extensive immunohistochemical study in 15 of these patients, using both well-known and novel antibodies directed against distinct compartments of the muscle fibers, including Z-disc and M-band proteins. Our morphological data revealed some significant differences between the distinct MFM subgroups: the consistent presence of 'rubbed-out' fibers in desminopathies and alphaB-crystallinopathies, an elevated frequency of vacuoles in ZASPopathies and myotilinopathies, and the presence of a few necrotic fibers in the two myotilinopathy patients. Immunohistochemistry showed that in MFM only a subset of Z-disc proteins, such as filamin C and its ligands myotilin and Xin, exhibited significant alterations in their localization, whereas other Z-disc proteins like alpha-actinin, myopodin and tritopodin, did not. In contrast, M-band proteins revealed no abnormalities in MFM. We conclude that the presence of 'rubbed-out' fibers are a suggestive feature for desminopathy or alphaB-crystallinopathy, and that MFM is not a general disease of the myofibril, but primarily affects a subgroup of stress-responsive Z-disc proteins.

Hydatid disease is a parasitic infection generally occurring in specific geographical areas. Exclusive involvement of the muscles is extremely uncommon, because implantation at this site would require passage through the filters of the liver and lung. We describe a patient with a mass in the popliteal fossa of the knee for 7 years who was seen for pain and was found to have hydatid disease.

Chanarin-Dorfman disease (CDD) is a rare genetic disorder characterized by ichthyosis, myopathy, central nervous system disturbances, and intracellular lipid storage in muscle fibers, hepatocytes, and granulocytes. We describe skeletal muscle magnetic resonance imaging findings in a case of CDD, outlining the potential role of GE T1-weighted opposed-phase sequence (chemical shift imaging) in the evaluation of lipid storage myopathies.

Paravertebral lumbar muscles are important for spine stabilization and mobility. They may be abnormal in several disorders that may be associated with pain or functional impairment. Special attention should be paid to the paravertebral muscles during imaging, so that a possible muscular disease is not overlooked, especially in patients with low back pain. This article reviews such imaging abnormalities.

Objective: To describe musculocutaneous MR-findings responsible for disability in chronic graft-versus-host disease (cGVHD) after allogeneic hematopoietic cell transplantation (HCT)., Material and Methods: Between June 2005 and February 2008, we performed whole-body musculoskeletal magnetic resonance imaging (MRI; n = 12) or regional MRI (n = 4) in 16 consecutive patients presenting with disabling sclerodermatous cGVHD (e.g., skin edema, fixed deep dermal sclerosis, joint contractures, painful muscular contractures, or myalgia)., Results: In all patients, MRI showed musculocutaneous abnormalities reflecting different degrees of inflammation and collagen tissue involvement of the skin (n = 10), subcutaneous fat tissue (n = 13), muscle fasciae (n = 16), subfascial muscular septae (n = 6), or findings compatible with myositis (n = 3). The most frequently involved muscle fasciae comprised those of the vastus lateralis muscle (n = 12), biceps femoris muscle (n = 11), gastrocnemius medialis muscle (n = 8), serratus anterior muscle, and latissimus dorsi muscle (each, n = 5). Increased signal of involved tissues on STIR-images and fat-saturated postgadolinium T1-weighted images represented the most frequent MR-signal abnormalities., Conclusion: MR imaging of musculocutaneous cGVHD allows accurate evaluation including assessment of deep tissue infiltration and assists in the differential diagnosis.

The perforation of the medial acetabular wall during total hip arthroplasty due to drilling is not uncommon. But, it has rarely been associated with serious adverse events. Here, we present a case report describing an iliacus hematoma with subsequent femoral nerve palsy after primary total hip arthroplasty in a 67-year-old woman who underwent primary total hip arthroplasty due to painful hip osteoarthritis. The diagnosis was made by pelvic magnetic resonance imaging. Conservative treatment was employed and the symptoms were resolved within 3 months. It should be borne in mind that femoral nerve palsy may occur after total hip arthroplasty. It may be due to a treatable cause, such as iliacus hematoma. So, pelvic MRI is recommended in such a condition, rather than just observation.

Behcet's disease is an inflammatory disease of unknown cause characterized by intermittent episodes of acute inflammation manifested by oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. We report a rare case of myonecrosis associated with Behcet's disease. Myonecrosis of Behcet's disease can mimic soft tissue abscess and therefore awareness of this entity in the appropriate clinical setting is important for initiation of appropriate and timely treatment.

In clinical practice various modalities are used for whole-body imaging of the musculoskeletal system, including radiography, bone scintigraphy, computed tomography, magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT). Multislice CT is far more sensitive than radiographs in the assessment of trabecular and cortical bone destruction and allows for evaluation of fracture risk. The introduction of combined PET-CT scanners has markedly increased diagnostic accuracy for the detection of skeletal metastases compared with PET alone. The unique soft-tissue contrast of MRI enables for precise assessment of bone marrow infiltration and adjacent soft tissue structures so that alterations within the bone marrow may be detected before osseous destruction becomes apparent in CT or metabolic changes occur on bone scintigraphy or PET scan. Improvements in hard- and software, including parallel image acquisition acceleration, have made high resolution whole-body MRI clinically feasible. Whole-body MRI has successfully been applied for bone marrow screening of metastasis and systemic primary bone malignancies, like multiple myeloma. Furthermore, it has recently been proposed for the assessment of systemic bone diseases predisposing for malignancy (e.g., multiple cartilaginous exostoses) and muscle disease (e.g., muscle dystrophy). The following article gives an overview on state-of-the-art whole-body imaging of the musculoskeletal system and highlights present and potential future applications, especially in the field of whole-body MRI.

Sarcoidosis is a noncaseating granulomatous disease that involves multiple organs, most commonly the lungs, lymph nodes, skin, and eyes, but may be clinically evident in any organ system. Symptomatic muscular sarcoidosis is very rare and has been reported in only 1.4% of known sarcoidosis cases, while skeletal involvement has been reported in 1-13%. Different types of muscular and osseous sarcoidosis have been described in the literature. We present a case of muscular sarcoidosis, which is unique in its clinical presentation and imaging findings. The presence of the marked periosteal reaction, which has never been described with muscular sarcoidosis, also makes this case unique.

We report an unusual case of a woman who sought medical attention for a painful mass within her anterolateral left thigh. The patient's symptoms waxed and waned with her menses. Magnetic resonance imaging revealed a 3 cm mass isointense to muscle and surrounded by a rim of decreased signal intensity on T1-weighted images. T2-weighted images with fat saturation revealed a low signal intensity mass with a surrounding rind of high signal intensity edema. Based on its magnetic resonance imaging characteristics, the lesion was initially thought to represent the sequela of prior trauma or perhaps a small fibrous tumor. An excisional biopsy of the mass, however, revealed endometrial tissue, and a pathologic diagnosis of intramuscular endometriosis was rendered.

Objective: Idiopathic and diabetic-associated muscle necrosis are similar, uncommon clinical entities requiring conservative management and minimal intervention to avoid complications and prolonged hospitalization. An early noninvasive diagnosis is therefore essential. We evaluated the magnetic resonance imaging (MRI) characteristics of muscle necrosis in 14 patients, in eight of whom the diagnoses were confirmed histologically., Design and Patients: Two experienced musculoskeletal radiologists performed retrospective evaluations of the MRI studies of 14 patients with the diagnoses of skeletal muscle infarction. In 10 cases gadolinium-enhanced (T1-weighted fat-suppressed) sequences were available along with T1-weighted, T2-weighted images and STIR sequences, while in four cases contrast-enhanced images were not available., Results: Eight patients had underlying diabetes and in six patients the cause of the myonecrosis was considered idiopathic. T1-weighted images demonstrated isointense swelling of the involved muscle, with mildly displaced fascial planes. There was effacement of the fat signal intensity within the muscle. Fat-suppressed T2-weighted images showed diffuse heterogeneous high signal intensity in the muscles suggestive of edema. Perifascial fluid collection was seen in eight cases. Subcutaneous edema was present in seven patients. Following intravenous gadolinium administration, MRI demonstrated a focal area of heterogeneously enhancing mass with peripheral enhancement. Within this focal lesion, linear dark areas were seen with serpentine enhancing streaks separating them in eight cases. In two cases, a central relatively nonenhancing mass with irregular margins and peripheral enhancement was noted. The peripheral enhancement involved a significant part of the muscle. No focal fluid collection was noted., Conclusions: We believe that the constellation of imaging findings on T1- and T2-weighted images and post-gadolinium sequences is highly suggestive of muscle necrosis. We consider certain specific findings on gadolinium-enhanced images to be characteristic. The findings reported here should provide radiologists with useful information in making the diagnosis of skeletal muscle necrosis without resorting to invasive procedures.

Myopathies ranging from myalgia to clinically asymptomatic creatine kinase (CK) elevation and to life-threatening rhabdomyolysis belong to the most important complications of lipid-lowering therapies with fibrates and 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, i.e., statins. Rhabdomyolysis is a rare side effect of statin therapy with an estimated incidence of 0.2/1 million prescriptions. Myalgia and muscle cramps were reported by up to 5% of patients, but they were observed with the same percentage in controls receiving placebo. Due to increasing numbers of patients under lipid-lowering therapy, however, more and more patients present in neuromuscular units with the differential diagnosis of a fibrate- or statin-induced myopathy.

This contribution outlines possibilities and limitations of whole-body MRI for investigating musculoskeletal diseases. Benefits and drawbacks of the novel whole-body MRI technology are discussed and a possible whole-body MRI sequence protocol for musculoskeletal examinations is proposed. Muscle, joint and bone diseases are discussed in which the application of whole-body MRI may be of advantage. Particularly, polymyositis, muscledystrophy, rheumatoid arthritis, spondylitis ancylosans, multiple trauma, skeletal metastases, multiple myeloma and malignant lymphoma are mentioned. Whole-body MRI opens new advantages for the examination of multifocal musculoskeletal diseases. The clinical benefit of this method for particular diseases has to be evaluated in further studies, however.

A soft tissue aneurysmal bone cyst located in the right gluteus medius of a 21-year-old man is reported. On conventional radiography, the lesion demonstrated a spherically trabeculated mass with a calcific rim. On CT scan, it showed a well-organized peripheral calcification resembling a myositis ossificans. On MRI, it presented as a multilocular, cystic lesion with fluid-fluid levels. The lesion had no solid components except for intralesional septa. Although findings on imaging and histology were identical to those described in classical aneurysmal bone cyst, diagnosis was delayed because of lack of knowledge of this entity and its resemblance to the more familiar post-traumatic heterotopic ossification (myositis ossificans).

Calcific myonecrosis is a rare and latent condition characterized by a dystrophic calcified lesion that can present 10-64 years following initial trauma. Of the 25 cases documented in English world literature, all have occurred in the lower extremity exclusively. We report a case of a 60-year-old man with a painless enlarging left forearm mass that was subsequently diagnosed as calcific myonecrosis. Awareness of this lesion arising outside of the lower extremity is important to avoid unnecessary surgical intervention and patient reassurance.

Endometriosis, an important gynecological disorder of reproductive women, affects most commonly the ovaries and less frequently the gastrointestinal tract, chest, urinary tract, and soft tissues. Endometriosis classically appears on MRI as a mass with a large cystic component and variable signal intensities on T1- and T2-weighted images due to the presence of variable degradation of hemorrhagic products. Endometriosis in an atypical location, an infiltrative appearance and without cystic-hemorrhagic components has rarely been described. We report on a 33-year-old woman with cyclic sciatica due to histologically documented infiltrative endometriosis involving the area of the left sciatic notch.