Secondary Myopathy Research Articles

Combination of primary and secondary myopathy in a patient with severe hypothyroidism

Combination of primary and secondary myopathy in a patient with severe hypothyroidism

Clinical applications of skeletal muscle diffusion tensor imaging.

Diffusion tensor imaging (DTI) may allow the determination of new threshold values, based on water anisotropy, to differentiate between healthy muscle and various pathological processes. Additionally, it may quantify treatment monitoring or training effects. Most current studies have evaluated the potential of DTI of skeletal muscle to assess sports-related injuries or therapy, and training monitoring. Another critical area of application of this technique is the characterization and monitoring of primary and secondary myopathies. In this manuscript, we review the application of DTI in the evaluation of skeletal muscle in these and other novel clinical scenarios, with emphasis on the use of quantitative imaging-derived biomarkers. Finally, the main limitations of the introduction of DTI in the clinical setting and potential areas of future use are discussed.

Progressing nemaline myopathy in a patient repeatedly operated on the spine: clinical case and literature review

Objective. To present a clinical case of a patient with spinal deformity associated with myopathy, which was initially undiagnosed and assessed as secondary myopathy after performed spinal surgeries.Material and Methods. Study design: clinical observation and review of the literature. A 51-year-old female patient has been repeatedly operated on for scoliotic deformity of the spine with progressing neurological disorders. Clinical and complex radiological assessment (spinal telemetry in sitting position, CT, MRI) of the pathology was performed. In connection with the atypical course of the disease, a pathohistological study of muscle tissue fragments was performed with their fixation in a solution of 10 % neutral formalin. After histological processing, the fragments were embedded in paraffin and stained with hematoxylin-eosin by the Masson trichromic method and by the PTAH method. The preparations were examined using the Pannoramic MIDI II BF hardware and software complex to obtain digital images using the Whole slide imaging technology in the Single layer and Extended focus modes and an AxioScope.A1 stereomicroscope with a digital camera.Results. Two patterns of distribution of nemaline rods of various density and configuration stained dark blue (by PTAH) were identified in sarcoplasm of parts of muscular tissues: diffusely throughout the myocyte and forming clusters of various sizes in transverse sections, and as filamentous structures – in longitudinal ones. Extensive fields of fatty degeneration, transformation of fibers into lipocytes, contracturely changed fibers, pictures of myophaga and areas of fibrosis of the interstitial space associated with residual myocytes were noted. Intramuscular nervous conductors were subjected to complete involution, singular nervous fibers were deformed, internal space was fibrotic. Neuromuscular spindles had unusually increased connective tissue capsule filled with lipocytes. Arterial vessels had fibrosis of t. adventicia and t. media, narrowed or obliterated lumen, and venous vessels were thin-walled and tortuous, which causes their permeability and can cause hemorrhage. The revealed changes in myocytes with the presence of nemaline rods are characteristic of primary nemaline myopathy.Conclusion. The atypicality of clinical manifestations, primarily of the neurological status in scoliosis, requires excluding neuromuscular disease by conducting a histopathological examination of the surgical material.

Emerging Strategies Targeting Catabolic Muscle Stress Relief.

Skeletal muscle wasting represents a common trait in many conditions, including aging, cancer, heart failure, immobilization, and critical illness. Loss of muscle mass leads to impaired functional mobility and severely impedes the quality of life. At present, exercise training remains the only proven treatment for muscle atrophy, yet many patients are too ill, frail, bedridden, or neurologically impaired to perform physical exertion. The development of novel therapeutic strategies that can be applied to an in vivo context and attenuate secondary myopathies represents an unmet medical need. This review discusses recent progress in understanding the molecular pathways involved in regulating skeletal muscle wasting with a focus on pro-catabolic factors, in particular, the ubiquitin-proteasome system and its activating muscle-specific E3 ligase RING-finger protein 1 (MuRF1). Mechanistic progress has provided the opportunity to design experimental therapeutic concepts that may affect the ubiquitin-proteasome system and prevent subsequent muscle wasting, with novel advances made in regards to nutritional supplements, nuclear factor kappa-light-chain-enhancer of activated B cells (NFκB) inhibitors, myostatin antibodies, β2 adrenergic agonists, and small-molecules interfering with MuRF1, which all emerge as a novel in vivo treatment strategies for muscle wasting.

Neuromuscular Disease Affecting the Larynx.

Neuromuscular diseases (NMDs) are diseases involving the motor nuclei of the cranial nerves and anterior horn cells of the spinal cord, the peripheral nerves, the neuromuscular junction, and/or muscle itself. The following groups of disorders from the WHO International Classification of Diseases are usually included in the classification of NMDs: (1) Motor neuron diseases or related disorders; (2) Disorders of nerve root, plexus or peripheral nerves; (3) Diseases of neuromuscular junction or muscle. These diseases include myasthenia gravis or certain specified neuromuscular junction disorders, primary disorders of muscle, and secondary myopathies. The following discussion selects NMDs from each group, which may have unique laryngeal features and more commonly involve the laryngologist and highlights different diagnostic and treatment considerations related to the larynx.

СОВРЕМЕННЫЙ ПОДХОД К ДИАГНОСТИКЕ И ЛЕЧЕНИЮ ПЕРВИЧНЫХ И ВТОРИЧНЫХ МИОПАТИЙ

СОВРЕМЕННЫЙ ПОДХОД К ДИАГНОСТИКЕ И ЛЕЧЕНИЮ ПЕРВИЧНЫХ И ВТОРИЧНЫХ МИОПАТИЙ

Secondary myopathy due to systemic diseases.

BackgroundSome systemic diseases also affect the skeletal muscle to various degrees and with different manifestations. This review aimed at summarizing and discussing recent advances concerning the management of muscle disease in systemic diseases.MethodLiterature review by search of MEDLINE, and Current Contents with appropriate search terms.ResultsSecondary muscle disease occurs in infectious disease, endocrine disorders, metabolic disorders, immunological disease, vascular diseases, hematological disorders, and malignancies. Muscle manifestations in these categories include pathogen‐caused myositis, muscle infarction, rhabdomyolysis, myasthenia, immune‐mediated myositis, necrotising myopathy, or vasculitis‐associated myopathy. Muscle affection may concern only a single muscle, a group of muscles, or the entire musculature. Severity of muscle affection may be transient or permanent, may be a minor part of or may dominate the clinical picture, or may be mild or severe, requiring invasive measures including artificial ventilation if the respiratory muscles are additionally involved. Diagnostic work‐up is similar to that of primary myopathies by application of non‐invasive and invasive techniques. Treatment of muscle involvement in systemic diseases is based on elimination of the underlying cause and supportive measures. The prognosis is usually fair if the causative disorder is effectively treatable but can be fatal in single cases if the entire musculature including the respiratory muscles is involved, in case of infection, or in case of severe rhabdomyolysis.ConclusionSecondary muscle manifestations of systemic diseases must be addressed and appropriately managed. Prognosis of secondary muscle disease in systemic diseases is usually fair if the underlying condition is accessible to treatment.

Особливості запальної відповіді у пацієнтів, хворих на ювенільний ревматоїдний артрит з проявами синдрому вторинної міопатії

Особливості запальної відповіді у пацієнтів, хворих на ювенільний ревматоїдний артрит з проявами синдрому вторинної міопатії

Оцінка інтенсивності больового синдрому у дітей, хворих на ювенільний ревматоїдний артрит з клінічними проявами вторинної міопатії

Оцінка інтенсивності больового синдрому у дітей, хворих на ювенільний ревматоїдний артрит з клінічними проявами вторинної міопатії

Оцінка функціонального стану нервово-м'язової системи у дітей, хворих на ювенільний ревматоїдний артрит з проявами синдрому вторинної міопатії

Оцінка функціонального стану нервово-м'язової системи у дітей, хворих на ювенільний ревматоїдний артрит з проявами синдрому вторинної міопатії

Monitoring of the treatment effect of the secondary myopathy syndrome in patients with juvenile rheumatoid arthritis

Dudnyk V. M., Bereznyckyi O. V., Vyzhga Yu. V. Monitoring of the treatment effect of the secondary myopathy syndrome in patients with juvenile rheumatoid arthritis. Journal of Education, Health and Sport. 2015;5(8):443-447. ISSN 2391-8306. DOI 10.5281/zenodo.31323 http://dx.doi.org/10.5281/zenodo.31323 http://ojs.ukw.edu.pl/index.php/johs/article/view/2015%3B5%288%29%3A443-447 https://pbn.nauka.gov.pl/works/632554 Formerly Journal of Health Sciences. ISSN 1429-9623 / 2300-665X. Archives 2011–2014 http://journal.rsw.edu.pl/index.php/JHS/issue/archive Deklaracja. Specyfika i zawartośc merytoryczna czasopisma nie ulega zmianie. Zgodnie z informacją MNiSW z dnia 2 czerwca 2014 r., ze w roku 2014 nie bedzie przeprowadzana ocena czasopism naukowych; czasopismo o zmienionym tytule otrzymuje tyle samo punktow co na wykazie czasopism naukowych z dnia 31 grudnia 2014 r. The journal has had 5 points in Ministry of Science and Higher Education of Poland parametric evaluation. Part B item 1089. (31.12.2014). © The Author (s) 2015; This article is published with open access at Licensee Open Journal Systems of Kazimierz Wielki University in Bydgoszcz, Poland and Radom University in Radom, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. This is an open access article licensed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 26.08.2015. Revised 05.09.2015. Accepted: 24.09.2015. Monitoring of the treatment effect of the secondary myopathy syndrome in patients with juvenile rheumatoid arthritis V. M. Dudnyk, O. V. Bereznyckyi, Yu. V. Vyzhga Vinnitsya national medical university N.I. Pirogov memorial Abstaract The syndrome of the secondary myopathy is one of the pathologies that is deeply spreaded in doctor’s practice and has a tendency to be increased being 30 – 60% of all chronicle pain syndromes in rheumatology practice. We provided examination of 43 patients with JRA to monitor effect of the treatment. The data presents that development of the secondary myopathy syndrome in patients with JRA is characterized with severe clinical currency of the disease, high inflammatory activity and significant influence on the quality of patient’s life so needed to be treated specific. Key words: secondary myopathy, juvenile rheumatoid arthritis.

Pena-Shokeir phenotype/fetal akinesia deformation sequence: From placenta to secondary myopathy

Pena-Shokeir phenotype/fetal akinesia deformation sequence: From placenta to secondary myopathy

Heat shock transcription factor 1 expression is spatially distributed and selectively down-regulated in the human placenta during labour

Heat shock transcription factor 1 expression is spatially distributed and selectively down-regulated in the human placenta during labour

Neospora in cattle and dogs: an update

The protozoan parasite Neospora caninum is one of the most frequently diagnosed abortifacient pathogens in cattle in the UK ( AHVLA Surveillance report, 2012 ). It is a leading cause of abortion worldwide but the mechanisms leading to abortion are not fully understood, which is hampering control on farms. In addition, N. caninum is a common cause of clinically relevant secondary inflammatory myopathies in dogs ( Podell, 2002 ). In this article we summarize current knowledge and recent research, and recommend options for control.

Skeletal muscle mitochondrial dysfunction during chronic obstructive pulmonary disease: central actor and therapeutic target

Muscle dysfunction is a common complication and an important prognostic factor in chronic obstructive pulmonary disease (COPD). As therapeutic strategies are still needed to treat this complication, gaining more insight into the process that leads to skeletal muscle decline in COPD appears to be an important issue. This review focuses on mitochondrial involvement in limb skeletal muscle alterations (decreased muscle mass, strength, endurance and power and increased fatigue) in COPD. Mitochondria are the main source of energy for the cells; they are involved in production of reactive oxygen species and activate an important pathway that leads to apoptosis. In COPD patients, skeletal muscles are characterized by decreased mitochondrial density and biogenesis, impaired activity and coupling of mitochondrial respiratory chain complexes, increased mitochondrial production of reactive oxygen species and, possibly, increased apoptosis. Of particular interest, a sedentary lifestyle, hypoxia, hypercapnia, tobacco smoking, corticosteroid therapy and, possibly, inflammation participate in this mitochondrial dysfunction, which is accessible to conventional therapies, such as exercise and tobacco cessation, as well as, potentially, to more innovative approaches, such as antioxidant treatment and supplementation with polyunsaturated fatty acids.

Nonsarcoid granulomatous myopathy: two cases and a review of literature

Granulomatous myopathy is an uncommon skeletal muscles disorder. It can develop in association with other granuloma-forming diseases and is then considered a secondary myopathy or, less frequently, a primary disorder for which no etiology is identified. Studies of granulomatous myopathies have focused on examining the differences between primary and secondary diseases. Herein, we describe two cases of nonsarcoid granulomatous myopathies, for which diagnostic work-up did not reveal an underlying granuloma-causing pathology. The patients exhibited similar histopathological characteristics in skeletal muscle biopsies. However, they had different clinical presentations and therapeutic responses. Specifically, one patient had distal muscle weakness with a poor response to immunosuppressive treatment, whereas the other had a more proximal muscle weakness distribution and a very good response to treatment with corticosteroids and azathioprine, resulting in remission. More studies are warranted to further characterize the clinical course and effect of different treatment modalities on nonsarcoid granulomatous myopathy.

Sudden death possibly related to lenalidomide given for cardiac and muscle AL amyloidosis secondary to light chain deposition disease

Restrictive cardiomyopathy due to AL amyloidosis has not been reported as the cause of sudden death. The risk of sudden death in AL amyloidosis may be further increased by potentially cardiotoxic medication, as in the following case. In a 69-year-old female, AL amyloidosis from light-chain deposition disease manifested as gastrointestinal pseudo-obstruction, restrictive cardiomyopathy, and secondary myopathy. AL amyloidosis was histologically confirmed by endomyocardial biopsy and muscle biopsy. One month after initiation of steroids and lenalidomide the patient suddenly died during sleep. It is speculated that sudden death was due to restrictive cardiomyopathy, cardiotoxicity of lenalidomide, pulmonary embolism, sudden unexplained death in epilepsy syndrome or stroke. The possible causes of sudden death are discussed. This case shows that AL amyloidosis from light-chain deposition disease may predominantly affect the intestines, myocardium and the skeletal muscle and that lenalidomide may have a beneficial effect on the amyloidosis but should be given with caution for its potential arrhythmogenic and thrombogenic side-effects.

Neurologische Erkankungen – Pathophysiologie, Diagnostik und Therapie neuromuskulärer Erkrankungen

Neuromuscular illnesses are characterized by a motor weakness or unusual muscular fatigue. Besides in some diseases sensory disturbances and pains are prominent. The causes of the disturbed interaction between nerve and muscle are varied and reach from illnesses of the motor anterior horn cells of the spinal cord about multifactorial lesions of peripheral nerves, a disturbance of the neuromuscular junction up to primary and secondary myopathies. In addition to the clinical examination electrophysiological procedures, specific laboratory tests from serum and CSF as well as the nerve or muscle biopsy allow a further etiology clarification. Few illnesses are separateable by specific genetic investigations.

Myositides

Idiopathic inflammatory myopathies (IIM) are diseases that are potentially amenable to immunomodulatory therapy. The challenge for the neuropathologist consists in distinguishing these myopathies from secondary inflammatory myopathies, especially in the context of some muscular dystrophies and metabolic diseases that may also show inflammatory infiltrates. There are generalized IIMs (dermatomyositis, polymyositis, sporadic inclusion body myositis) and focal ones (e.g., proliferative myositis, macrophagic myofasciitis). This review provides diagnostic criteria for each of these and includes pathogenetic mechanisms where available.

Atypical manifestation of late onset limb girdle muscular dystrophy presenting with recurrent falling and shoulder dysfunction: a case report

IntroductionMyopathies can be sub-classified into congenital, hereditary, mitochondrial, and secondary myopathies.Congenital myopathies are usually diagnosed post partal or in early childhood. Manifestation in adolescence is uncommon and most cases occur as sporadic mutations. Therefore, there is a risk of under diagnosing this disease in middle-aged patients showing pain, dysfunction, recurrent trauma or falls, where muscle atrophy is seen as a secondary injury.Case presentationOur report is about a 54 year old Caucasian woman with an extended history of pain, loss of function and weakness in her right shoulder. The clinical picture showed a frozen right shoulder. The main finding was a marked limb-muscle atrophy of both delta- und biceps-muscles and a rotator cuff tear that had developed over years. Previous medical consultations attributed the atrophy to recurrent falls, shoulder dysfunction and pain. Conservative treatment (analgesics, physiotherapy, training) had failed.The familiar anamnesis was free of any neurological diseases or other genetic diseases.MRI showed a sub-total proximal muscular limb atrophy and a rotator cuff tear in both shoulders. An incision-biopsy of the right delta- and biceps-muscle revealed a chronical myopathy. The level of creatinkinasis was expected to be high but measurements showed values only slightly above normal. Immunohistochemistry, eventually revealed a mild form of LGMD (type 2I). Due to the pattern of symptoms and diagnostic results we described the case as atypical LGMD.ConclusionOur case presents a phenotype of a late onset of limb girdle muscular dystrophy syndrome associated with shoulder pain and dysfunction and recurrent falls. This kind of disease is not very common. In particular, muscle atrophy in the elderly is generally seen as a secondary injury. This case should remind us of the importance of a differential diagnosis of a late onset of muscular dystrophy-syndrome in the elderly, since an early diagnosis offers more treatment options, therefore preventing a rapid progression.