scholarly journals Symptomatic muscle involvement in neurosarcoidosis: a clinicopathological study of 5 cases

2001 ◽  
Vol 59 (2B) ◽  
pp. 347-352 ◽  
Author(s):  
Rosana Herminia Scola ◽  
Lineu Cesar Werneck ◽  
Daniel Monte Serrat Prevedello ◽  
Priscila Greboge ◽  
Fábio Massaiti Iwamoto
Keyword(s):  
Side Effects ◽  
Normal Values ◽  
Clinical Course ◽  
The Other ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Clinicopathological Study ◽  
Steroid Side Effects

We report on the clinical course and histopathologic muscle alterations of five patients diagnosed with neurosarcoidosis, who underwent biopsy due to their muscle manifestations. The five patients were females and only one was less than 40 years of age. Proximal muscle weakness was presented by all and only two patients complained of myalgia. Only normal values of serum muscle enzymes were detected. Electromyography revealed diverse findings such as normal, myopathic and neuropathic patterns. Granuloma was not present in one muscle biopsy. Two patients thoroughly recovered by taking only prednisone and one patient required a methotrexate addition for 3 months before becoming asymptomatic. The other two patients received azathioprine, one due to steroid side effects but without a satisfactory evolution, and the other to strengthen the prednisone régime, with excellent results.

A Case of Immune-Mediated Necrotizing Myopathy With Associated Skeletal Muscle Involvement by Sarcoid Granulomata: A Rare Association

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S69-S69
Author(s):  
Shalla Akbar ◽  
Sandhya Dasaraju ◽  
Osama Elkadi
Keyword(s):  
Skeletal Muscle ◽  
Muscle Weakness ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Skeletal Muscle Involvement

Abstract Skeletal muscle involvement by noncaseating granulomata occurs in a variety of conditions, including sarcoidosis, infections, and rarely in association with primary inflammatory myopathies such as inclusion body myositis (IBM) and dermatomyositis (DM). Sarcoid myopathy is typically asymptomatic; however, a picture of acute myositis with proximal muscle weakness has been described. Immune-mediated necrotizing myopathy (IMNM) is a subgroup of inflammatory myopathies typically presenting with proximal muscle weakness and markedly elevated muscle enzymes, mostly occurring in the setting of statin treatment. IMNM is associated with positive autoantibodies, but a subset of cases is antibody negative. Here we describe a case of myopathy occurring in association with sarcoidosis with combined features of granulomatous and necrotizing myopathy. The patient was a 54-year-old African American male with medical history significant for statin use 3 years ago, which was discontinued due to myalgia and elevated muscle enzymes and biopsy-proven sarcoidosis diagnosed on a pulmonary lymph node biopsy. He presented with progressive worsening of bilateral proximal weakness involving the upper and lower extremities. Electromyogram showed features of active myopathy with no evidence of peripheral neuropathy. Myositis panel was negative for the following antibodies: anti-Jo1, Mi-2, anti-Ku, PL-7, PL-12, OJ, EJ, and SRP. However, there was elevation of aldolase, CRP, and CK-MB. Biopsy of thigh and deltoid muscle showed necrotic muscle fibers, myophagocytosis with associated minimal inflammation, and multiple well-formed nonnecrotizing granulomas with multinucleated giant cells. Myopathic features include increased internalized nuclei, round atrophic fibers, and scattered split fibers. Specific features of IBM or DM were not present. Conclusion Myopathies developing or worsening after discontinuation of statin are rare. The association of necrotizing myopathy with sarcoidosis is not well described in the literature. Additional studies are warranted to elucidate this association.

scholarly journals Diagnosis of dermatomyositis and polymyositis: a study of 102 cases

2000 ◽  
Vol 58 (3B) ◽  
pp. 789-799 ◽  
Author(s):  
ROSANA HERMINIA SCOLA ◽  
LINEU CESAR WERNECK ◽  
DANIEL MONTE SERRAT PREVEDELLO ◽  
EDIMAR LEANDRO TODERKE ◽  
FÁBIO MASSAITI IWAMOTO
Keyword(s):  
Weight Loss ◽  
Creatine Kinase ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Malignant Neoplasm ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Systemic Involvement ◽  
Perifascicular Atrophy

Patients with dermatomyositis (DM) or polymyositis (PM) were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p <0.01]. DM patients complained more arthralgia [p <0.05], dysphagia [p <0.03] and weight loss [p <0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p <0.002]. PM presented more significant increase in creatine kinase (CK) [p <0.02] and in alanine aminotransferase (ALT) [p <0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early DM group [p <0.03]. CONCLUSION: A small association with connective-tissue diseases and neoplasms was found. DM and PM are clinically different. DM presents systemic involvement affecting the skin, developing more severe arthralgia, dysphagia and weight loss and presenting higher values of ESR. PM presents a restricted and more significant involvement of muscles generating more weakness complaints and higher levels of serum muscle enzymes.

Polymyositis and Dermatomyositis

2019 ◽  
pp. 187-189
Author(s):  
Josephina A. Vossen
Keyword(s):  
Muscle Biopsy ◽  
Clinical Examination ◽  
Immune Suppression ◽  
Imaging Modality ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Enzyme

Chapter 42 discusses polymyositis and dermatomyositis, which are idiopathic inflammatory myopathies characterized by muscle inflammation, proximal muscle weakness, and elevated muscle enzymes. Idiopathic inflammatory myopathies represent a heterogeneous group of muscle diseases. Diagnosis is based on clinical examination, muscle enzyme laboratory values, electromyography (EMG), and muscle biopsy. MRI is the most important imaging modality used in diagnosis and management. Radiographs and CT may detect calcinosis. Treatment is primarily by immune suppression.

scholarly journals Heart–Lung–Muscle Anti-SAE Syndrome: An Atypical Severe Combination

2018 ◽  
Vol 8 (1) ◽  
pp. 20
Author(s):  
Elisabet Zamora ◽  
Elena Seder-Colomina ◽  
Susana Holgado ◽  
Bibiana Quirant-Sanchez ◽  
José Mate ◽  
...  
Keyword(s):  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Resonance Imaging ◽  
Cardiac Enzymes ◽  
Muscle Involvement ◽  
Progressive Dyspnea ◽  
Nonspecific Interstitial Pneumonia ◽  
Work Up

A 78-year-old man with 3 months of progressive dyspnea, dysphony, dysgeusia, and proximal muscle weakness was diagnosed of probably idiopathic inflammatory myopathy with nonspecific interstitial pneumonia. Variable degrees of atrioventricular block and persistently elevated cardiac enzymes indicated a diagnosis of myocarditis, confirmed with cardiac magnetic resonance imaging and endomyocardial biopsy. A comprehensive immune work-up revealed anti-small ubiquitin-like modifier-1 activating enzyme (anti-SAE) antibody, a novel myositis-specific antibody, previously described mainly with overt cutaneous dermatomyositis and late skeletal muscle manifestations. Here, heart–lung–muscle involvement combined with anti-SAE antibodies was a severe combination.

scholarly journals Isolated Oropharyngeal Dysphagia as the Initial Presentation of Anti-SRP Immune-Mediated Necrotizing Myopathy

2020 ◽  
Author(s):  
Pat Korathanakhun ◽  
Thanyalak Amornpojnimman
Keyword(s):  
Muscle Weakness ◽  
Clinical Course ◽  
Signal Recognition Particle ◽  
Oropharyngeal Dysphagia ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Electrodiagnostic Study

A 51-year-old male initially presented with a progressive course of isolated oropharyngeal dysphagia prior to the clinical course of painful symmetrical proximal muscle weakness without sensory deficit which rendered him to wheelchairbound status within 5 months. The physical examination revealed symmetrical proximal muscle weakness without sensory symptoms. The initial serum creatine kinase was extremely high and the electrodiagnostic study revealed a myopathic pattern. A muscle biopsy of the left biceps suggested a diagnosis of immune-mediated necrotizing myopathy (IMNM) and the serum anti-signal recognition particle (SRP) autoantibody was finally detected. This case presented a rare form of anti-SRP IMNM in which isolated oropharyngeal dysphagia preceded the onset of proximal muscle weakness.

scholarly journals Statin and fibrate associed myopathy: study of eight patients

2004 ◽  
Vol 62 (2a) ◽  
pp. 257-261 ◽  
Author(s):  
Alzira A. Siqueira Carvalho ◽  
Ürsula Waleska Poti Lima ◽  
Raul Alberto Valiente
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Clinical Course ◽  
Lipid Lowering ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Infiltration ◽  
Lipid Lowering Drugs ◽  
Ragged Red Fibers

Lipid-lowering drugs have been occasionally associated with neuromuscular symptoms and muscle biopsy changes. We reported the clinical course and the muscle biopsy in eight patients with hyperlipoproteinemia, treated with lipid -lowering drugs (statins/fibrates). Five patients had myalgias while; in two cases there was proximal muscle weakness. All patients became asymptomatic after the withdrawal of the drug, although creatine kinase remained elevated. We performed muscle biopsy in six cases from three months to two years after suspension of the drug. We found variation in fibers diameters in all cases, with necrosis of fibers in five cases, inflammatory infiltration in one case, the presence of vacuolated fiber in one patient and ragged-red fibers in three subjects. We concluded that although the muscle biopsy findings were not specific, the prolonged use of statins and or fibrates might induce a chronic myopathy even in the absence of symptoms.

scholarly journals Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Naomi Fei ◽  
Sarah Sofka
Keyword(s):  
Polycythemia Vera ◽  
Muscle Weakness ◽  
Case Reports ◽  
Inflammatory Myopathy ◽  
Primary Myelofibrosis ◽  
Clinical Findings ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
First Case

Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association.

scholarly journals Juvenile Onset Clinically Amyopathic Dermatomyositis Presenting as Calcinosis Cutis

2018 ◽  
Vol 8 (2) ◽  
pp. 101-104
Author(s):  
Kuldeep Verma ◽  
Mudita Gupta ◽  
Saru Thakur
Keyword(s):  
Muscle Weakness ◽  
Inflammatory Disorder ◽  
Proximal Muscle Weakness ◽  
Amyopathic Dermatomyositis ◽  
Proximal Muscle ◽  
Juvenile Onset ◽  
Calcinosis Cutis ◽  
Muscle Involvement ◽  
Cutaneous Manifestations ◽  
Clinically Amyopathic Dermatomyositis

Dermatomyositis (DM) is an autoimmune inflammatory disorder characterized by involvement of muscles and skin. Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. Rarely, DM presents with cutaneous features of the disease without muscle involvement for a period of more than six months. Such cases are classified into a category of clinically amyopathic dermatomyositis (CADM) which includes amyopathic DM and hypomyopathic DM. We present a case of a 14-year-old child who presented with calcinosis cutis and cutaneous findings suggestive of the disease without muscle involvement. The child was diagnosed as a case of juvenile onset CADM.J Enam Med Col 2018; 8(2): 101-104

Autoimmune necrotising myopathy and HMGCR antibodies

2018 ◽  
Vol 18 (2) ◽  
pp. 151-155 ◽  
Author(s):  
Kushan Karunaratne ◽  
Dimitri Amiras ◽  
Matthew C Pickering ◽  
Monika Hofer ◽  
Stuart Viegas
Keyword(s):  
Creatine Kinase ◽  
Clinical Course ◽  
Serum Creatine Kinase ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Immunomodulatory Treatment ◽  
Lower Serum ◽  
Immune Mediated ◽  
Lower Serum Cholesterol ◽  
Asymptomatic Elevation

Statins lower serum cholesterol concentrations by inhibiting the enzyme 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR). Muscle side effects are relatively common and include asymptomatic elevation of serum creatine kinase (CK), myalgia, proximal muscle weakness and rhabdomyolysis. More recently, a subset of cases of immune-mediated necrotising myopathy has been found to have antibodies against HMGCR. It is often an aggressive and debilitating myopathy and has a complex pathogenesis characterised by fibre necrosis, usually with minimal associated inflammation. Not all such patients are taking statins. The general consensus is that best treatment involves withdrawing the statin and giving immunosuppressive and immunomodulatory treatment. We describe three cases of HMGCR-related immune-mediated necrotising myopathy, detailing their clinical course and subsequent management, illustrating the spectrum of this disorder.

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