scholarly journals Dysphagia as the Presenting Symptom for Inclusion Body Myositis

2021 ◽  
Vol 9 ◽  
pp. 232470962110502
Author(s):  
Marcus Juan Esteban ◽  
Darine Kassar ◽  
Osvaldo Padilla ◽  
Richard McCallum
Keyword(s):  
Inclusion Body ◽  
Muscle Weakness ◽  
Poor Prognosis ◽  
Inclusion Body Myositis ◽  
Multidisciplinary Approach ◽  
Oral Steroid ◽  
Inflammatory Myopathies ◽  
Major Improvement ◽  
Presenting Symptoms ◽  
Progressive Muscle Weakness

Dysphagia can be one of the manifestations of inflammatory myopathies (IMs). In some patients, it can be one of the presenting symptoms or the only symptom. We present a patient with dysphagia and progressive muscle weakness who was eventually diagnosed with inclusion body myositis (IBM). Treatment with oral steroid provided no major improvement in symptoms and thus was eventually stopped. Dysphagia in IMs is associated with complications and poor prognosis. A multidisciplinary approach is needed in its diagnosis and management as this report exemplifies.

scholarly journals Physical therapy improves motion in a patient with inclusion body myositis - a case report

2020 ◽  
Vol 77 (11) ◽  
pp. 1216-1220
Author(s):  
Jelena Stevanovic ◽  
Maja Vulovic ◽  
Danijela Pavicevic ◽  
Mihailo Bezmarevic ◽  
Andjelka Stojkovic ◽  
...  
Keyword(s):  
Case Report ◽  
Physical Therapy ◽  
Inclusion Body ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Clinical Symptoms ◽  
Inflammatory Myopathy ◽  
Functional Abilities ◽  
Progressive Muscle Weakness

Introduction. Inclusion body myositis (IBM) is a rare form of inflammatory myopathy with a slowly progressive course. It is manifested by early weakness and atrophy of skeletal muscles, especially forearm muscles and the quadriceps. At the very beginning of the disease, clinical symptoms are not pronounced, therefore it is difficult to diagnose. Case report. A forty-eight-year-old female patient visited her doctor due to the weakness of muscles in arms and legs. Five years prior to this, she was treated by a neurologist and a physiatrician on several occasions with different diagnoses for progressive muscle weakness. During the last hospitalization, IBM was diagnosed after the muscle biopsy findings. After the diagnosis, the patient underwent intensive physical therapy in order to preserve the ability to independently perform everyday activities and stability of walk. Conclusion. IBM is a rare clinical entity which often takes several years to be diagnosed. Progressive muscle weakness in elderly should point to possible IBM diagnosis, which is only confirmed by muscle biopsy. Physical therapy has a significant role in the treatment as it leads to improvement of functional abilities of the patients in their daily activities, thus reducing the disability degree.

Idiopathic inflammatory myopathies: polymyositis and dermatomyositis

2011 ◽  
pp. 385-404
Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq
Keyword(s):  
Inclusion Body ◽  
Clinical Features ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Breakdown

Epidemiology and diagnosis 386 Clinical features of polymyositis and dermatomyositis 388 Investigation of polymyositis and dermatomyositis 392 Autoantibodies in myositis 394 Treatment of polymyositis and dermatomyositis 398 Inclusion-body myositis 401 Polymyositis and dermatomyositis in children 402 • The idiopathic inflammatory myopathies are characterized by proximal muscle weakness and evidence of autoimmune-mediated muscle breakdown. These disorders include:...

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

scholarly journals Expression of the inclusion body myopathy 3 mutation in Drosophila depresses myosin function and stability and recapitulates muscle inclusions and weakness

2012 ◽  
Vol 23 (11) ◽  
pp. 2057-2065 ◽  
Author(s):  
Yang Wang ◽  
Girish C. Melkani ◽  
Jennifer A. Suggs ◽  
Anju Melkani ◽  
William A. Kronert ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Muscle Weakness ◽  
Wild Type ◽  
Cytoplasmic Inclusions ◽  
Inclusion Body Myopathy ◽  
Progressive Muscle Weakness ◽  
Drosophila Model ◽  
Dominant Disease ◽  
Patients Experience ◽  
Muscle Myosin

Hereditary myosin myopathies are characterized by variable clinical features. Inclusion body myopathy 3 (IBM-3) is an autosomal dominant disease associated with a missense mutation (E706K) in the myosin heavy chain IIa gene. Adult patients experience progressive muscle weakness. Biopsies reveal dystrophic changes, rimmed vacuoles with cytoplasmic inclusions, and focal disorganization of myofilaments. We constructed a transgene encoding E706K myosin and expressed it in Drosophila (E701K) indirect flight and jump muscles to establish a novel homozygous organism with homogeneous populations of fast IBM-3 myosin and muscle fibers. Flight and jump abilities were severely reduced in homozygotes. ATPase and actin sliding velocity of the mutant myosin were depressed >80% compared with wild-type myosin. Light scattering experiments and electron microscopy revealed that mutant myosin heads bear a dramatic propensity to collapse and aggregate. Thus E706K (E701K) myosin appears far more labile than wild-type myosin. Furthermore, mutant fly fibers exhibit ultrastructural hallmarks seen in patients, including cytoplasmic inclusions containing aberrant proteinaceous structures and disorganized muscle filaments. Our Drosophila model reveals the unambiguous consequences of the IBM-3 lesion on fast muscle myosin and fibers. The abnormalities observed in myosin function and muscle ultrastructure likely contribute to muscle weakness observed in our flies and patients.

Muscle fiber dysfunction contributes to clinical muscle weakness in inclusion body myositis

2017 ◽  
Vol 27 ◽  
pp. S154
Author(s):  
S. Lassche ◽  
A. Rietveld ◽  
A. Heerschap ◽  
J. Van Hees ◽  
M. Hopman ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Muscle Weakness ◽  
Muscle Fiber ◽  
Inclusion Body Myositis

Inclusion body myositis in twins

Neurology ◽  
1998 ◽  
Vol 51 (2) ◽  
pp. 598-600 ◽  
Author(s):  
Anthony A. Amato ◽  
Robert T. Shebert
Keyword(s):  
Genetic Susceptibility ◽  
Inclusion Body ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Late Onset ◽  
Inclusion Body Myopathy ◽  
Different Ages ◽  
Sporadic Inclusion Body Myositis ◽  
Volar Forearm ◽  
Progressive Weakness

Sporadic inclusion body myositis (s-IBM) is characterized by late onset of slowly progressive weakness that involves the quadriceps and volar forearm muscles early in the course of the disease. There are hereditary forms of inclusion body myopathy (h-IBM) that histologically resemble s-IBM. The lack of inflammation on biopsy and the different ages at onset and patterns of muscle weakness distinguish s-IBM from h-IBM. We report twin brothers with the typical clinical and histologic features of s-IBM. The occurrence of s-IBM in these twins suggests the possibility of a genetic susceptibility to developing s-IBM.

Histopathological features of the idiopathic inflammatory myopathies

2018 ◽  
Author(s):  
Marianne de Visser and Eleonora M.A. Aronica
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Adult Patients ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Histopathological Features ◽  
Muscle Biopsies

In adult patients with presumed idipathic inflammatory myopathy (IIM) without a characteristic and diagnostic dermatomyositis rash, muscle biopsy is mandatory to confirm the IIM diagnosis and to exclude a myopathy which would not respond to glucocorticoids or other immunosuppressants, including inclusion body myositis. This chapter discusses when, where, and how to undertake muscle biopsies, when to repeat them, how to interpret their results, and how these relate to IIM subtypes and disease processes.

scholarly journals Sporadic Inclusion Body Myositis Manifesting as Isolated Muscle Weakness of the Finger Flexors Three Years after Disease Onset

2016 ◽  
Vol 55 (23) ◽  
pp. 3521-3524 ◽  
Author(s):  
Yuichi Suwa ◽  
Naoki Suzuki ◽  
Temma Soga ◽  
Ryuhei Harada ◽  
Aya Shibui ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Disease Onset ◽  
Sporadic Inclusion Body Myositis

scholarly journals Sarcoidosis: Is It a Possible Trigger of Inclusion Body Myositis?

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Ali Zakaria ◽  
Issam Turk ◽  
Kenneth Leung ◽  
Ana Capatina-Rata ◽  
Waseem Farra
Keyword(s):  
Inclusion Body ◽  
Muscle Weakness ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Granulomatous Inflammation ◽  
Stage Iv ◽  
Unknown Etiology ◽  
African American Female ◽  
Multisystem Disorder ◽  
Distal Muscle

Sarcoidosis is a multisystem disorder of unknown etiology, characterized pathologically by the presence of nonnecrotizing granulomatous inflammation in affected organs. Although skeletal muscle is involved in 50–80 percent of individuals with sarcoidosis, symptomatic myopathy has been shown to be a rare manifestation of the disease. Inclusion body myositis (IBM) is a rare acquired idiopathic inflammatory myopathy with the insidious onset of asymmetric and distal muscle weakness that characteristically involves the quadriceps, tibialis anterior, and forearm flexors. Moreover, dysphagia can be the presenting complaint in one-third of patients. Herein, we are presenting a case of 67-year-old African American female who presented with one-month history of new onset progressive dyspnea on exertion. She was diagnosed with stage IV sarcoidosis based on chest CT scan findings and transbronchial lung biopsy revealing nonnecrotizing granulomatous inflammation. Over the next three months after her diagnosis, she presented to the hospital with progressive dysphagia associated with asymmetrical distal muscle weakness. A quadriceps muscle biopsy revealed features consistent with inclusion body myositis. We are reporting this case as it may support the hypothesis of sarcoidosis being a trigger that possibly promotes the development of inclusion body myositis, leading to a very poor prognosis.

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