scholarly journals Asymptomatic hyper-creatine-kinase-emia as sole manifestation of inclusion body myositis

2013 ◽  
Vol 5 (2) ◽  
pp. 11 ◽  
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger ◽  
Gabor G. Kovacs
Keyword(s):  
Creatine Kinase ◽  
Inclusion Body ◽  
Muscle Biopsy ◽  
Nerve Conduction ◽  
Inclusion Body Myositis ◽  
Nerve Conduction Studies ◽  
Differential Diagnoses ◽  
Sporadic Inclusion Body Myositis

Sporadic inclusion body myositis (sIBM) usually manifests with painless weakness of the hand, finger and hip flexors. Absence of symptoms or signs, but mild hyper-CK-emia as the sole manifestation of IBM, has not been reported. We report the case of a 73-year-old male who presented with asymptomatic recurrent hyper-CK-emia ranging from 200 to 1324 U/L (n<171 U/L), since 10 years. Clinical neurologic investigation, nerve conduction studies and EMG were non-informative. Muscle biopsy surprisingly revealed sIBM. sIBM may be asymptomatic and may manifest with hyper-CK-emia exclusively. So, it has to be included in the differential diagnoses of asymptomatic hyper-CK-emia.

scholarly journals A Rare Case of Sporadic Inclusion Body Myositis with Atypical Presentation

2021 ◽  
Author(s):  
Manokaran Chinnusamy ◽  
Sathiyanarayanan Janakiraman ◽  
Ramesh Bala Arivazhagan
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Rare Case ◽  
Immunosuppressive Agents ◽  
Inflammatory Myopathy ◽  
Atypical Presentation ◽  
Sporadic Inclusion Body Myositis

AbstractSporadic inclusion body myositis (IBM) is the most common acquired inflammatory myopathy that occurs after the age of 50 years. IBM typically involves wrist and finger flexors and quadriceps, but all sporadic IBM may not have the classic presentation of distal arm and proximal leg involvement. Treating physicians must be aware of this atypical presentation to avoid the misdiagnosis of IBM, leading to treatment with immunosuppressive agents. The aim of this study is to increase the awareness among physicians about the atypical presentation of IBM and to emphasize the importance of muscle biopsy in such cases. Here we report a case of 52 years old male diagnosed with sporadic IBM by muscle biopsy presented with atypical presentation.

scholarly journals Familial Inclusion Body Myositis (FIBM)

2019 ◽  
Vol 17 (2) ◽  
pp. 193-195
Author(s):  
Marco Orsini ◽  
Mariana Pimentel Mello ◽  
Marcos RG de Freitas ◽  
Osvaldo JM Nascimento
Keyword(s):  
Creatine Kinase ◽  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Inflammatory Cell ◽  
Late Onset ◽  
Serum Creatine Kinase ◽  
Inflammatory Cell Infiltration ◽  
Cell Infiltration ◽  
Rimmed Vacuoles ◽  
Sporadic Inclusion Body Myositis

Familial inclusion body myositis (FIBM) is extremely rare. The disease is characterized by relatively late onset, selective and early involvement of quadriceps, forearm and finger flexors, only mild increase of serum creatine kinase CK level, frequent rimmed vacuoles in muscle histopathology with substantial inflammatory cell infiltration. The combination of clinical, histological, immunopathological and immunogenetic features indicates that these patients have a disease identical to sporadic inclusion body myositis.

scholarly journals Granulomatosis-associated myositis

Neurology ◽  
2019 ◽  
Vol 94 (9) ◽  
pp. e910-e920 ◽  
Author(s):  
Yannick Dieudonné ◽  
Yves Allenbach ◽  
Olivier Benveniste ◽  
Sarah Leonard-Louis ◽  
Baptiste Hervier ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Diagnostic Criteria ◽  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Sporadic Inclusion Body Myositis ◽  
Matching Criteria

ObjectiveTo refine the predictive significance of muscle granuloma in patients with myositis.MethodsA group of 23 patients with myositis and granuloma on muscle biopsy (granuloma-myositis) from 8 French and Belgian centers was analyzed and compared with (1) a group of 23 patients with myositis without identified granuloma (control-myositis) randomly sampled in each center and (2) a group of 20 patients with sporadic inclusion body myositis (sIBM) without identified granuloma (control-sIBM).ResultsAll but 2 patients with granuloma-myositis had extramuscular involvement, including signs common in sarcoidosis that were systematically absent in the control-myositis and the control-sIBM groups. Almost half of patients with granuloma-myositis matched the diagnostic criteria for sIBM. In these patients, other than the granuloma, the characteristics of the myopathy and its nonresponse to treatment were similar to the control-sIBM patients. Aside from 1 patient with myositis overlapping with systemic sclerosis, the remaining patients with granuloma-myositis did not match the criteria for a well-defined myositis subtype, suggesting pure sarcoidosis. Matching criteria for sIBM was the sole feature independently associated with nonresponse to myopathy treatment in patients with granuloma-myositis.ConclusionPatients with granuloma-myositis should be carefully screened for sIBM associated with sarcoidosis in order to best tailor their care.

scholarly journals SAT0332 ANTIBODIES AGAINST CYTOSOLIC 5’-NUCLEOTIDASE 1A IN SPORADIC INCLUSION BODY MYOSITIS: ASSOCIATION WITH CLINICAL AND MRI FEATURES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1112.1-1112
Author(s):  
R. Dejthevaporn ◽  
S. Shah ◽  
S. Wastling ◽  
J. Thornton ◽  
T. Yousry ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Rating Scale ◽  
Fatty Infiltration ◽  
Connective Tissue Diseases ◽  
Diagnostic Process ◽  
Antibody Testing ◽  
Muscle Mri ◽  
Mri Features ◽  
Sporadic Inclusion Body Myositis

Background:Autoantibodies directed against cytosolic 5´-nucleotidase 1A (cN1A) have been identified in sporadic inclusion body myositis (sIBM) and other connective tissue diseases. Anti-cN1A antibodies may support the diagnostic process for sIBM as well as potentially provide clues for disease pathogenesis. Nevertheless, the utility of anti-cN1A autoantibody testing in clinical practice remains unclear and requires validation.Objectives:To investigate the association between anti-cN1A antibody status and clinical and MRI features in patients with sIBM.Methods:Data for patients fulfilling European Neuromuscular Centre (ENMC) 2011 criteria for sIBM were obtained from a natural history study database. Demographic, clinical, functional assessment, and muscle MRI data in patients with sIBM who had anti-cN1A autoantibody testing were collected and analysed. Comparisons between subgroups with anti-cN1A antibody status were performed with the Mann-Whitney or Fisher’s exact tests, as appropriate.Results:Forty-nine patients with sIBM had anti-cN1A autoantibody testing, of whom 17 (34.7%) were positive. Twelve patients had muscle MRI performed (seropositivity=5). Demographics, disease duration at antibody testing and overall disease pattern were closely matched in antibody positive and negative cohorts. Dysphagia was more common in the seropositive subgroup (77% vs 47%, p=0.070). Antibody positive patients were more severely affected with a trend to lower IBM functional rating scale (IBMFRS) scores (22.4±8.4 vs 26.7±6.4, p=0.09) with significantly worse ability to climb stairs (0.9±0.9, 1.7±1.1, p=0.02). On T1-weighted MRI more fatty infiltration was found in seropositive patients (Mercuri score: 3.0±0.8 vs 1.7±0.7, p=0.03). Short tau inversion recovery (STIR) hyperintensity was more conspicuous in seropositive patients (STIR extent score: 2.4±0.6 vs 1.4±0.7, p=0.04).Conclusion:There was a trend for more dysphagia and severity of dysphagia in seropositive patients. Differences in upper limb involvement were not seen according to IBMFRS and Medical Research Council (MRC) strength grades. Seropositive patients were more severely affected at the lower limb level, in terms of muscle weakness, physical function, MRI fatty infiltration and muscle inflammation. These results suggest positive antibody status is associated with a worse phenotype. These results have potential implications in clinical trials: whether antibody status influences treatment response should be assessed.Disclosure of Interests:Revadee Dejthevaporn: None declared, Sachit Shah: None declared, Stephen Wastling: None declared, John Thornton: None declared, Tarek Yousry: None declared, Jasper M Morrow: None declared, Pedro M Machado Consultant of: PMM: Abbvie, Celgene, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB, Speakers bureau: PMM: Abbvie, BMS, Lilly, MSD, Novartis, Pfizer, Roche and UCB

Novel demonstration of conformationally modified tau in sporadic inclusion-body myositis muscle fibers

2011 ◽  
Vol 503 (3) ◽  
pp. 229-233 ◽  
Author(s):  
Anna Nogalska ◽  
Carla D’Agostino ◽  
W. King Engel ◽  
Valerie Askanas
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Muscle Fibers ◽  
Sporadic Inclusion Body Myositis

Chronic sarcoid myopathy mimicking sporadic inclusion body myositis

2019 ◽  
Vol 182 ◽  
pp. 84-86 ◽  
Author(s):  
Masayuki Miyazaki ◽  
Madoka Mori-Yoshimura ◽  
Toshiyuki Yamamoto ◽  
Yasushi Oya ◽  
Yuko Saito ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Sporadic Inclusion Body Myositis
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