TDP-43 plasma levels do not differentiate sporadic inclusion body myositis from other inflammatory myopathies

2010 ◽  
Vol 120 (6) ◽  
pp. 825-826 ◽  
Author(s):  
H. Bea Kuiperij ◽  
Wilson F. Abdo ◽  
Baziel G. van Engelen ◽  
H. Jurgen Schelhaas ◽  
Marcel M. Verbeek
Keyword(s):  
Inclusion Body ◽  
Plasma Levels ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathies ◽  
Sporadic Inclusion Body Myositis

scholarly journals BACE-1, PS-1 and sAPPβ Levels Are Increased in Plasma from Sporadic Inclusion Body Myositis Patients: Surrogate Biomarkers among Inflammatory Myopathies

2015 ◽  
Vol 21 (1) ◽  
pp. 817-823 ◽  
Author(s):  
Marc Catalán-García ◽  
Glòria Garrabou ◽  
Constanza Morén ◽  
Mariona Guitart-Mampel ◽  
Ingrid Gonzalez-Casacuberta ◽  
...  
Keyword(s):  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathies ◽  
Sporadic Inclusion Body Myositis ◽  
Bace 1

scholarly journals Diminished distal dorsal finger wrinkling in patients with sporadic inclusion body myositis

2021 ◽  
Vol 2 (4) ◽  
Author(s):  
Ava Yun Lin ◽  
◽  
Benjamin Koo ◽  
Leo H Wang ◽  
◽  
...  
Keyword(s):  
Motor Neuron ◽  
Inclusion Body ◽  
Inclusion Body Myositis ◽  
Neuromuscular Disorders ◽  
Clinical Signs ◽  
Inflammatory Myopathies ◽  
Delay In Diagnosis ◽  
Sporadic Inclusion Body Myositis ◽  
Distal Interphalangeal ◽  
Physical Exam Finding

Sporadic Inclusion Body Myositis (IBM) is the most common acquired myopathy after the age of 45. Often there is a delay in diagnosis as the disease may be mistaken for other inflammatory myopathies and other neuromuscular disorders such as motor neuron disease. One of the hallmarks of the disease is distal finger flexor weakness but non-neurologists who see IBM may not have the expertise to make this assessment. Other clinical signs that require less expertise may be of value in identifying this disorder. We present three cases of patients with IBM who had loss of wrinkling of the dorsal distal interphalangeal joints of the fingers that was not seen in any control patients who had no finger flexor weakness. The loss of wrinkling of the dorsal distal interphalangeal joints of the fingers is an additional physical exam finding that could distinguish IBM from other neuromuscular disorders.

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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