Inclusion body myositis in twins

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.

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1001. Experimental Targeted Gene Therapy for Quadriceps Muscle Weakness for Sporadic Inclusion Body Myositis (sIBM) with Implications for Other Neuromuscular Disorders

Molecular Therapy ◽

10.1016/s1525-0016(16)40404-1 ◽

2008 ◽

Vol 16 ◽

pp. S375-S376

Keyword(s):

Gene Therapy ◽

Inclusion Body ◽

Muscle Weakness ◽

Inclusion Body Myositis ◽

Neuromuscular Disorders ◽

Quadriceps Muscle ◽

Sporadic Inclusion Body Myositis ◽

Targeted Gene Therapy

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

Internal Medicine ◽

10.2169/internalmedicine.55.7285 ◽

2016 ◽

Vol 55 (23) ◽

pp. 3521-3524 ◽

Cited By ~ 1

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

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Familial Inclusion Body Myositis (FIBM)

Revista Neurociências ◽

10.34024/rnc.2009.v17.8579 ◽

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.

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Inclusion-Body Myositis Associated with Alzheimer’s Disease

Case Reports in Medicine ◽

10.1155/2013/536231 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3 ◽

Cited By ~ 4

Author(s):

Danijela Levacic ◽

Leema Reddy Peddareddygari ◽

David Nochlin ◽

Leroy R. Sharer ◽

Raji P. Grewal

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Inclusion Body ◽

Inclusion Body Myositis ◽

Tau Proteins ◽

Muscle Pathology ◽

Rimmed Vacuoles ◽

Pathologic Features ◽

Sporadic Inclusion Body Myositis ◽

Progressive Weakness

Sporadic inclusion-body myositis (s-IBM) is a myopathy that is characterized by progressive weakness and muscle pathology demonstrating inflammation and rimmed vacuoles. In addition, similar to the pathology observed in the brains of patients with Alzheimer’s disease, the deposition of beta-amyloid and phosphorylated tau proteins in muscle fibers has been reported. These shared pathologic features have prompted hypotheses suggesting a shared etiology of these two conditions. We report a case of a 73-year-old woman initially diagnosed with s-IBM who later developed Alzheimer’s disease.

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Sporadic Inclusion Body Myositis: An Acquired Mitochondrial Disease with Extras

Biomolecules ◽

10.3390/biom9010015 ◽

2019 ◽

Vol 9 (1) ◽

pp. 15 ◽

Cited By ~ 3

Author(s):

Boel De Paepe

Keyword(s):

Inclusion Body ◽

Mitochondrial Disease ◽

Inclusion Body Myositis ◽

Late Onset ◽

Mitochondrial Dynamics ◽

Muscle Disorders ◽

Morphological Alterations ◽

Mitochondrial Alterations ◽

Sporadic Inclusion Body Myositis ◽

Mitochondrial Abnormality

The sporadic form of inclusion body myositis (IBM) is the most common late-onset myopathy. Its complex pathogenesis includes degenerative, inflammatory and mitochondrial aspects. However, which of those mechanisms are cause and which effect, as well as their interrelations, remain partly obscured to this day. In this review the nature of the mitochondrial dysregulation in IBM muscle is explored and comparison is made with other muscle disorders. Mitochondrial alterations in IBM are evidenced by histological and serum biomarkers. Muscular mitochondrial dynamics is disturbed, with deregulated organelle fusion leading to subsequent morphological alterations and muscle displays abnormal mitophagy. The tissue increases mitochondrial content in an attempt to compensate dysfunction, yet mitochondrial DNA (mtDNA) alterations and mild mtDNA depletion are also present. Oxidative phosphorylation defects have repeatedly been shown, most notably a reduction in complex IV activities and levels of mitokines and regulatory RNAs are perturbed. Based on the cumulating evidence of mitochondrial abnormality as a disease contributor, it is therefore warranted to regard IBM as a mitochondrial disease, offering a feasible therapeutic target to be developed for this yet untreatable condition.

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