inclusion body myositis

Summary

Summary: Progressive myopathies characterized by the presence of inclusion bodies on muscle biopsy. Sporadic and hereditary forms have been described. The sporadic form is an acquired, adult-onset inflammatory vacuolar myopathy affecting proximal and distal muscles. Familial forms usually begin in childhood and lack inflammatory changes. Both forms feature intracytoplasmic and intranuclear inclusions in muscle tissue. (Adams et al., Principles of Neurology, 6th ed, pp1409-10)

Top Publications

  1. Askanas V, Engel W. Inclusion-body myositis: muscle-fiber molecular pathology and possible pathogenic significance of its similarity to Alzheimer's and Parkinson's disease brains. Acta Neuropathol. 2008;116:583-95 pubmed publisher
    ..Similarities include, in the respective tissues, cellular aging, mitochondrial abnormalities, oxidative and endoplasmic-reticulum stresses, proteasome inhibition and multiprotein aggregates. ..
  2. Guyant Marechal L, Laquerriere A, Duyckaerts C, Dumanchin C, Bou J, Dugny F, et al. Valosin-containing protein gene mutations: clinical and neuropathologic features. Neurology. 2006;67:644-51 pubmed
    ..IBMPFD belongs to a class of genetic diseases associated with an alteration of the ubiquitin-proteasome system. ..
  3. Dalakas M, Koffman B, Fujii M, Spector S, Sivakumar K, Cupler E. A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM. Neurology. 2001;56:323-7 pubmed
    ..with prednisone improves muscle strength and alters endomysial inflammation in patients with sporadic inclusion body myositis (s-IBM)...
  4. Ferrer I, Carmona M, Blanco R, Moreno D, Torrejón Escribano B, Olive M. Involvement of clusterin and the aggresome in abnormal protein deposits in myofibrillar myopathies and inclusion body myositis. Brain Pathol. 2005;15:101-8 pubmed
    ..b>Inclusion body myositis (IBM) is characterized by the presence of rimmed vacuoles, eosinophilic inclusions in the cytoplasm, rare ..
  5. Raju R, Vasconcelos O, Granger R, Dalakas M. Expression of IFN-gamma-inducible chemokines in inclusion body myositis. J Neuroimmunol. 2003;141:125-31 pubmed
    ..of effector T cells, we examined their expression in the muscle biopsies of patients with sporadic inclusion body myositis (s-IBM) and disease controls...
  6. Gossrau G, Gestrich B, Koch R, Wunderlich C, Schroder J, Schroeder S, et al. Apolipoprotein E and alpha-1-antichymotrypsin polymorphisms in sporadic inclusion body myositis. Eur Neurol. 2004;51:215-20 pubmed
    Sporadic inclusion body myositis (s-IBM) is a progressive muscle disease of unknown aetiology...
  7. Broccolini A, Ricci E, Cassandrini D, Gliubizzi C, Bruno C, Tonoli E, et al. Novel GNE mutations in Italian families with autosomal recessive hereditary inclusion-body myopathy. Hum Mutat. 2004;23:632 pubmed
    ..Interestingly, in two of our families distinct mutations affected nucleotide c.616 in exon 3 (c.616delG and c.616G>A). The possibility of specific portions of the gene being more prone to mutations remains to be elucidated. ..
  8. Lindberg C, Klintberg L, Oldfors A. Raised troponin T in inclusion body myositis is common and serum levels are persistent over time. Neuromuscul Disord. 2006;16:495-7 pubmed
    ..CK) and creatine kinase isoenzyme MB (CKMB) were measured in 42 consecutive patients with sporadic inclusion body myositis (s-IBM). 26 patients (62%) had a cTnT level >0...
  9. Dalakas M. Sporadic inclusion body myositis--diagnosis, pathogenesis and therapeutic strategies. Nat Clin Pract Neurol. 2006;2:437-47 pubmed
    Sporadic inclusion body myositis (sIBM) presents with a characteristic clinical phenotype of slow-onset weakness and atrophy, affecting proximal and distal limb muscles and facial and pharyngeal muscles...

More Information

Publications91

  1. Vattemi G, Engel W, McFerrin J, Askanas V. Endoplasmic reticulum stress and unfolded protein response in inclusion body myositis muscle. Am J Pathol. 2004;164:1-7 pubmed
    ..containing either amyloid-beta (Abeta) or phosphorylated tau, are the characteristic feature of sporadic inclusion body myositis (s-IBM) muscle biopsies, we studied expression and immunolocalization of five ER chaperones, calnexin, ..
  2. Müntzing K, Lindberg C, Moslemi A, Oldfors A. Inclusion body myositis: clonal expansions of muscle-infiltrating T cells persist over time. Scand J Immunol. 2003;58:195-200 pubmed
    b>Inclusion body myositis (IBM) is a chronic inflammatory myopathy. The muscle histology is characterized by infiltration of T cells, which invade and apparently destroy muscle fibres...
  3. Li J, Yin C, Okamoto H, Jaffe H, Oldfield E, Zhuang Z, et al. Proteomic analysis of inclusion body myositis. J Neuropathol Exp Neurol. 2006;65:826-33 pubmed
    Sporadic inclusion body myositis (IBM) is the most frequently acquired inflammatory myopathy of late adult life, yet its diagnostic criteria and pathogenesis remain poorly defined...
  4. Kitazawa M, Trinh D, LaFerla F. Inflammation induces tau pathology in inclusion body myositis model via glycogen synthase kinase-3beta. Ann Neurol. 2008;64:15-24 pubmed publisher
    b>Inclusion body myositis (IBM) is an inflammatory muscle disease, although the role of inflammation remains to be elucidated...
  5. Price P, Santoso L, Mastaglia F, Garlepp M, Kok C, Allcock R, et al. Two major histocompatibility complex haplotypes influence susceptibility to sporadic inclusion body myositis: critical evaluation of an association with HLA-DR3. Tissue Antigens. 2004;64:575-80 pubmed
    Previous studies of sporadic inclusion body myositis (sIBM) have shown a strong association with HLA-DR3 and other components of the 8...
  6. Sparks S, Ciccone C, Lalor M, Orvisky E, Klootwijk R, Savelkoul P, et al. Use of a cell-free system to determine UDP-N-acetylglucosamine 2-epimerase and N-acetylmannosamine kinase activities in human hereditary inclusion body myopathy. Glycobiology. 2005;15:1102-10 pubmed
    ..This cell-free approach can be applied to other glycosylation pathway enzymes that are difficult to evaluate in whole cells because their substrate specificities overlap with those of ancillary enzymes. ..
  7. Needham M, Corbett A, Day T, Christiansen F, Fabian V, Mastaglia F. Prevalence of sporadic inclusion body myositis and factors contributing to delayed diagnosis. J Clin Neurosci. 2008;15:1350-3 pubmed publisher
    The prevalence of sporadic inclusion body myositis (sIBM) is variable in different populations and ethnic groups. A previous survey in Western Australia in 2000 found a prevalence of 9.3 per million population...
  8. Needham M, Mastaglia F. Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches. Lancet Neurol. 2007;6:620-31 pubmed
    b>Inclusion body myositis is the most common acquired muscle disease in older individuals, and its prevalence varies among countries and ethnic groups...
  9. Huizing M, Rakocevic G, Sparks S, Mamali I, Shatunov A, Goldfarb L, et al. Hypoglycosylation of alpha-dystroglycan in patients with hereditary IBM due to GNE mutations. Mol Genet Metab. 2004;81:196-202 pubmed
    ..These findings resemble those found for other congenital muscular dystrophies, suggesting that HIBM may be a "dystroglycanopathy," and providing an explanation for the muscle weakness of patients with GNE mutations. ..
  10. Schmidt J, Rakocevic G, Raju R, Dalakas M. Upregulated inducible co-stimulator (ICOS) and ICOS-ligand in inclusion body myositis muscle: significance for CD8+ T cell cytotoxicity. Brain. 2004;127:1182-90 pubmed
    ..Because in the muscle of patients with sporadic inclusion body myositis (sIBM) clonally expanded CD8+ T cells invade major histocompatibility complex (MHC) class I-expressing ..
  11. Dabby R, Lange D, Trojaborg W, Hays A, Lovelace R, Brannagan T, et al. Inclusion body myositis mimicking motor neuron disease. Arch Neurol. 2001;58:1253-6 pubmed
    To describe the clinical and electrophysiologic features of patients with inclusion body myositis that was misinterpreted as motor neuron disease...
  12. Weihl C, Pestronk A, Kimonis V. Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia. Neuromuscul Disord. 2009;19:308-15 pubmed publisher
  13. Hinderlich S, Salama I, Eisenberg I, Potikha T, Mantey L, Yarema K, et al. The homozygous M712T mutation of UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase results in reduced enzyme activities but not in altered overall cellular sialylation in hereditary inclusion body myopathy. FEBS Lett. 2004;566:105-9 pubmed
    ..Nevertheless, no reduced cellular sialylation was found in those cells by colorimetric assays and lectin analysis, indicating that HIBM is not directly caused by an altered overall expression of sialic acids. ..
  14. Karpati G, O Ferrall E. Sporadic inclusion body myositis: pathogenic considerations. Ann Neurol. 2009;65:7-11 pubmed publisher
    Sporadic inclusion body myositis is the commonest acquired disease of skeletal muscles after 50 years of age, and as such it has commanded a great deal of attention of investigators over the past 25 years...
  15. Kannanayakal T, Mendell J, Kuret J. Casein kinase 1 alpha associates with the tau-bearing lesions of inclusion body myositis. Neurosci Lett. 2008;431:141-5 pubmed publisher
    b>Inclusion body myositis and Alzheimer's disease are age-related disorders characterized in part by the appearance of intracellular lesions composed of filamentous aggregates of the microtubule-associated protein tau...
  16. Lunemann J, Schmidt J, Dalakas M, MUNZ C. Macroautophagy as a pathomechanism in sporadic inclusion body myositis. Autophagy. 2007;3:384-6 pubmed
    Skeletal muscle fibers show a high level of constitutive and starvation-induced macroautophagy. Sporadic Inclusion Body Myositis (sIBM) is the most common acquired skeletal muscle disease in patients above the age of 50 years and is ..
  17. Schmidt J, Barthel K, Wrede A, Salajegheh M, Bahr M, Dalakas M. Interrelation of inflammation and APP in sIBM: IL-1 beta induces accumulation of beta-amyloid in skeletal muscle. Brain. 2008;131:1228-40 pubmed publisher
    ..degeneration, the two major hallmarks of the skeletal muscle pathology in sporadic inclusion body myositis (sIBM), have remained elusive...
  18. Ricci E, Broccolini A, Gidaro T, Morosetti R, Gliubizzi C, Frusciante R, et al. NCAM is hyposialylated in hereditary inclusion body myopathy due to GNE mutations. Neurology. 2006;66:755-8 pubmed
    ..If further confirmed in larger series of patients, this may be a useful diagnostic marker of GNE-related HIBM. ..
  19. Askanas V, Engel W. Inclusion-body myositis: a myodegenerative conformational disorder associated with Abeta, protein misfolding, and proteasome inhibition. Neurology. 2006;66:S39-48 pubmed
  20. Badrising U, Maat Schieman M, van Duinen S, Breedveld F, van Doorn P, van Engelen B, et al. Epidemiology of inclusion body myositis in the Netherlands: a nationwide study. Neurology. 2000;55:1385-7 pubmed
    Epidemiologic data on inclusion body myositis (IBM) are scarce, and possibly biased, because they are derived from larger neuromuscular centers...
  21. Broccolini A, Gidaro T, Morosetti R, Gliubizzi C, Servidei T, Pescatori M, et al. Neprilysin participates in skeletal muscle regeneration and is accumulated in abnormal muscle fibres of inclusion body myositis. J Neurochem. 2006;96:777-89 pubmed
    ..We investigated a possible role of NEP in inclusion body myositis (IBM) and other acquired and hereditary muscle disorders and found that in all myopathies NEP expression ..
  22. De Bleecker J, Meire V, Declercq W, Van Aken E. Immunolocalization of tumor necrosis factor-alpha and its receptors in inflammatory myopathies. Neuromuscul Disord. 1999;9:239-46 pubmed
    ..We immunolocalized TNF-alpha and its receptors in polymyositis, inclusion body myositis and dermatomyositis...
  23. van der Meulen M, Hoogendijk J, Moons K, Veldman H, Badrising U, Wokke J. Rimmed vacuoles and the added value of SMI-31 staining in diagnosing sporadic inclusion body myositis. Neuromuscul Disord. 2001;11:447-51 pubmed
    Problems in diagnosing sporadic inclusion body myositis may arise if all clinical features fit a diagnosis of polymyositis, but the muscle biopsy shows some rimmed vacuoles...
  24. Alexandrescu D, Bhagwati N, Fomberstein B, Wolfe D, Feliz A, Wiernik P. Steroid-responsive inclusion body myositis associated with endometrial cancer. Clin Exp Rheumatol. 2005;23:93-6 pubmed
    b>Inclusion body myositis (IBM) is an uncommon chronic inflammatory myopathy. Although the association between other myopathies and cancer has been well established, the relationship between IBM and neoplasia is not completely understood...
  25. Askanas V, Engel W. Inclusion-body myositis, a multifactorial muscle disease associated with aging: current concepts of pathogenesis. Curr Opin Rheumatol. 2007;19:550-9 pubmed
    ..About 100 papers related to the subject were published in 2006 and the first part of 2007 (we cite only articles most relevant to this review)...
  26. Oh T, Brumfield K, Hoskin T, Kasperbauer J, Basford J. Dysphagia in inclusion body myositis: clinical features, management, and clinical outcome. Am J Phys Med Rehabil. 2008;87:883-9 pubmed publisher
    To evaluate the clinical features, treatment strategies, and outcome of dysphagia in patients with inclusion body myositis.
  27. Sallum A, Kiss M, Silva C, Wakamatsu A, Vianna M, Sachetti S, et al. Difference in adhesion molecule expression (ICAM-1 and VCAM-1) in juvenile and adult dermatomyositis, polymyositis and inclusion body myositis. Autoimmun Rev. 2006;5:93-100 pubmed
    ..In contrast, VCAM-1 seems not to play a major role in JDM, as previously described in PM, DM and IBM. Adhesion molecule expression in JDM presents a differential characteristic when compared to PM, DM and IBM...
  28. Greenberg S, Bradshaw E, Pinkus J, Pinkus G, Burleson T, Due B, et al. Plasma cells in muscle in inclusion body myositis and polymyositis. Neurology. 2005;65:1782-7 pubmed
    Previous immunohistochemical studies of muscle from patients with inclusion body myositis and polymyositis found many more T cells than B cells, suggesting a role for intramuscular cell-mediated immune mechanisms rather than humoral ..
  29. Cherin P, Menard D, Mouton P, Viallard J, Le Hello C, Authier F, et al. Macrophagic myofasciitis associated with inclusion body myositis: a report of three cases. Neuromuscul Disord. 2001;11:452-7 pubmed
    We describe three patients with macrophagic myofasciitis and inclusion body myositis. All patients fulfilled diagnostic criteria for inclusion body myositis and myopathologic criteria for macrophagic myofasciitis...
  30. Tsuruta Y, Yamada T, Yoshimura T, Satake M, Ogata K, Yamamoto T, et al. Inclusion body myositis associated with hepatitis C virus infection. Fukuoka Igaku Zasshi. 2001;92:370-6 pubmed
    b>Inclusion body myositis (IBM) is a chronic progressive inflammatory myopathy in elders. Three patients with chronic hepatitis C developed IBM...
  31. Rutkove S, Parker R, Nardin R, Connolly C, Felice K, Raynor E. A pilot randomized trial of oxandrolone in inclusion body myositis. Neurology. 2002;58:1081-7 pubmed
    b>Inclusion body myositis (IBM) remains without effective therapy. As anabolic steroids have myotrophic properties, the authors studied whether a synthetic androgen, oxandrolone, would have efficacy in IBM.
  32. Matsuura E, Umehara F, Nose H, Higuchi I, Matsuoka E, Izumi K, et al. Inclusion body myositis associated with human T-lymphotropic virus-type I infection: eleven patients from an endemic area in Japan. J Neuropathol Exp Neurol. 2008;67:41-9 pubmed
    ..was to investigate the association of human T-lymphotropic virus-type I (HTLV-I) infection with sporadic inclusion body myositis in 11 patients from an endemic area in Japan...
  33. Sugarman M, Kitazawa M, Baker M, Caiozzo V, Querfurth H, LaFerla F. Pathogenic accumulation of APP in fast twitch muscle of IBM patients and a transgenic model. Neurobiol Aging. 2006;27:423-32 pubmed
    b>Inclusion body myositis (IBM) is the most common age-related degenerative skeletal muscle disorder. The aberrant intracellular accumulation of the beta-amyloid (Abeta) peptide within skeletal muscle is a pathological hallmark of IBM...
  34. Forman M, Mackenzie I, Cairns N, Swanson E, Boyer P, Drachman D, et al. Novel ubiquitin neuropathology in frontotemporal dementia with valosin-containing protein gene mutations. J Neuropathol Exp Neurol. 2006;65:571-81 pubmed
    ..Our findings are consistent with the hypothesis that the pathology associated with VCP gene mutations is the result of impairment of ubiquitin-based degradation pathways...
  35. Krivickas L, Amato A, Krishnan G, Murray A, Frontera W. Preservation of in vitro muscle fiber function in dermatomyositis and inclusion body myositis: a single fiber study. Neuromuscul Disord. 2005;15:349-54 pubmed
    Five patients with untreated dermatomyositis, five with inclusion body myositis, and 16 healthy elderly volunteer subjects (controls) underwent open (dermatomyositis and inclusion body myositis) or percutaneous (controls) muscle biopsy...
  36. Vattemi G, Nogalska A, King Engel W, D Agostino C, Checler F, Askanas V. Amyloid-beta42 is preferentially accumulated in muscle fibers of patients with sporadic inclusion-body myositis. Acta Neuropathol. 2009;117:569-74 pubmed publisher
    ..Thus, in s-IBM muscle fibers, Abeta42 is accumulated more than Abeta40. We suggest that Abeta42 oligomers and their cytotoxicity may play an important role in the s-IBM pathogenesis...
  37. Mastaglia F, Needham M, Scott A, James I, Zilko P, Day T, et al. Sporadic inclusion body myositis: HLA-DRB1 allele interactions influence disease risk and clinical phenotype. Neuromuscul Disord. 2009;19:763-5 pubmed publisher
    ..The findings indicate that interactions between the HLA-DRB1*03 allele and other alleles at the DRB1 locus can influence disease susceptibility and the clinical phenotype in sIBM...
  38. Maurage C, Bussiere T, Sergeant N, Ghesteem A, Figarella Branger D, Ruchoux M, et al. Tau aggregates are abnormally phosphorylated in inclusion body myositis and have an immunoelectrophoretic profile distinct from other tauopathies. Neuropathol Appl Neurobiol. 2004;30:624-34 pubmed
    Sporadic inclusion body myositis (s-IBM) is the most frequent progressive acquired inflammatory myopathy in people older than 50 years...
  39. Nogalska A, Terracciano C, D Agostino C, King Engel W, Askanas V. p62/SQSTM1 is overexpressed and prominently accumulated in inclusions of sporadic inclusion-body myositis muscle fibers, and can help differentiating it from polymyositis and dermatomyositis. Acta Neuropathol. 2009;118:407-13 pubmed publisher
  40. Weihl C, Temiz P, Miller S, Watts G, Smith C, Forman M, et al. TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2008;79:1186-9 pubmed publisher
    ..TDP-43 inclusions were also found in 78% of sporadic inclusion body myositis (sIBM) muscles...
  41. Greenberg S. Inclusion body myositis: review of recent literature. Curr Neurol Neurosci Rep. 2009;9:83-9 pubmed
    b>Inclusion body myositis (IBM) is a progressive inflammatory skeletal muscle disease of unknown cause and without effective treatment. This article discusses existing literature, emphasizing disease mechanisms and models...
  42. Chahin N, Engel A. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology. 2008;70:418-24 pubmed
    To correlate muscle biopsy findings with prebiopsy and postbiopsy clinical course and response to therapy in polymyositis (PM) and sporadic inclusion body myositis (IBM).
  43. Nakano S, Shinde A, Ito H, Kusaka H. Messenger RNA degradation may be inhibited in sporadic inclusion body myositis. Neurology. 2005;65:420-5 pubmed
    To integrate an immune-mediated mechanism and the disturbed protein expression in sporadic inclusion body myositis (IBM).
  44. Sadjadi R, Rose M. What determines quality of life in inclusion body myositis?. J Neurol Neurosurg Psychiatry. 2010;81:1164-6 pubmed publisher
    ..a better choice from among the various ways we currently measure the severity of a muscle disease such as inclusion body myositis (IBM)...
  45. Raju R, Dalakas M. Gene expression profile in the muscles of patients with inflammatory myopathies: effect of therapy with IVIg and biological validation of clinically relevant genes. Brain. 2005;128:1887-96 pubmed
    ..obtained before and after therapy from patients with dermatomyositis (DM) who improved and patients with inclusion body myositis (sIBM) who did not improve after controlled trials with three monthly intravenous immunoglobulin (IVIg) ..
  46. Halawani D, LeBlanc A, Rouiller I, Michnick S, Servant M, Latterich M. Hereditary inclusion body myopathy-linked p97/VCP mutations in the NH2 domain and the D1 ring modulate p97/VCP ATPase activity and D2 ring conformation. Mol Cell Biol. 2009;29:4484-94 pubmed publisher
    ..Therefore, we propose that hIBMPFTD p97/VCP mutants p97(R155P) and p97(A232E) possess structural defects that may compromise the mechanism of p97/VCP activity within large multiprotein complexes...
  47. Selva O Callaghan A, Mijares Boeckh Behrens T, Labrador Horrillos M, Solans Laque R, Ma Grau Junyent J, Vilardell Tarres M. Anti-PM-Scl antibodies in a patient with inclusion body myositis. Rheumatology (Oxford). 2003;42:1016-8 pubmed
  48. Wilson F, Ytterberg S, St Sauver J, Reed A. Epidemiology of sporadic inclusion body myositis and polymyositis in Olmsted County, Minnesota. J Rheumatol. 2008;35:445-7 pubmed
    To determine the incidence and prevalence of sporadic inclusion body myositis (sIBM) and polymyositis (PM) in a population-based study.
  49. Salajegheh M, Rakocevic G, Raju R, Shatunov A, Goldfarb L, Dalakas M. T cell receptor profiling in muscle and blood lymphocytes in sporadic inclusion body myositis. Neurology. 2007;69:1672-9 pubmed
    ..Sporadic IBM (sIBM) is characterized by invasion of non-necrotic MHC-I class-expressing muscle fibers by clonally expanded CD8+ cells. Whether the endomysial cells expand in situ or are recruited from the circulation is unclear...
  50. Fidzianska A, Rowinska Marcinska K, Hausmanowa Petrusewicz I. Coexistence of X-linked recessive Emery-Dreifuss muscular dystrophy with inclusion body myositis-like morphology. Acta Neuropathol. 2004;107:197-203 pubmed
    ..Two patients demonstrated a typical inclusion body myositis (IBM)-like morphology. The third patient had only minor changes...
  51. Savelkoul P, Manoli I, Sparks S, Ciccone C, Gahl W, Krasnewich D, et al. Normal sialylation of serum N-linked and O-GalNAc-linked glycans in hereditary inclusion-body myopathy. Mol Genet Metab. 2006;88:389-90 pubmed
  52. Badrising U, Schreuder G, Giphart M, Geleijns K, Verschuuren J, Wintzen A, et al. Associations with autoimmune disorders and HLA class I and II antigens in inclusion body myositis. Neurology. 2004;63:2396-8 pubmed
    Whether autoimmune mechanisms play a role in the pathogenesis of inclusion body myositis (IBM) is unknown...
  53. Johnson L, Collier K, Edwards D, Philippe D, Eastwood P, Walters S, et al. Improvement in aerobic capacity after an exercise program in sporadic inclusion body myositis. J Clin Neuromuscul Dis. 2009;10:178-84 pubmed publisher
    The study aimed to investigate the effects of a combined functional and aerobic exercise program on aerobic capacity, muscle strength, and functional mobility in a group of patients with sporadic inclusion body myositis (IBM).
  54. Engel W, Askanas V. Inclusion-body myositis: clinical, diagnostic, and pathologic aspects. Neurology. 2006;66:S20-9 pubmed
    ..Available treatments are of only slight, temporary benefit for only some s-IBM patients, indicating a desperate need for definitive therapies...
  55. Broccolini A, Gliubizzi C, Pavoni E, Gidaro T, Morosetti R, Sciandra F, et al. alpha-Dystroglycan does not play a major pathogenic role in autosomal recessive hereditary inclusion-body myopathy. Neuromuscul Disord. 2005;15:177-84 pubmed
    ..Therefore, the subtle changes within the alpha-DG glycosylation pattern, detected in HIBM muscles, likely do not play a key pathogenic role in this disorder...
  56. Ranque Francois B, Maisonobe T, Dion E, Piette J, Chauveheid M, Amoura Z, et al. Familial inflammatory inclusion body myositis. Ann Rheum Dis. 2005;64:634-7 pubmed
    To compare familial inflammatory inclusion body myositis (IBM) with hereditary inclusion body myopathies and sporadic IBM.
  57. Mastaglia F. Sporadic inclusion body myositis: variability in prevalence and phenotype and influence of the MHC. Acta Myol. 2009;28:66-71 pubmed
    Sporadic inclusion body myositis (sIBM) is the most common myopathy presenting over the age of 40 years but its prevalence varies considerably in different populations...
  58. Muth I, Barthel K, Bahr M, Dalakas M, Schmidt J. Proinflammatory cell stress in sporadic inclusion body myositis muscle: overexpression of alphaB-crystallin is associated with amyloid precursor protein and accumulation of beta-amyloid. J Neurol Neurosurg Psychiatry. 2009;80:1344-9 pubmed publisher
    In the pathology of sporadic inclusion body myositis (sIBM), the relevance of cell stress molecules such as the heat shock protein alphaB-crystallin, particularly in healthy appearing muscle fibres, has remained elusive.
  59. Weihl C, Miller S, Hanson P, Pestronk A. Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice. Hum Mol Genet. 2007;16:919-28 pubmed
    ..TgVCP-RH animals will be a valuable tool for understanding the pathogenesis of IBM and the role of the UPS in skeletal muscle...
  60. Lindberg C, Trysberg E, Tarkowski A, Oldfors A. Anti-T-lymphocyte globulin treatment in inclusion body myositis: a randomized pilot study. Neurology. 2003;61:260-2 pubmed
    The authors performed an open, randomized trial in patients with inclusion body myositis comparing 1) 12-month treatment with oral methotrexate 7...
  61. Nishino I, Noguchi S, Murayama K, Driss A, Sugie K, Oya Y, et al. Distal myopathy with rimmed vacuoles is allelic to hereditary inclusion body myopathy. Neurology. 2002;59:1689-93 pubmed
    ..Recently, HIBM was shown to be associated with the mutations in the gene encoding the bifunctional enzyme, UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE)...
  62. Eisenberg I, Avidan N, Potikha T, Hochner H, Chen M, Olender T, et al. The UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase gene is mutated in recessive hereditary inclusion body myopathy. Nat Genet. 2001;29:83-7 pubmed
    ..Our findings indicate that GNE is the gene responsible for recessive HIBM...
  63. Yamada T, Minohara M, Imaiso Y, Sakae N, Hara H, Tanaka K, et al. High-dose vitamin C therapy for inclusion body myositis. Fukuoka Igaku Zasshi. 2001;92:99-104 pubmed
    The efficiency of high-dose vitamin C therapy for inclusion body myositis (IBM) was assessed.
  64. Chen X, Ghribi O, Geiger J. Rabbits fed cholesterol-enriched diets exhibit pathological features of inclusion body myositis. Am J Physiol Regul Integr Comp Physiol. 2008;294:R829-35 pubmed publisher
    Sporadic inclusion body myositis (IBM) is the most common age-related muscle disease in humans; however, its etiology is unknown, there are few animal models for this disease, and effective treatments have not been identified...
  65. Walter M, Lochmuller H, Toepfer M, Schlotter B, Reilich P, Schroder M, et al. High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study. J Neurol. 2000;247:22-8 pubmed
    Sporadic inclusion body myositis (s-IBM) is an acquired inflammatory muscle disease of unknown cause. In general, s-IBM presents with slowly progressive, asymmetric weakness, and atrophy of skeletal muscle...
  66. Pandya J, Fasth A, Zong M, Arnardottir S, Dani L, Lindroos E, et al. Expanded T cell receptor V?-restricted T cells from patients with sporadic inclusion body myositis are proinflammatory and cytotoxic CD28null T cells. Arthritis Rheum. 2010;62:3457-66 pubmed publisher
    Sporadic inclusion body myositis (IBM) is characterized by T cell infiltrates in muscle tissue, but their functional role is unclear...
  67. Salajegheh M, Raju R, Schmidt J, Dalakas M. Upregulation of thrombospondin-1(TSP-1) and its binding partners, CD36 and CD47, in sporadic inclusion body myositis. J Neuroimmunol. 2007;187:166-74 pubmed
    ..The TSP-complex is another inflammatory mediator associated with chronic inflammation in IBM that may perpetuate the immune responses to local antigens in response to TNF-alpha...
  68. Neumann M, Mackenzie I, Cairns N, Boyer P, Markesbery W, Smith C, et al. TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations. J Neuropathol Exp Neurol. 2007;66:152-7 pubmed
    ..TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations...
  69. Scott A, Allcock R, Mastaglia F, Nishino I, Nonaka I, Laing N. Sporadic inclusion body myositis in Japanese is associated with the MHC ancestral haplotype 52.1. Neuromuscul Disord. 2006;16:311-5 pubmed
    In Caucasians, sporadic inclusion body myositis has been associated with the MHC ancestral haplotypes; HLA-A1, B8, DR3 (8.1AH) and HLA-B35, DR1 (35.2AH)...
  70. Weihl C, Dalal S, Pestronk A, Hanson P. Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation. Hum Mol Genet. 2006;15:189-99 pubmed
    ..Undegraded mutant DeltaF508-CFTR also accumulates in these aggregates. We conclude that IBMPFD mutations in p97/VCP disrupt ERAD and that this may contribute to the pathogenesis of IBMPFD...
  71. Schwarzmeier J, Hamwi A, Preisel M, Resl C, Preusser M, Sluga E, et al. Positive troponin T without cardiac involvement in inclusion body myositis. Hum Pathol. 2005;36:917-21 pubmed
    ..A biopsy of the right quadriceps muscle led to the diagnosis of inclusion body myositis. In contrast to cTnT, cardiac troponin I could not be detected retrospectively in any of her serum ..
  72. Wójcik S, Engel W, McFerrin J, Askanas V. Myostatin is increased and complexes with amyloid-beta within sporadic inclusion-body myositis muscle fibers. Acta Neuropathol. 2005;110:173-7 pubmed
    ..Our study suggests that myostatin/myostatin precursor, either alone, or bound to Abeta, may play a novel role in the pathogenesis of s-IBM...
  73. Dalakas M, Hohlfeld R. Polymyositis and dermatomyositis. Lancet. 2003;362:971-82 pubmed
    ..Early initiation of therapy is essential, since both polymyositis and dermatomyositis respond to immunotherapeutic agents. New immunomodulatory agents currently being tested in controlled trials may prove promising for difficult cases...
  74. Parissis D, Karkavelas G, Taskos N, Milonas I. Inclusion body myositis in a patient with a presumed diagnosis of post-polio syndrome. J Neurol. 2003;250:619-21 pubmed
  75. Darvish D, Vahedifar P, Huo Y. Four novel mutations associated with autosomal recessive inclusion body myopathy (MIM: 600737). Mol Genet Metab. 2002;77:252-6 pubmed
  76. Rose M, McDermott M, Thornton C, Palenski C, Martens W, Griggs R. A prospective natural history study of inclusion body myositis: implications for clinical trials. Neurology. 2001;57:548-50 pubmed
    Eleven patients with untreated inclusion body myositis (IBM) were prospectively studied during a 6-month period that included muscle strength, lean body mass, and muscle mass measurements...
  77. Mowzoon N, Sussman A, Bradley W. Mycophenolate (CellCept) treatment of myasthenia gravis, chronic inflammatory polyneuropathy and inclusion body myositis. J Neurol Sci. 2001;185:119-22 pubmed
    ..demyelinating polyneuropathy (CIDP), one patient with secondary polymyositis (PM), and one patient with inclusion body myositis (IBM). Side effects were mild...
  78. Nakano S, Shinde A, Fujita K, Ito H, Kusaka H. Histone H1 is released from myonuclei and present in rimmed vacuoles with DNA in inclusion body myositis. Neuromuscul Disord. 2008;18:27-33 pubmed
    To investigate myonuclear alterations in sporadic inclusion body myositis (s-IBM), we immuno-localized histones in muscles in 11 patients. The examination showed that vacuolar rims were frequently positive for histone H1...
  79. Tateyama M, Fujihara K, Misu T, Itoyama Y. CCR7+ myeloid dendritic cells together with CCR7+ T cells and CCR7+ macrophages invade CCL19+ nonnecrotic muscle fibers in inclusion body myositis. J Neurol Sci. 2009;279:47-52 pubmed publisher
    ..investigated the expression of this chemokine system in the muscles of seven patients with inclusion body myositis (IBM)...
  80. Fratta P, Engel W, McFerrin J, Davies K, Lin S, Askanas V. Proteasome inhibition and aggresome formation in sporadic inclusion-body myositis and in amyloid-beta precursor protein-overexpressing cultured human muscle fibers. Am J Pathol. 2005;167:517-26 pubmed
    ..Accordingly, proteasome dysfunction in s-IBM muscle fibers may play a role in accumulation of misfolded, potentially cytotoxic proteins and may be induced by increased intracellular AbetaPP/Abeta...
  81. Eisenberg I, Grabov Nardini G, Hochner H, Korner M, Sadeh M, Bertorini T, et al. Mutations spectrum of GNE in hereditary inclusion body myopathy sparing the quadriceps. Hum Mutat. 2003;21:99 pubmed
    ..The mechanism leading to this unique phenotype still remains to be elucidated...
  82. van der Meulen M, Hoogendijk J, Jansen G, Veldman H, Wokke J. Absence of characteristic features in two patients with inclusion body myositis. J Neurol Neurosurg Psychiatry. 1998;64:396-8 pubmed
    According to recently published criteria a diagnosis of definite sporadic inclusion body myositis is made if the typical histopathological abnormalities (rimmed vacuoles and abnormal accumulations of proteins, in addition to mononuclear ..