Abstract
Every acute physician has come across elderly patients presenting with muscle weakness and falls. Distinction between normal age related muscle wasting and pathological muscle atrophy can be difficult. It is important for acute physicians to be aware of the differential diagnosis of such a presentation. Inclusion body myositis(IBM) is the most common type of acquired myopathy above the age of 50.1 This article aims to highlight the diagnosis and management of this chronic illness. It is frequently misdiagnosed as polymyositis and wrongly treated with steroids.3 The typical symptoms and signs are illustrated by a recent case which presented to our department (see box 1).
[download-attachments container="div" style="list" dipslay_icon="1" display_count="1" display__size="0"]
References
- Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med 1991; 325: 1487–98.
- Hopkinson ND, Hunt C, Powell RJ, et al. Inclusion body myositis: An under diagnosed condition? Ann Rheum Dis. 1993 Feb; 52(2): 147–51.
- Mastalgia H to Mastalgia FL. Inclusion body myositis: current pathogenetic concepts and diagnostic and therapeutic approaches. Lancet Neurol. 2007
Jul; 6(7): 620–631. - Lofz BP, Engel AG, Nishino H, et al. Inclusion body myositis. Observations in 40 patients. Brain.1989 Jun; (Pt3): 727–747.
- Askanas V, Engel WK. Sporadic IBM and hereditary IBM, Current concepts of diagnosis and pathogenesis. Curr Opin Rheum. 1998 Nov; 10(6): 530–542.
- Shamim EA, Rider LG, Pandey JP, et al. Differences in idiopathic inflammatory myopathy phenotypes and genotypes between Mesoamerican Mestizos and
North American caucasians. Arthritis Rheum 2002; 46: 1885–93. - Flanigan K, Lauria G, Griffin J, et al. Age-related biology and diseases of muscle and nerve. Neurol Clinics of North America. 1998 Aug; 16(3): 660.
- Garlepp MJ, Laing B, et al. HLA associations with inclusion body myositis. Clin Exp Immunol 1994; 98: 40–45.
- Badrising UA, Schreuder GMT, et al. Associations with autoimmune disorders and HLA class I and class II antigens in inclusion body myositis.
Neurology 2004; 63: 2396–2398. - Yunis EJ, Samaha FJ. Inclusion body myositis. Lab Invest.1971 Sep; 25(3):240–248.
- Barohn RJ, Amato AA, et al. Inclusion body myositis: explanation for poor response to immunosuppressive therapy. Neurology 1995; 45: 1302–04.
- Askanas V, Engel WK. Inclusion body myositis: a myodegenerative conformational disorder associated with ABeta protein misfolding, and proteasome inhibition. Neurology 2006; 66: S39–48.
- Askanas V, Engel WK. Sporadic inclusion body myositis and hereditary inclusion body myopathies: diseases of oxidative stress and aging? Arch Neurol 1998; 55: 915–20.
- Askanas V, Engel WK. Sporadic inclusion body myositis and its similarities to Alzheimer disease brain: recent approaches to diagnosis and pathogenesis, and relation to aging. Scand J Rheumatol 1998; 27: 389–405.
- Houser SM, Calabrese LH. Dysphagia in patients with inclusion body myositis. Laryngoscope 1998; 108: 1001–05.
- Griggs RC, Askanas V, etal. Inclusion body myositis and myopathies. Ann Neurol 1995; 38: 705–13.
- Pruitt JN 2nd, Showalter CJ et al. Sporadic inclusion body myositis: counts of different types of abnormal fibres. Ann Neurol 1996; 39: 139–43.
- David - Hilton -Jones. Muscle Diseases - Inflammatory myopathies. Brains diseases of Nervous system; eleventh edition: 492–495.
- Hatanaka Y, Oh SJ. Single-fibre electromyography in sporadic inclusion body myositis. Clinical Neurophysiol. 2007 July; 118(7): 1563–8.
- Dabby R, Lange DJ, et al. Inclusion body myositis mimicking motor neuron disease. Arch Neurol 2001; 58: 1253–56.
- Villanova M, Kawai M. Rimmed vacuoles of IBM and oculopharyngeal muscular dystrophy. Brain Research. 1993; 603, no 2: 343–347.
- Gianvito M, Vincent A. Idiopathic inflammatory myopathies. Immunological aspects. Autoantibodies in neurological diseases: 85.
- Peng A, Koffman BM, et al. Disease progression in sporadic inclusion body myositis: observation in 78 patients. Neurology 2000; 55: 296–298.
- Griggs RC. The current status of treatment for inclusion body myositis. Neurology 2006; 66(suppl 1): S30–2.
- Mastaglia F, Garlepp MJ, et al. Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve 2003; 27: 407–25.
- Barohn RJ, Amato AA, et al. Inclusion body myositis: explanation for poor response to immunosuppressive therapy. Neurology 1995; 45:1302–04.
- Badrising U, Maat-Schieman M, et al. Comparison of weakness progression in inclusion body myositis during treatment with methotrexate or placebo. Ann Neurol 2002 March; 51(3): 369–72.
- Amato A, Barohn R, et al. Inclusion body myositis: treatment with intravenous immunoglobulin. Neurology 1994; 444: 1516–18.
- Dalakas M, Sonies B, et al. Treatment of inclusion body myositis with IVIG: a double-blind, placebo-controlled study. Neurology 1997; 48:712–16.
- Walter M, Lochmuller H, et al. High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study. J Neurol; 247: 22–28.
- Cherin P, Pelletier S, et al. Intravenous immunoglobulin for dysphagia of inclusion body myositis. Neurology 2002; 58: 326.
- Lindberg C, Trysberg E, et al. Anti T-lymphocyte globulin treatment in inclusion body myositis. Neurology 2003; 61: 260–262.
- Darrow DH, Hoffman HT, et al. Management of dysphagia in inclusion body myositis. Arch Otolaryngol Head Neck Surg 1992; 118: 313–17.
- Alexanderon H, Lundberg IE. The role of exercise in the rehabilitation of idiopathic inflammatory myopathies. CurrOpin Rheumatol 2005; 17(2): 164–71.
