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242 Labioche I, Liozon E, et al Refractory polymyositis responding to in iximab: Extended follow-up Rheumatology (Oxford) 2004;43(4):531 532 243 Sprott H, Glatzel M, et al Treatment of myositis with etanercept (Enbrel), a recombinant human soluble fusion protein of TNF-alpha type II receptor and IgG1 Rheumatology (Oxford) 2004;43(4):524 526 244 Levine TD Rituximab in the treatment of dermatomyositis: An open-label pilot study Arthritis Rheum 2005;52(2): 601 607 245 Dau PC Plasmapheresis in idiopathic in ammatory myopathy Experience with 35 patients Arch Neurol 1981; 38(9):544 552 246 Herson S, Cherin P, et al The association of plasma exchange synchronized with intravenous gamma globulin therapy in severe intractable polymyositis J Rheumatol 1992;19(5):828 829 247 Miller FW, Leitman SF, et al Controlled trial of plasma exchange and leukopheresis in polymyositis and dermatomyositis N Engl J Med 1992;326(21):1380 1384 248 Hubbard WN, Walport MJ, et al Remission from polymyositis after total body irradiation Br Med J (Clin Res Ed) 1982;284(6333):1915 1916 249 Kelly JJ, Madoc-Jones H, et al Response to total body irradiation in dermatomyositis Muscle Nerve 1988;11(2):120 123 250 Morgan SH, Bernstein RM, et al Total body irradiation and the course of polymyositis Arthritis Rheum 1985;28(7): 831 835 251 Cherin P, Herson S, et al Failure of total body irradiation in polymyositis: Report of three cases Br J Rheumatol 1992;31(4):282 283 252 Kelly JJ, Jr, Madoc-Jones H, et al Total body irradiation not effective in inclusion body myositis Neurology 1986;36(9):1264 1266 253 Askanas V, Alvarez RB, et al beta-Amyloid precursor epitopes in muscle bers of inclusion body myositis Ann Neurol 1993;34(4):551 560 254 Majithia V, Harisdangkul V Mycophenolate mofetil (Cellcept): An alternative therapy for autoimmune in ammatory myopathy Rheumatology 2005;44(3):386 389 255 Tausche AK, Meurer M Mycophenolate mofetil for dermatomyositis Dermatology 2001;202:341 343
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Myopathies Associated with Systemic Disease
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Myopathies can occur in the setting of a variety of neuromuscular disorders Previous chapters have discussed in ammatory myopathies that can occur in the setting of connective tissue diseases (eg, systemic lupus erythematosus, mixed connective tissue disease, Sjogren syndrome, and rheumatoid arthritis) and systemic infections (eg, HIV) Myopathies occurring as complications of medications (toxic myopathies) are also dealt with elsewhere in the book In this chapter we will focus on myopathies related to endocrine disturbances, electrolyte imbalance, nutritional de ciency, and amyloidosis Thyrotoxic myopathy is characterized by proximal muscle weakness and atrophy2 4,6,7 Some individuals have severe shoulder girdle atrophy and scapular winging2 Distal extremity weakness can be the predominant feature in approximately 20% of patients4 Myalgias and fatigue are common Some patients develop dysphagia, dysphonia, and respiratory distress due to involvement of bulbar, esophageal-pharyngeal muscles, and ventilatory muscles8,9 Weakness of extraocular muscles and proptosis occur in the setting of Grave disease but the sphincters are spared in hyperthyroidism Rarely, rhabdomyolysis with myoglobinuria can develop in severe thyrotoxicosis10 Muscle stretch tendon re exes are often brisk In addition, fasciculations and myokymia are occasionally seen which probably re ects thyrotoxicosis-induced irritability of anterior horn cells or peripheral nerves11 13 An association of hyperthyroidism with peripheral neuropathy is quite rare, but a demyelinating polyneuropathy has been reported11 Other manifestations of hyperthyroidism include nervousness, anxiety, psychosis, tremor, increased perspiration, heat intolerance, palpitations, insomnia, diarrhea, increased appetite, and weight loss Common signs include goiter, tachycardia, atrial brillation, widened pulse pressure, as well as warm, thin, and moist skin Myasthenia gravis can develop in association with Grave disease It can be challenging while trying to distinguish which neuromuscular symptoms are related to Grave disease and which are related to myasthenia gravis Muscle weakness associated with hyperthyroidism does not uctuate or signi cantly improve with anticholinesterase inhibitors Thyrotoxicosis is also associated with an unusual form of hypokalemic periodic paralysis Thyrotoxic periodic paralysis appears to occur sporadically, although there may be an autosomal dominant inherited susceptibility It has been commonly reported in Asians, but it is not restricted to this population2,5 Thyrotoxic periodic paralysis is also more common in males The attacks of weakness are similar in onset, frequency, duration, and pattern familial hypokalemic periodic paralysis (see 29) While most cases of hypokalemic periodic paralysis occur within the rst three decades of
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