Abstract

Proximal muscle weakness can present acutely or subacutely to the Acute Medical Unit. Early diagnosis of the underlying pathology is essential due to life threatening complications such as respiratory failure and cardiac disturbances as well as causing significant levels of disability. The diagnosis requires thorough history-taking and examination to discern evidence of true weakness, assess its onset, distribution and severity followed by extensive investigations including a CK level, which if high should raise suspicion of rhabdomyolysis. Assessment of respiratory function should be done promptly to identify patients with associated respiratory muscle weakness and treatment should not be delayed waiting for definitive and confirmatory investigations. Poor response to treatment is unusual when diagnosis is correct; this raises the possibility of an alternative diagnosis.

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References

1. Alshekhlee A, Kaminski HJ, Ruff RL; Neuromuscular manifestations of endocrine disorders. Neurol Clin 2002; 20: 35–58, v–vi.
2. Rodolico C, Toscano A, Benvenga S, et al; Myopathy as the persistently isolated symptomatology of primary autoimmune hypothyroidism. Thyroid 1998; 8(11): 1033-8.
3. Crum-Cianflone NE; Bacterial, fungal, parasitic, and viral myositis. Clin. Microbiol. Rev 2008; 21: 473-494.
4. Abd TT, Jacobson TA; Statin-induced myopathy: a review and update. Expert Opin. Drug Saf 2011; 10: 373-387.
5. Shek A, Ferrill MJ; Statin-fibrate combination therapy. Ann Pharmacother 2001; 35: 908-917.
6. Pasternak RC, Smith SC, Bairey-Merz CN, et al; ACC/AHA/NHLBI clinical advisory on the use and safety of statins. J Am Coll Cardiol 2002; 40: 567-572.
7. Joint Formulary Committee. British National Formulary. ed 21. London: BMJ Group and Pharmaceutical Press; 2013
8. Oskarsson B; Myopathy: Five New Things. Neurology 2011; 76: S14-19.
9. Pereira RM, Freire de Carvalho J; Glucocorticoid-induced myopathy. Joint Bone Spine 2011; 78: 41-44.
10. Lane RJM, Mastaglia FL. Drug-induced myopathies in man. Lancet 1978; 2: 562-6.
11. Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 2002; 347: 261-71.
12. Proven A, Gabriel SE, O’Fallon WM et al. Polymyalgia rheumatica with low erythrocyte sedimentation rate at diagnosis. J Rheumatol 1999; 26: 1333-7.
13. Kyle V, Hazleman BL. The clinical and laboratory course of polymyalgia rheumatica/giant cell arteritis after the first two months of treatment. Ann Rheum Dis 1993; 52: 847-50.
14. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barre syndrome. Ann Neurol 1990; 27: S21-24.
15. Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326:1130.
16. Paradiso G, Tripoli J, Galicchio S, Fejerman N. Epidemiological, clinical, and electrodiagnostic findings in childhood Guillain-Barre syndrome: a reappraisal. Ann Neurol 1999; 46: 701-07.
17. Polman CH, Reingold SC, Banwell B et al; Diagnostic Criteria for Multiple Sclerosis: 2010 Revisions to the McDonald Criteria. Ann Neurol 2011; 69: 292-302.
18. Russell JW, Berent-Spillson A, Vincent AM et al. Oxidative injury and neuropathy in diabetes and impaired glucose tolerance. Neurobiol Dis. Jun 2008; 30(3): 420-9.
19. Casey EB, Harrison MJG; Diabetic Amyotrophy: A Follow-up Study. BMJ 1972; 1: 656-659.
20. Wirtz PW, Sotodeh M, Nijnuis M, et al; Difference in distribution of muscle weakness between myasthenia gravis and the Lambert– Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry 2002; 73:766–768.
21. Lang B, Newsom-Davis J, Wray D, et al; Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome. Lancet 1981; 2: 224–226.
22. Warren JD, Blumbergs PC, Thompson PD; Rhabdomyolysis: a review. Muscle Nerve 2002; 25(3): 332-47.
23. Dockrell DH, Sundar S, Angus BJ, Hobson RP: Infectious Diseases, Colledge NR, Walker BR, Ralston SH, editors: Davidson’s Principles and Practice of Medicine, ed 21, Edinburgh, Churchill Livingstone 2010; 341-345.
24. Georgakis C-DC, Falasca G, Georgakis A, Heymann WR; Scleromyxedema. Clinics in Dermatology 2006: 24: 493-497.
25. Madariaga MG; Polymyositis-like syndrome in hypothyroidism: review of cases reported over the past twenty-five years. Thyroid 2002; 12: 331–336.
26. Rider LG, Miller FW; Laboratory evaluation of the inflammatory myopathies. Clin Diagn Lab Immunol 1995; 2: 1–9.
27. Love LA, Leff RL, Fraser DD, et al; A new approach to the classification of idiopathic inflammatory myopathy: myositisspecific autoantibodies define useful homogeneous patient groups. Medicine 1991; 70: 360-37.
28. Stern R, Godbold JH, Chess Q, et al; ECG abnormalities in polymyositis. Arch Intern Med 1984; 144: 2185-2189.
29. Aggarwal R, Lebiedz-Odrobina D, Sinha A, Manadan A, Case JP. Serum Cardiac Troponin T, but Not Troponin I, is elevated in Idiopathic Inflammatory Myopathies. The Journal of Rheumatology 2009, 36 (12): 2711-14.
30. Fardet L, Dupuy A, Gain M et al; Factors associated with underlying malignancy in a retrospective cohort of 121 patients with dermatomyositis. Medicine (Baltimore) 2009; 88: 91-97.
31. Hill CL, Zhang Y, Sigurgeirsson B, et al; Frequency of specific cancer types in dermatomyositis and polymyositis: a populationbased study. The Lancet 2001; 357: 96-100.
32. Fudman EJ, Schnitzer TJ; Dermatomyositis without creatine kinase elevation: poor prognostic sign. Am J Med 1986; 80: 329–32.
33. Oddis C; Idiopathic inflammatory myopathies: a treatment update. Curr Rheumatol Rep 2003; 5: 431–6.
34. Mastaglia F, Garlepp M, Phillips B, et al; Inflammatory myopathies: clinical, diagnostic and therapeutic aspects. Muscle Nerve 2003; 27: 407–25.
35. Amato AA, Grigs RC. Treatment of idiopathic inflammatory myopathies. Curr Opin Neurol 2003; 16: 569–75.
36. Kiely P, Heron C, Bruckner F; Presentation and management of idiopathic inflammatory muscle disease: four case reports and commentary from a series of 78 patients. Rheumatology 2003; 42: 575–82.
37. Mastaglia F, Zilko P. Inflammatory myopathies: how to treat the difficult cases. J Clin Neurosci 2003; 10: 99–10