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Neurology pp 217–251Cite as

Neuromuscular Disorders

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Abstract

This chapter reviews evidence behind the epidemiologic, diagnostic, prognostic, and treatment topics in the more commonly encountered neuromuscular diseases. Clinical questions and evidence-based answers are discussed for amyotrophic lateral sclerosis, diabetic peripheral neuropathy, inclusion body myositis, and myasthenia gravis.

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References

  1. Sorenson E, Stalker A, Kurland L, Windebank A. Amyotrophic lateral sclerosis in Olmsted County, Minnesota, 1925–1998. Neurology. 2002;59:280–2.

    PubMed  Google Scholar 

  2. Fang F, Valdimarsdóttir U, Bellocco R, Ronnevi L, Sparén P, Fall K, et al. Amyotrophic lateral sclerosis in Sweden, 1991–2005. Arch Neurol. 2009;66(4):515–9.

    Article  PubMed  Google Scholar 

  3. Zoccolella S, Beghi E, Palagano G, et al. Analysis of survival and prognostic factors in amyotrophic lateral sclerosis: a population based study. J Neurol Neurosurg Psychiatry. 2008;79:33–7.

    Article  PubMed  CAS  Google Scholar 

  4. Mandrioli J, Faglioni P, Nichelli P, et al. Amyotrophic lateral sclerosis: prognostic indicators of survival. Amyotrophic Lateral Sclerosis. 2006;7:217–26.

    Article  Google Scholar 

  5. Bensimon G, Lacomblez L, Meninger V, et al. A controlled trial of Riluzole in amyotrophic lateral sclerosis. N Engl J Med. 1994;330:585–91.

    Article  PubMed  CAS  Google Scholar 

  6. Miller RG, Mitchell JD, Lyon M, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2007:CD001447.

    Google Scholar 

  7. Fagius J. Chronic cryptogenic polyneuropathy. The search for a cause. Acta Neurol Scand. 1983;67:173–80.

    Article  PubMed  CAS  Google Scholar 

  8. Prineas J. Polyneuropathies of undetermined cause. Acta Neurol Scand. 1970;46:1–72.

    Article  Google Scholar 

  9. Smith AG, Singleton JR. The diagnostic yield of a standardized approach to idiopathic sensory-predominant neuropathy. Arch Intern Med. 2004;164:1021–5.

    Article  PubMed  Google Scholar 

  10. Genuth S, Alberti KG, Bennett P, et al. Follow-up report on the diagnosis of diabetes mellitus. Diabet Care. 2003;26:3160–7.

    Article  Google Scholar 

  11. Singleton JR, Smith AG, Bromberg MB. Increased prevalence of impaired glucose tolerance in patients with painful sensory neuropathy. Diabet Care. 2001;24:1448–53.

    Article  CAS  Google Scholar 

  12. Hoffman-Snyder C, Smith BE, Ross MA, Hernandez J, Bosch EP. Value of the oral glucose tolerance test in the evaluation of chronic idiopathic axonal polyneuropathy. Arch Neurol. 2006;63:1075–9.

    Article  PubMed  Google Scholar 

  13. Centers for Disease Control and Prevention (CDC). The prevalence of diabetes and impaired fasting glucose in adults – United States 1999–2000. MMWR Morb Mortal Wkly Rep. 2003;52:833–7.

    Google Scholar 

  14. Novella SP, Inzucchi SE, Goldstein JM. The frequency of undiagnosed diabetes and impaired glucose tolerance in patients with idiopathic sensory neuropathy. Muscle Nerve. 2001;24:1229–31.

    Article  PubMed  CAS  Google Scholar 

  15. Sumner CJ, Sheth S, Griffin JW, Cornblath DR, Polydefkis M. The spectrum of neuropathy in diabetes and impaired glucose tolerance. Neurology. 2003;60:108–11.

    PubMed  CAS  Google Scholar 

  16. Low PA. Symptomatic treatment of painful neuropathy. JAMA. 1998;280:1863–4.

    Article  PubMed  CAS  Google Scholar 

  17. Ziegler D, Nowak H, Kempler P, Vargha P, Low PA. Treatment of symptomatic diabetic polyneuropathy with antioxidant alpha-lipoic acid: a meta-analysis. Diabet Med. 2004;21:114–21.

    Article  PubMed  CAS  Google Scholar 

  18. The Diabetes Control and Complications Trial Research Group. The effect of intensive diabetes therapy on the development and progression of neuropathy. Ann Intern Med. 1995;122:561–8.

    Google Scholar 

  19. Ziegler D, Ametov A, Barinov A, Dyck PJ, Gurieva I, Low PA, et al. Oral treatment with alpha-lipoic acid improves symptomatic diabetic polyneuropathy: The SYDNEY 2 Trial. Diabet Care. 2006;29:2365–70.

    Article  CAS  Google Scholar 

  20. Tang J, Wingerchuk D, Crum B, Rubin DI, Demaerschalk BM. Alpha-lipoic acid may improve symptomatic diabetic polyneuropathy. Neurologist. 2007;13:164–7.

    Article  PubMed  Google Scholar 

  21. Dyck PJ, Karnes JL, O’Brien PC, Litchy WJ, Low PA, Melton 3rd LJ. The Rochester Diabetic Neuropathy Study: reassessment of tests and criteria for diagnosis and staged severity. Neurology. 1992;42:1164–70.

    PubMed  CAS  Google Scholar 

  22. Dalakas MC. Polymyositis, dermatomyositis and inclusion body myositis. N Engl J Med. 1991;325:1487–98.

    Article  PubMed  CAS  Google Scholar 

  23. Amato AA, Barohn RJ. Idiopathic inflammatory myopathies. Neurol Clin. 1997;15:615–48.

    Article  PubMed  CAS  Google Scholar 

  24. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–7.

    Article  PubMed  CAS  Google Scholar 

  25. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:403–7.

    Article  PubMed  CAS  Google Scholar 

  26. Griggs RC, Askanas V, DiMauro S, et al. Inclusion body myositis and myopathies. Ann Neurol. 1995;38:705–13.

    Article  PubMed  CAS  Google Scholar 

  27. Hoogendijk JE, Amato AA, Lecky BR, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10–12 October 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004;14:337–45.

    Article  PubMed  Google Scholar 

  28. Lotz BP, Engel AG, Nishino H, et al. Inclusion body myositis. Observations in 40 patients. Brain. 1989;112:727–47.

    Article  PubMed  Google Scholar 

  29. Mendell JR, Sahenk Z, Gales T, et al. Amyloid filaments in inclusion body myositis. Novel findings provide insight into nature of filaments. Arch Neurol. 1991;48:1229–34.

    PubMed  CAS  Google Scholar 

  30. Chahin N, Engel AG. Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM. Neurology. 2008;70:418–24.

    Article  PubMed  Google Scholar 

  31. Dahlbom K, Lindberg C, Oldfors A. Inclusion body myositis: morphological clues to correct diagnosis. Neuromuscul Disord. 2002;12:853–7.

    Article  PubMed  CAS  Google Scholar 

  32. Choy EHS, Hoogendijk JE, Lecky B, et al. Immunosuppressant and immunomodulatory treatment for dermatomyositis and polymyositis. Cochrane Database Syst Rev. 2005;3:CD003643. doi:10.1002/14651858.CD003643.pub2.

  33. Griggs RC. The current status of treatment for inclusion-body myositis. Neurology. 2006;66:S30–2.

    Article  PubMed  Google Scholar 

  34. Brannagan 3rd TH. Current treatments of chronic immune-mediated demyelinating polyneuropathies. Muscle Nerve. 2009;39:563–78.

    Article  PubMed  CAS  Google Scholar 

  35. Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as a treatment for dermatomyositis. N Engl J Med. 1993;329:1993–2000.

    Article  PubMed  CAS  Google Scholar 

  36. Dalakas MC, Sonies S, Dambrosia JM, et al. Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study. Neurology. 1997;48:712–6.

    PubMed  CAS  Google Scholar 

  37. Walter MC, Lochmuller H, Toepfer M, et al. High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study. J Neurol. 2000;247:22–8.

    Article  PubMed  CAS  Google Scholar 

  38. Dalakas MC, Koffman B, Fujii M, et al. A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM. Neurology. 2001;56:323–7.

    PubMed  CAS  Google Scholar 

  39. Phillips LH, Torner JC. Epidemiologic evidence for a changing natural history of myasthenia gravis. Neurology. 1996;47:1233–8.

    PubMed  Google Scholar 

  40. Poulas K, Tsibri E, Kokla A, Papanastasiou D, Tsouloufis T, Marinou M, et al. Epidemiology of seropositive myasthenia gravis in Greece. J Neurol Neurosurg Psych. 2001;71:353–6.

    Article  Google Scholar 

  41. Phillips LH. The epidemiology of Myasthenia Gravis. Ann NY Acad Sci. 2003;998:407–12.

    Article  PubMed  Google Scholar 

  42. Chan KH, Lachance DH, Harper CM, Lennon VA. Frequency of seronegativity in adult-acquired generalized myasthenia gravis. Muscle Nerve. 2007;36:651–8.

    Article  PubMed  CAS  Google Scholar 

  43. Husain AM, Massey JM, Howard JF, Sanders DB. Acetylcholine receptor antibody measurements in acquired myasthenia gravis. Ann NY Acad Sci. 1998;841:471–4.

    Article  PubMed  CAS  Google Scholar 

  44. Oh SJ, Kim DE, Kuruoglu R, Bradley RJ, Dwyer D. Diagnostic sensitivity of the laboratory tests in myasthenia gravis. Muscle Nerve. 1992;15:720–4.

    Article  PubMed  CAS  Google Scholar 

  45. Sanders DB, Howard JF, Johns TR. Single-fiber electromyography in myasthenia gravis. Neurology. 1979;29:68–76.

    PubMed  CAS  Google Scholar 

  46. Padua L, Stalberg E, LoMonaco M, Evoli A, Batocchi A, Tonali P. SFEMG in ocular myasthenia gravis diagnosis. Clin Neurophysiol. 2000;111:1203–7.

    Article  PubMed  CAS  Google Scholar 

  47. Farrugia ME, Weir AI, Cleary M, Cooper S, Metcalfe R, Mallik A. Concentric and single fiber needle electrodes yield comparable jitter results in myasthenia gravis. Muscle Nerve. 2009;39(5):579–85.

    Article  PubMed  Google Scholar 

  48. Benatar M, Hammad M, Doss-Riney H. Concentric-needle single-fiber electromyography for the diagnosis of myasthenia gravis. Muscle Nerve. 2006;34(2):163–8.

    Article  PubMed  Google Scholar 

  49. Sanders DB, Hart IK, Mantegazza R, Shukla SS, Siddiqi ZA, De Baets MHV, et al. An international phase II randomized trial of mycophenolate mofetil in myasthenia gravis. Neurology. 2008;71:400–6.

    Article  PubMed  CAS  Google Scholar 

  50. Muscle Study Group. A trial of mycophenolate mofetil with prednisone as initial immunotherapy in myasthenia gravis. Neurology. 2008;71:394–9.

    Article  Google Scholar 

  51. Gronseth GS, Barohn RJ. Practice parameter: Thymectomy for autoimmune myasthenia gravis (an evidence-based review). Report of the quality standards subcommittee of the American Academy of Neurology. Neurology. 2000;55:7–15.

    PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Neurology Department, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA

    Brian A. Crum MD

  2. Department of Neurology, Mayo Clinic, Jacksonville, FL, USA

    Devon I. Rubin MD & Elliot L. Dimberg MD

  3. Neurology Department, Mayo College of Medicine, Mayo Clinic, Scottsdale, AZ, USA

    Brent P. Goodman MD

Authors
  1. Brian A. Crum MD
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  2. Devon I. Rubin MD
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  3. Elliot L. Dimberg MD
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  4. Brent P. Goodman MD
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Corresponding author

Correspondence to Brian A. Crum MD .

Editor information

Editors and Affiliations

  1. London Health Sciences Centre, Dept. Clinical Neurosciences, University of Western Ontario, Windermere Road 339, London, N6A 5A5, Ontario, Canada

    Jorge G. Burneo

  2. , Neurology, Mayo Clinic Hospital, E. Mayo Blvd. 5777, Phoenix, 85054, Arizona, USA

    Bart M. Demaerschalk

  3. London Health Sciences Centre, Dept. Clinical Neurosciences, University of Western Ontario, Windermere Road 339, London, N6A 5A5, Ontario, Canada

    Mary E. Jenkins

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Crum, B.A., Rubin, D.I., Dimberg, E.L., Goodman, B.P. (2012). Neuromuscular Disorders. In: Burneo, J., Demaerschalk, B., Jenkins, M. (eds) Neurology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-88555-1_9

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  • DOI: https://doi.org/10.1007/978-0-387-88555-1_9

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-88554-4

  • Online ISBN: 978-0-387-88555-1

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