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Urological Care for Patients with Progressive Neurological Conditions pp 151–158Cite as

Spinal Muscular Atrophy/Lambert Eaton Myasthenic Syndrome

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Abstract

Spinal muscular atrophy (SMA) and Lambert Eaton Myasthenic Syndrome (LEMS) are progressive neurologic conditions that manifest as muscular weakness. SMA is hereditary and LEMS is acquired, and LEM is frequently associated with an underlying malignancy such as small cell lung cancer. Both SMA and LEMS patients can develop urinary symptoms as part of the disease progression. SMA can be associated with bothersome nocturia and urinary frequency. Urinary retention has also been reported among LEMS and SMA patients. Urologic treatment goals are supportive and focused on reducing lower urinary tract symptoms and improving bladder emptying, if needed. Both SMA and LEMS patients have high surgical risk due to sensitivity to neuromuscular blocking agents.

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References

  1. Prior TW, Finanger E. Spinal muscular atrophy. GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1352/. Accessed on 03 Dec 2018.

  2. Pellizzoni L, Kataoka N, Charroux B, Dreyfuss G. A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing. Cell. 1998;95:615.

    Article  CAS  PubMed  Google Scholar 

  3. Wehner KA, Ayala L, Kim Y, Young PJ, Hosler BA, Lorson CL, et al. Survival motor neuron protein in the nucleolus of mammalian neurons. Brain Res. 2002;945(2):160–73.

    Article  CAS  PubMed  Google Scholar 

  4. Ogino S, Wilson RB. Genetic testing and risk assessment for spinal muscular atrophy (SMA). Hum Genet. 2002;111:477.

    Article  CAS  PubMed  Google Scholar 

  5. Gendron NH, MacKenzie AE. Spinal muscular atrophy: molecular pathophysiology. Curr Opin Neurol. 1999;12(2):137–42.

    Article  CAS  PubMed  Google Scholar 

  6. Panigrahi I, Kesari A, Phadke SR, Mittal B. Clinical and molecular diagnosis of spinal muscular atrophy. Neurol India. 2002;50(2):117–22.

    CAS  PubMed  Google Scholar 

  7. Motomura M, Johnston I, Lang B, et al. An improved diagnostic assay for Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry. 1995;58:85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Elmqvist D, Lambert EH. Detailed analysis of neuromuscular transmission in a patient with the myasthenic syndrome sometimes associated with bronchogenic carcinoma. Mayo Clin Proc. 1968;43:689.

    CAS  PubMed  Google Scholar 

  9. Benatar M, Blaes F, Johnston I, et al. Presynaptic neuronal antigens expressed by a small cell lung carcinoma cell line. J Neuroimmunol. 2001;113:153.

    Article  CAS  PubMed  Google Scholar 

  10. Wirtz PW, Bradshaw J, Wintzen AR, Verschuuren JJ. Associated autoimmune diseases in patients with the Lambert-Eaton myasthenic syndrome and their families. J Neurol. 2004;251:1255.

    Article  PubMed  Google Scholar 

  11. Wirtz PW, Smallegange TM, Wintzen AR, Verschuuren JJ. Differences in clinical features between the Lambert-Eaton myasthenic syndrome with and without cancer: an analysis of 227 published cases. Clin Neurol Neurosurg. 2002;104:359.

    Article  PubMed  Google Scholar 

  12. Darras BT. Spinal muscular atrophies. Pediatr Clin N Am. 2015;62:743.

    Article  Google Scholar 

  13. Arnold WD, Kassar D, Kissel JT. Spinal muscular atrophy: diagnosis and management in a new therapeutic era. Muscle Nerve. 2015;51:157.

    Article  CAS  PubMed  Google Scholar 

  14. Thomas NH, Dubowitz V. The natural history of type I (severe) spinal muscular atrophy. Neuromuscul Disord. 1994;4:497.

    Article  CAS  PubMed  Google Scholar 

  15. Kolb SJ, Coffey CS, Yankey JW, et al. Natural history of infantile-onset spinal muscular atrophy. Ann Neurol. 2017;82:883.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Kolb SJ, Kissel JT. Spinal muscular atrophy. Neurol Clin. 2015;33:831.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Clark CV, Newsom-Davis J, Sanders MD. Ocular autonomic nerve function in Lambert-Eaton myasthenic syndrome. Eye (Lond). 1990;4 (. Pt 3:473.

    Article  Google Scholar 

  18. O’Suilleabhain P, Low PA, Lennon VA. Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates. Neurology. 1998;50:88.

    Article  PubMed  Google Scholar 

  19. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. O’Neill JH, Murray NM, Newsom-Davis J. The Lambert-Eaton myasthenic syndrome. A review of 50 cases. Brain. 1988;111(Pt 3):577.

    Article  PubMed  Google Scholar 

  21. Preston DC, Shapiro BE. Neuromuscular junction disorders. In: Electromyography and neuromuscular disorders: clinical-electrophysiologic correlations. 3rd ed: Elsevier; 2013. p. 529.

    Google Scholar 

  22. Motomura M, Johnston I, Lang B, et al. An improved diagnostic assay for Lambert-Eaton myasthenic syndrome. J Neurol Neurosurg Psychiatry. 1995;58:85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Lennon VA, Kryzer TJ, Griesmann GE, et al. Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. N Engl J Med. 1995;332:1467.

    Article  CAS  PubMed  Google Scholar 

  24. Oskoui M, Levy G, Garland CJ, et al. The changing natural history of spinal muscular atrophy type 1. Neurology. 2007;69:1931.

    Article  CAS  PubMed  Google Scholar 

  25. Kolb SJ, Kissel JT. Spinal muscular atrophy. Neurol Clin. 2015;33:831.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Zerres K, Rudnik-Schöneborn S. Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classifications. Arch Neurol. 1995;52:518.

    Article  CAS  PubMed  Google Scholar 

  27. Kaufmann P, McDermott MP, Darras BT, et al. Prospective cohort study of spinal muscular atrophy types 2 and 3. Neurology. 2012;79:1889.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Rudnik-Schöneborn S, Hausmanowa-Petrusewicz I, Borkowska J, Zerres K. The predictive value of achieved motor milestones assessed in 441 patients with infantile spinal muscular atrophy types II and III. Eur Neurol. 2001;45:174.

    Article  PubMed  Google Scholar 

  29. Iannaccone ST. Modern management of spinal muscular atrophy. J Child Neurol. 2007;22(8):974–8. https://doi.org/10.1177/0883073807305670.

    Article  PubMed  Google Scholar 

  30. Islander G. Anesthesia and spinal muscle atrophy. Paediatr Anaesth. 2013;23(9):804–16. https://doi.org/10.1111/pan.12159. Epub 2013 Apr 19

    Article  PubMed  Google Scholar 

  31. Graham RJ, Athiraman U, Laubach AE, Sethna NF. Anesthesia and perioperative medical management of children with spinal muscular atrophy. Paediatr Anaesth. 2009;19(11):1054–63. https://doi.org/10.1111/j.1460-9592.2009.03055.x. Epub 2009 Jun 25

    Article  PubMed  Google Scholar 

  32. Weingarten TN, Araka CN, Mogensen ME. Et. Al. Lambert-Eaton myasthenic syndrome during anesthesia: a report of 37 patients. J Clin Anesth. 2014;26(8):648–53. https://doi.org/10.1016/j.jclinane.2014.09.009. Epub 2014 Nov 18

    Article  PubMed  Google Scholar 

  33. von Gontard A, Laufersweiler-Plass C, Backes M, Zerres K, Rudnik-Schöneborn S. Enuresis and urinary incontinence in children and adolescents with spinal muscular atrophy. BJU Int. 2001;88(4):409–13.

    Article  Google Scholar 

  34. Querin G, Bertolin C, Da Re E, et al. Non-neural phenotype of spinal and bulbar muscular atrophy: results from a large cohort of Italian patients. J Neurol Neurosurg Psychiatry. 2016;87(8):810–6. https://doi.org/10.1136/jnnp-2015-311305. Epub 2015 Oct 26

    Article  PubMed  Google Scholar 

  35. Uemura M, Nishimura K, Nakagawa M, et al. A case of Lambert-Eaton myasthnic syndrome associated with small cell lung carcinoma representing as urinary retention. Hinyokika Kiyo. 2003;49(9):535–8.

    PubMed  Google Scholar 

  36. Satoh K, Motomura M, Suzu H, et al. Neurogenic bladder in Lambert-Eaton myasthenic syndrome and its response to 3,4-diaminopyridine. J Neurol Sci. 2001;183(1):1–4.

    Article  CAS  PubMed  Google Scholar 

  37. Cameron AP. Medical management of neurogenic bladder with oral therapy. Transl Androl Urol. 2016;5(1):51–62. https://doi.org/10.3978/j.issn.2223-4683.2015.12.07.

    Article  PubMed  PubMed Central  Google Scholar 

  38. Nitti VW, Khullar V, Kerrebroeck P. Et. al. Mirabegron for the treatment of overactive bladder: a prespecified pooled efficacy analysis and pooled safety analysis of three randomised, double-blind, placebo-controlled, phase III studies. Int J Clin Pract. 2013;67(7):619–32. https://doi.org/10.1111/ijcp.12194. Epub 2013 May 21

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Yeowell G, Smith P, Nazir J, Hakimi Z, Siddiqui E, Fatoye F. Real-world persistence and adherence to oral antimuscarinics and mirabegron in patients with overactive bladder (OAB): a systematic literature review. BMJ Open. 2018;8(11):e021889. https://doi.org/10.1136/bmjopen-2018-021889.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Wöllner J, Pannek J. Initial experience with the treatment of neurogenic detrusor overactivity with a new β-3 agonist (mirabegron) in patients with spinal cord injury. Spinal Cord. 2016;54(1):78–82. https://doi.org/10.1038/sc.2015.195. Epub 2015 Oct 27

    Article  PubMed  Google Scholar 

  41. Peyronnet B, Gamé X, Vurture G, Nitti VW, Brucker BM. Botulinum toxin use in Neurourology. Rev Urol. 2018;20(2):84–93. https://doi.org/10.3903/riu0792.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Tullman M, Chartier-Kastler E, Kohan A. Et. al., Low-dose onabotulinumtoxinA improves urinary symptoms in noncatheterizing patients with MS. Neurology. 2018;91(7):e657–65. https://doi.org/10.1212/WNL.0000000000005991. Epub 2018 Jul 20

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  43. Sadiq A, Brucker BM. Management of neurogenic lower urinary tract dysfunction in multiple sclerosis patients. Curr Urol Rep. 2015;16(7):44. https://doi.org/10.1007/s11934-015-0519-5.

    Article  PubMed  Google Scholar 

  44. Vurture G, Peyronnet B, Feigin A. Et. al. outcomes of intradetrusor onabotulinum toxin a injection in patients with Parkinson’s disease. Neurourol Urodyn. 2018;37(8):2669–77. https://doi.org/10.1002/nau.23717. Epub 2018 May 16

    Article  CAS  PubMed  Google Scholar 

  45. Stoffel JT, Peterson AC, Sandhu JS, AM S, Wei JT, Lightner DJ. AUA white paper on nonneurogenic chronic urinary retention: consensus definition, treatment algorithm, and outcome end points. J Urol. 2017;198(1):153–60. https://doi.org/10.1016/j.juro.2017.01.075. Epub 2017 Feb 3

    Article  PubMed  Google Scholar 

  46. Suzukia Y, Sano N, Shinonaga C, Fukuda M, Hyodo M, Morimoto T. Successful botulinum toxin treatment of dysphagia in a spinal muscular atrophy type 2 patient. Brain and Development. 2007;29(10):662–5. Epub 2007 May 25

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Urology, New York University, Langone Health, New York, NY, USA

    Gregory Vurture, Benoit Peyronnet & Benjamin M. Brucker

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  1. Gregory Vurture
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  2. Benoit Peyronnet
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  3. Benjamin M. Brucker
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Correspondence to Benjamin M. Brucker .

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Editors and Affiliations

  1. Division of Neurourology and Pelvic Reconstruction, Department of Urology, University of Michigan Medical School, Ann Arbor, MI, USA

    John T. Stoffel

  2. Division of Urology, Department of Surgery, Greenville Health System, Greenville, SC, USA

    Elizabeth V. Dray

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Vurture, G., Peyronnet, B., Brucker, B.M. (2020). Spinal Muscular Atrophy/Lambert Eaton Myasthenic Syndrome. In: Stoffel, J.T., Dray, E.V. (eds) Urological Care for Patients with Progressive Neurological Conditions. Springer, Cham. https://doi.org/10.1007/978-3-030-23277-1_17

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  • DOI: https://doi.org/10.1007/978-3-030-23277-1_17

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  • Online ISBN: 978-3-030-23277-1

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