Skip to main content

Advertisement

SpringerLink
Log in

Sphincter EMG as a diagnostic tool in autonomic disorders

  • REVIEW ARTICLE
  • Published:
Clinical Autonomic Research Aims and scope Submit manuscript

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disease presenting with a combination of parkinsonian, cerebellar, and autonomic (including cardiovascular, urinary, and anorectal) dysfunction. It is pathologically defined, but at present lacks a definitive clinical diagnostic test. The majority of patients with probable MSA have an abnormal sphincter EMG. Patients with idiopathic Parkinson’s disease do not show marked sphincter EMG abnormalities. Therefore, these abnormalities can be used to distinguish MSA from idiopathic Parkinson’s disease in the first 5 years after disease onset. In contrast, similar sphincter EMG abnormalities are found in some, though not many, patients with dementia with Lewy bodies, pure autonomic failure, progressive supranuclear palsy, and spinocerebellar ataxia type 3. Thus, the limitations of the sphincter EMG test should also be kept in mind. Sphincter EMG and relevant sacral autonomic tests are diagnostic tools for autonomic disorders, reflecting the common and significant involvement of the sacral cord in MSA.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Abrams P, Cardozo L, Fall M, Griffiths D, Rosier P, Ulmsten U, van Kerrebrock P, Victor A, Wein A (2002) The standardization of terminology of lower urinary tract function: report from the standardization sub-committee of the international continence society. Neurourol Urodynam 21:167–178

    Article  Google Scholar 

  2. Amborova P, Hubkal P, Ulkova I, Hulin I (2003) The pacemaker activity of interstitial cells of cajal and gastric electrical activity. Physiol Res 52:275–284

    Google Scholar 

  3. Argiolas A, Melis MR (2005) Central control of penile erection: role of the paraventricular nucleus of the hypothalamus. Prog Neurobiol 76:1–21

    Article  PubMed  CAS  Google Scholar 

  4. Bassotti G, Maggio D, Battaglia E, Giulietti O, Spinozzi F, Reboldi G, Serra AM, Emanuelli G, Chiarioni G (2000) Manometric investigation of anorectal function in early and late stage Parkinson’s disease. J Neurol Neurosurg Psychiatry 68:768–770

    Article  PubMed  CAS  Google Scholar 

  5. Beck RO, Betts CD, Fowler CJ (1994) Genitourinary dysfunction in multiple system atrophy: clinical features and treatment in 62 cases. J Urol 151:1336–1341

    PubMed  CAS  Google Scholar 

  6. Betts CD, Kapoor R, Fowler CJ (1992) Pontine pathology and voiding dysfunction. Br J Urol 70:100–102

    Article  PubMed  CAS  Google Scholar 

  7. Blok BF, Holstege G (1999) The central control of micturition and continence: implications for urology. Br J Urol Int 83(Suppl 2):1–6

    Google Scholar 

  8. Blok BF, de Weerd H, Holstege G (1997) The pontine micturition center projects to sacral cord GABA immunoreactive neurons in the cat. Neurosci Lett 233:109–112

    Article  PubMed  CAS  Google Scholar 

  9. Broens P, Vanbeckevoort D, Bellon E, Penninckx F (2002) Combined radiographic and manometric study of rectal filling sensation. Dis Colon Rectum 45:1016–1022

    Article  PubMed  Google Scholar 

  10. Chandiramani VA, Palace J, Fowler CJ (1997) How to recognize patients with parkinsonism who should not have urological surgery. Br J Urol 80:100–104

    PubMed  CAS  Google Scholar 

  11. Daniel SE (1992) The neuropathology and neurochemistry of multiple system atrophy. In: Bannister R, Mathias CJ (eds) Autonomic failure, 3rd edn. Oxford Medical Publications, Oxford, pp 564–585

    Google Scholar 

  12. de Groat WC (2006) Integrative control of the lower urinary tract: preclinical perspective. BJP 147:S25–S40

    Google Scholar 

  13. Dominguez JM, Hull EM (2005) Dopamine, the medial preoptic area, and male sexual behavior. Physiol Behav 86:356–368

    Article  PubMed  CAS  Google Scholar 

  14. Eardley I, Quinn NP, Fowler CJ, Kirby RS, Parkhouse HF, Marsden CD, Bannister R (1989) The value of urethral sphincter electromyography in the differential diagnosis of parkinsonism. Br J Urol 64:360–362

    Article  PubMed  CAS  Google Scholar 

  15. Fisher C, Gross J, Zuch AJ (1965) Cycle of penile erection synchronous with dreaming (REM) sleep. Arch Gen Psychiatry 12:29–45

    PubMed  CAS  Google Scholar 

  16. Fowler CJ (2006) Integrated control of lower urinary tract: clinical perspective. BJP 147:S14–S24

    CAS  Google Scholar 

  17. Fowler CJ, Kirby RS, Harrison MJ, Milroy EJ, Turner-Warwick R (1984) Individual motor unit analysis in the diagnosis of disorders of urethral sphincter innervation. J Neurol Neurosurg Psychiatr 47:637–641

    Article  PubMed  CAS  Google Scholar 

  18. Gilad R, Giladi N, Korczyn AD, Gurevich T, Sadeh M (2001) Quantitative anal sphincter EMG in multisystem atrophy and 100 controls. J Neurol Neurosurg Psychiatr 71:596–599

    Article  PubMed  CAS  Google Scholar 

  19. Giladi N, Simon ES, Korczyn AD, Groozman GB, Orlov Y, Shabtai H, Drory VE (2000) Anal sphincter EMG does not distinguish between multiple system atrophy and Parkinson’s disease. Muscle Nerve 23:731–734

    Article  PubMed  CAS  Google Scholar 

  20. Hansen MB (2003) Neurohumoral control of GI motility. Physiol Res 52:1–30

    PubMed  CAS  Google Scholar 

  21. Hirschkowitz M, Schmidt MH (2005) Sleep-related erections: clinical perspectives and neural mechanisms. Sleep Med Rev 9:311–329

    Article  Google Scholar 

  22. Ito T, Sakakibara R, Uchiyama T, Liu Z, Yamamoto T, Hattori T (2006b) Videomanometry of the pelvic organs; a comparison of the normal lower urinary and GI tracts. Int J Urol 13: 29–35

    Article  PubMed  Google Scholar 

  23. Ito T, Sakakibara R, Yasuda K, Yamamoto T, Uchiyama T, Liu Z, Yamanishi T, Awa Y, Yamamoto K, Hattori T (2006) Incomplete emptying and urinary retention in multiple system atrophy: when does it occur and how do we manage it? Mov Disord 21:816–823

    Article  PubMed  Google Scholar 

  24. Kavia RB, Datta SN, Dasgupta R, Elneil S, Fowler CJ (2006) Urinary retention in women: its causes and management. BJU Int 97:281–287

    Article  PubMed  Google Scholar 

  25. Kavia RBC, Dasgupta R, Fowler CJ (2005) Functional imaging and the central control of the bladder. J Comp Neurol 493:27–32

    Article  PubMed  Google Scholar 

  26. Kellow JE, Delvaux M, Azpiroz F, Camilleri M, Quigley EMM, Thompson DG (1999) Principles of applied neurogastroenterology: physiology/motility-sensation. Gut 45:17–24

    Article  Google Scholar 

  27. Kihira T, Yoshida S, Yoshimasu F, Wakayama I, Yase Y (1997) Involvement of Onuf’s nucleus in amyotrophic lateral sclerosis. J Neurol Sci 147:81–88

    Article  PubMed  CAS  Google Scholar 

  28. Kirchhof K, Mathias CJ, Fowler CJ (1999) The relationship of uro-genital dysfunction to other features of autonomic failure in MSA. Clin Auton Res 9:1–28

    Article  Google Scholar 

  29. Lefaucheur JP (2006) Neurophysiological testing in anorectal disorders. Muscle Nerve 33:324–333

    Article  PubMed  Google Scholar 

  30. Libelius R, Johansson F (2000) Quantitative electromyography of the external anal sphincter in Parkinson’s disease and multiple system atrophy. Muscle Nerve 23:1250–1256

    Article  PubMed  CAS  Google Scholar 

  31. Liu MT, Rayport S, Jiang L, Murphy DL, Gershon MD (2002) Expression and function of 5-HT3 receptors in the enteric neurons of mice lacking the serotonin transporter. Am J Physiol Gastrointest Liver Physiol 283:G1398–G1411

    PubMed  CAS  Google Scholar 

  32. Liu Z, Sakakibara R, Nakazawa K, Uchiyama T, Yamamoto T, Ito T, Hattori T (2004) Micturition-related neuronal firing in the periaqueductal gray area in cats. Neuroscience 126:1075–1082

    Article  PubMed  CAS  Google Scholar 

  33. Mannen T, Iwata M, Toyokura Y, Nagashima K (1982) The Onuf’s nucleus and the external anal sphincter muscles in amyotrophic lateral sclerosis and Shy-Drager syndrome. Acta Neuropathol 58:255–260

    Article  PubMed  CAS  Google Scholar 

  34. Mashidori T, Yamanishi T, Yoshida K, Sakakibara R, Sakurai K, Hirata K (2007) Continuous urinary incontinence presenting as the initial symptoms demonstrating acontractile detrusor and intrinsic sphincter deficiency in multiple system atrophy. Int J Urol 14(10):972–974

    Article  PubMed  Google Scholar 

  35. Matsumoto G, Hisamitsu T, De Groat WC (1995) Role of glutamate and NMDA receptors in the descending limb of the spinobulbospinal micturition reflex pathway of the rat. Neurosci Lett 183:58–61

    Article  PubMed  CAS  Google Scholar 

  36. Nahm F, Freeman R (2003) Sphincter electromyography and multiple system atrophy. Muscle Nerve 28:18–26

    Article  PubMed  Google Scholar 

  37. Onufrowicz B (1899) Notes on the arrangement and function of the cell groups in the sacral region of the spinal cord. J Nerv Ment Dis 26:498–504

    Google Scholar 

  38. Palace J, Chandiramani VA, Fowler CJ (1997) Value of sphincter electromyography in the diagnosis of multiple system atrophy. Muscle Nerve 20:1396–1403

    Article  PubMed  CAS  Google Scholar 

  39. Paviour DC, Williams DC, Fowler CJ, Quinn NP, Lees AJ (2005) Is sphincter electromyography a helpful investigation in the diagnosis of multiple system atrophy? A retrospective study with pathological diagnosis. Mov Disord 20:1425–1430

    Article  PubMed  Google Scholar 

  40. Pellegrinetti A, Moscato G, Siciliano G, Bonuccelli U, Orlandi G, Maritato P, Sartucci F (2003) Electrophysiological evaluation of genito-sphincteric dysfunction in multiple system atrophy. Int J Neurosci 113:1353–1369

    Article  PubMed  CAS  Google Scholar 

  41. Podnar S (2007) Neurophysiology of the neurogenic lower urinary tract disorders. Clin Neurophysiol 118:1423–1437

    Article  PubMed  Google Scholar 

  42. Podnar S, Fowler CJ (2004) Sphincter electromyography in diagnosis of multiple system atrophy: technical issues. Muscle Nerve 29:151–156

    Article  PubMed  Google Scholar 

  43. Podnar S, Rodi Z, Lukanovic A, Trsinar B, Vodusek DB (1999) Standardization of anal sphincter EMG: technique of needle examination. Muscle Nerve 22:400–403

    Article  PubMed  CAS  Google Scholar 

  44. Podnar S, Vodusek DB (2001) Protocol for clinical neurophysiologic examination of the pelvic floor. Neurourol Urodyn 20:669–682

    Article  PubMed  CAS  Google Scholar 

  45. Ravits J, Hallett M, Nilsson J, Polinsky R, Dambrosia J (1996) Electrophysiological tests of autonomic function in patients with idiopathic autonomic failure syndromes. Muscle Nerve 19:758–763

    Article  PubMed  CAS  Google Scholar 

  46. Rodi Z, Denislic M, Vodusek D (1996) External anal sphincter electromyography in the differential diagnosis of parkinsonism. J Neurol Neurosurg Psychiatry 60:460–461

    Article  PubMed  CAS  Google Scholar 

  47. Sakakibara R, Fowler CJ (2001) Brain disease (chapter 9). In: Fowler CJ (ed) Seminars in clinical neurology (by World Federation of Neurology) neurologic bladder, bowel, and sexual function. Elsevier, Boston, pp 229–243

    Google Scholar 

  48. Sakakibara R, Hattori T, Kita K, Arai K, Yamanishi T, Yasuda K (1998) Stress-induced urinary incontinence in patients with spinocerebellar degeneration. J Neurol Neurosurg Psychiatry 64(3):389–391

    Article  PubMed  CAS  Google Scholar 

  49. Sakakibara R, Hattori T, Tojo M, Yamanishi T, Yasuda K, Hirayama K (1993) Micturitional disturbance in progressive supranuclear palsy. J Auton Nerv Syst 45:101–106

    Article  PubMed  CAS  Google Scholar 

  50. Sakakibara R, Hattori T, Uchiyama T, Asahina M, Yamanishi T (2000) Micturitional disturbance in pure autonomic failure. Neurology 54:499–501

    PubMed  CAS  Google Scholar 

  51. Sakakibara R, Hattori T, Uchiyama T, Kita K, Asahina M, Suzuki A, Yamanishi T (2000) Urinary dysfunction and orthostatic hypotension in multiple system atrophy: which is the more common and earlier manifestation? Neurol Neurosurg Psychiatry 68:65–69

    Article  CAS  Google Scholar 

  52. Sakakibara R, Hattori T, Uchiyama T, Yamanishi T (2001) Videourodynamic and sphincter motor unit potential analyses in Parkinson’s disease and multiple system atrophy. J Neurol Neurosurg Psychiatry 71:600–606

    Article  PubMed  CAS  Google Scholar 

  53. Sakakibara R, Hattori T, Yasuda K, Yamanishi T (1996) Micturitional disturbance and pontine tegmental lesion; urodynamic and MRI analyses of the vascular cases. J Neurol Sci 141:105–110

    Article  PubMed  CAS  Google Scholar 

  54. Sakakibara R, Ito T, Uchiyama T, Asahina M, Liu Z, Yamamoto T, Yamanaka Y, Hattori T (2005) Lower urinary tract function in dementia of Lewy body type (DLB). J Neurol Neurosurg Psychiatry 76:729–732

    Article  PubMed  CAS  Google Scholar 

  55. Sakakibara R, Mori M, Fukutake T, Kita K (1997) Orthostatic hypotension in a case with multiple sclerosis. Clin Auton Res 7:163–165

    Article  PubMed  CAS  Google Scholar 

  56. Sakakibara R, Nakazawa K, Shiba K, Nakajima Y, Uchiyama T, Yoshiyama M, Yamanishi T, Hattori T (2002) Firing patterns of micturition-related neurons in the pontine storage centre in cats. Auton Neurosci Basic Clin 99:24–30

    Article  Google Scholar 

  57. Sakakibara R, Odaka T, Uchiyama T, Asahina M, Yamaguchi K, Yamaguchi T, Yamanishi T, Hattori T (2004) Colonic transit time, sphincter EMG and rectoanal videomanometry in multiple system atrophy. Mov Disord 19:924–929

    Article  PubMed  Google Scholar 

  58. Sakakibara R, Uchiyama T, Arai K, Yamanishi T, Hattori T (2004) Lower urinary tract dysfunction in Machado–Joseph disease: a study of 11 clinical-urodynamic observations. J Neurol Sci. 218:67–72

    Article  PubMed  Google Scholar 

  59. Sakakibara R, Uchiyama T, Yamanishi T, Hattori T (2004) Urinary function in patients with corticobasal degeneration; comparison with normal subjects. Neurourol Urodyn 23:154–158

    Article  PubMed  Google Scholar 

  60. Sakakibara R, Uchiyama T, Yamanishi T, Shirai K, Hattori T (2008) Bladder and bowel dysfunction in Parkinson’s disease. J Neural Transm 115:443–460

    Article  PubMed  CAS  Google Scholar 

  61. Sakuta M, Nakanishi T, Toyokura Y (1978) Anal muscle electromyograms differ in amyotrophic lateral sclerosis and Shy-Drager syndrome. Neurology 28:1289–1293

    PubMed  CAS  Google Scholar 

  62. Sasaki M (2005) Role of Barrington’s nucleus in micturition. J Comp Neurol 493:21–26

    Article  PubMed  Google Scholar 

  63. Schwarz J, Kornhuber M, Bischoff C, Straube A. (1997) Electromyography of the external anal sphincter in patients with Parkinson’s disease and multiple system atrophy: frequency of abnormal spontaneous activity and polyphasic motor unit potentials. Muscle Nerve 20:1167–1172

    Article  PubMed  CAS  Google Scholar 

  64. Singer C, Weiner WJ, Sanchez-Ramos JR, Ackerman M (1989) Sexual dysfunction in men with Parkinson’s disease. J Neurol Rehab 3:199–204

    Google Scholar 

  65. Steers WD (2002) Pathophysiology of overactive and urge urinary incontinence. Rev Urol 4(Suppl 4):S7–S18

    PubMed  Google Scholar 

  66. Stocchi F, Carbone A, Inghilteri M, Monge A, Ruggieri S, Berardelli A, Manfredi M (1997) Urodynamic and neurophysiological evaluation in Parkinson’s disease and multiple system atrophy. J Neurol Neurosurg Psychiatry 62:507–511

    Article  PubMed  CAS  Google Scholar 

  67. Thor KB (2003) Serotonin and norepinephrine involvement in efferent pathways to the urethral rhabdosphincter: implications for treating stress urinary incontinence. Urology 62:3–9

    Article  PubMed  Google Scholar 

  68. Tison F, Arne P, Sourgen C, Chrysostome V, Yeklef F (2000) The value of external anal sphincter electromyography for the diagnosis of multiple system atrophy. Mov Disord 15:1148–1157

    Article  PubMed  CAS  Google Scholar 

  69. Tsujimura A, Miyagawa Y, Fujita K, Matsuoka Y, Takahashi T, Takao T, Matsumiya K, Osaki Y, Takasawa M, Oku N, Hatazawa J, Shigeo Kaneko S, Okuyama A (2006) Brain processing of audiovisual sexual stimuli inducing penile erection: a positron emission tomography study. J Urol 176:679–683

    Article  PubMed  Google Scholar 

  70. Valldeoriola F, Valls-Sole J, Tolosa E, Marti M (1995) Striated anal sphincter denervation in patients with progressive supranuclear palsy. Mov Disord 10:550–555

    Article  PubMed  CAS  Google Scholar 

  71. van Furth WR, Wolterink G, van Ree JM (1995) Regulation of masculine sexual behavior; involvement of brain opioids and dopamine. Brain Res Rev 21:162–184

    Article  PubMed  Google Scholar 

  72. Vaughan CJ, Aherne AM, Lane E, Power O, Carey RM, O’Connell DP (2000) Identification and regional distribution of the dopamine D1A receptor in the GI tract. Am J Physiol Regul Integr Comp Physiol 279:R599–R609

    PubMed  CAS  Google Scholar 

  73. Vodusek DB (2001) Sphincter EMG and differential diagnosis of multiple system atrophy. Mov Disord 16:600–607

    Article  PubMed  CAS  Google Scholar 

  74. Wakabayashi K, Takahashi H (1997) Neuropathology of autonomic nervous system in Parkinson’s disease. Eur Neurol 38(Suppl 2):2–7

    Article  PubMed  Google Scholar 

  75. Walker JK, Gainetdinov RR, Mangel AW, Caron MG, Shetzline MA (2000) Mice lacking the dopamine transporter display altered regulation of distal colonic motility. Am J Physiol Gastrointest Liver Physiol 279:G311–G318

    PubMed  CAS  Google Scholar 

  76. Wenning GK, Ben-Schlomo Y, Magalhaes M, Daniel S, Quinn N (1994) Clinical features and natural history of multiple system atrophy. Brain 117:835–845

    Article  PubMed  Google Scholar 

  77. Yaguchi H, Soma H, Miyazaki Y, Tashiro J, Yabe I, Kikuchi S, Sasaki H, Kakizaki H, Moriwaka F, Tashiro K (2004) A case of acute urinary retention caused by periaqueductal grey lesion. J Neurol Neurosurg Psychiatry 75:1202–1203

    Article  PubMed  CAS  Google Scholar 

  78. Yamamoto T, Sakakibara R, Uchiyama T, Liu Z, Ito T, Awa Y, Yamanishi T, Hattori T (2006) Neurological diseases that cause detrusor hyperactivity with impaired contractile function. Neurourol Urodynam 25:356–360

    Article  Google Scholar 

  79. Yamamoto T, Sakakibara R, Uchiyama T, Liu Z, Ito T, Awa Y, Yamanishi T, Hattori T (2005) When is Onuf’s nucleus involved in multiple system atrophy? A sphincter electromyography study. J Neurol Neurosurg Psychiatry 76:1645–1648

    Article  PubMed  CAS  Google Scholar 

  80. Yamanaka Y, Asahina M, Hiraga A, Sakakibara R, Oka H, Hattori T (2007) Over 10 years of isolated autonomic failure preceding dementia and Parkinsonism in two patients with Lewy body disease. Mov Disord 22:595–597

    Article  PubMed  Google Scholar 

  81. Yokoyama O, Yoshiyama M, Namiki M, de Groat WC (2002) Changes in dopaminergic and glutamatergic excitatory mechanisms of micturition reflex after middle cerebral artery occlusion in conscious rats. Exp Neurol 173:129–135

    Article  PubMed  CAS  Google Scholar 

  82. Yokoyama O, Yotsuyanagi S, Akino H, Moriyama H, Matsuta Y, Namiki M (2003) RNA synthesis in pons necessary for maintenance of bladder overactivity after cerebral infarction in rat. J Urol 169:1878–1884

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurology Division, Dept. of Internal Medicine, Sakura Medical Center, Toho University, 564-1 Shimoshizu, Sakura, 285-8741, Japan

    Ryuji Sakakibara MD, PhD & Masahiko Kishi

  2. Dept. of Neurology, Chiba University, Chiba, Japan

    Ryuji Sakakibara MD, PhD & Tomoyuki Uchiyama

  3. Dept. of Urology, Dokkyo Medical College, Tochigi, Japan

    Tomonori Yamanishi

Authors
  1. Ryuji Sakakibara MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Tomoyuki Uchiyama
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Tomonori Yamanishi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Masahiko Kishi
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ryuji Sakakibara MD, PhD.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sakakibara, R., Uchiyama, T., Yamanishi, T. et al. Sphincter EMG as a diagnostic tool in autonomic disorders. Clin Auton Res 19, 20–31 (2009). https://doi.org/10.1007/s10286-008-0489-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10286-008-0489-5

Keywords

Search

Navigation