Skip to main content
Log in

Muscle MRI in severe Guillain–Barré syndrome with motor nerve inexcitability

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

We report on the clinical, electrophysiological, and lower-limb musculature MRI findings in a severe demyelinating Guillain–Barré syndrome (GBS) patient with follow-up over 6 months. After 3 weeks of tetraplegia and mechanical ventilation, there was progressive improvement until almost complete recovery. On day 4 after onset, electrophysiological study revealed absent F waves and widespread conduction block. On four further electrophysiological studies on days 12, 19, 45, and 150, there was marked and reversible slow down of motor conduction velocities in upper-limb nerves, and persistent inexcitability of lower-limb nerves. Mild signs of active denervation were recorded in calf and foot muscles as of day 45. On day 39, MRI T2-weighted fat-suppressed images showed patchy hypersignal of variable intensity involving pelvic, thigh, and calf muscles, which disappeared in a second imaging study on day 190; in this study T1-weighted images did not disclose muscle fatty atrophy. We conclude that in severe demyelinating GBS prolonged motor nerve inexcitability should not necessarily be taken as a predictor of poor prognosis, and that MRI is useful in assessing the topography and evolution of muscle denervation.

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

Access this article

Subscribe and save

Springer+
from $39.99 /Month
  • Starting from 10 chapters or articles per month
  • Access and download chapters and articles from more than 300k books and 2,500 journals
  • Cancel anytime
View plans

Buy Now

Price includes VAT (Canada)

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Hughes RA, Cornblath DR (2005) Guillain–Barré syndrome. Lancet 366:1653–1666

    Article  PubMed  CAS  Google Scholar 

  2. van Doorn PA, Ruts L, Jacobs BC (2008) Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 7:939–950

    Article  PubMed  Google Scholar 

  3. Griffin JW, Li CY, Ho TW, Tian M, Gao CY, Xue P, Mishu B, Cornblath DR, Macko C, McKhann GM, Asbury AK (1996) Pathology of the motor-sensory axonal Guillain–Barré syndrome. Ann Neurol 39:17–28

    Article  PubMed  CAS  Google Scholar 

  4. Ropper AH (1986) Severe acute Guillain–Barré syndrome. Neurology 36:429–432

    Article  PubMed  CAS  Google Scholar 

  5. Sedano MJ, Calleja J, Canga E, Berciano J (1994) Guillain–Barré syndrome in Cantabria, Spain. An epidemiological and clinical study. Acta Neurol Scand 89:287–292

    Article  PubMed  CAS  Google Scholar 

  6. Rajabally YA, Uncini A (2012) Outcome and its predictors in Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 83:711–718

    Article  PubMed  Google Scholar 

  7. Miller RG, Peterson GW, Daube JR, Albers JW (1998) Prognostic value of electrodiagnosis in Guillain–Barré syndrome. Muscle Nerve 11:769–774

    Article  Google Scholar 

  8. Koltzenburg M, Yousry T (2007) Magnetic resonance imaging of skeletal muscle. Curr Opin Neurol 20:595–599

    Article  PubMed  Google Scholar 

  9. Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM (2008) MRI appearance of muscle denervation. Skelet Radiol 37:397–404

    Article  CAS  Google Scholar 

  10. Deroide N, Bousson V, Daguet E, Dumurgier J, Tin SN, Hannouche D, Richette P, Beaudreuil J, Lioté F, Lévy B, Vicaut E, Laredo JD, Kubis N (2012) Muscle magnetic resonance imaging sensitivity does not decrease in chronic, mild, or proximal lower limb neuropathies. Muscle Nerve 45:659–667

    Article  PubMed  Google Scholar 

  11. Uetani M, Hayashi K, Matsunaga N, Imamura K, Ito N (1993) Denervated skeletal muscle: MR imaging work in progress. Radiology 189:511–515

    PubMed  CAS  Google Scholar 

  12. Fleckenstein JL, Watumull D, Conner KE, Ezaki M, Greenlee RG Jr, Bryan WW, Chason DP, Parkey RW, Peshock RM, Purdy PD (1993) Denervated human skeletal muscle: MR imaging evaluation. Radiology 187:213–218

    PubMed  CAS  Google Scholar 

  13. West GA, Haynor DR, Goodkin R, Tsuruda JS, Bronstein AD, Kraft G, Winter T, Kliot M (1994) Magnetic resonance imaging signal changes in denervated muscles after peripheral nerve injury. Neurosurgery 35:1077–1085

    Article  PubMed  CAS  Google Scholar 

  14. Bendszus M, Koltsenburg M (2001) Visualization of denervated muscle by gadolinium-enhanced MRI. Neurology 57:1708–1711

    Article  Google Scholar 

  15. Bendszus M, Koltzenburg M, Wessig C, Solymosi L (2002) Sequential MR imaging of denervated muscle: experimental study. AJNR Am J Neuroradiol 23:1427–1431

    PubMed  Google Scholar 

  16. Bendszus M, Wessig C, Reiners K, Bartsch AJ, Solymosi L, Koltzenberg M (2003) MR imaging in the differential diagnosis of neurogenic foot drop. AJNR Am J Neuroradiol 24:1283–1289

    PubMed  Google Scholar 

  17. Wessig C, Koltzenburg M, Reiners K, Solymosi L, Bendszus M (2004) Muscle magnetic resonance imaging of denervation and reinnervation: correlation with electrophysiology and histology. Exp Neurol 185:254–261

    Article  PubMed  Google Scholar 

  18. Holl N, Echaniz-Laguna A, Bierry G, Mohr M, Loeffler JP, Moser T, Dietemann JL, Kremer S (2008) Diffusion-weighted MRI of denervated muscle: a clinical and experimental study. Skelet Radiol 37:1111–1117

    Article  Google Scholar 

  19. Berciano J, García A, Villagrá NT, González F, Ramón C, Illa I, Berciano MT, Lafarga M (2009) Severe Guillain–Barré syndrome: sorting out the pathological hallmark in an electrophysiological axonal case. J Peripher Nerv Syst 14:54–63

    Article  PubMed  Google Scholar 

  20. Berciano J, Gallardo E, Fernández-Torre JL, González-Quintanilla V, Infante J (2012) Magnetic resonance imaging of lower limb musculature in acute motor axonal neuropathy. J Neurol 259:1111–1116

    Article  PubMed  Google Scholar 

  21. van Koningsveld R, Steyerberg EW, Hughes RA, Swan AV, van Doorn PA, Jacobs BC (2007) A clinical prognostic scoring system for Guillain–Barré syndrome. Lancet Neurol 6:589–594

    Article  PubMed  Google Scholar 

  22. Lewis RA (2007) Neuropathies associated with conduction block. Curr Opin Neurol 20:525–530

    Article  PubMed  Google Scholar 

  23. Cornblath DR (1990) Electrophysiology in Guillain–Barré syndrome. Ann Neurol 27(suppl):S17–S20

    Article  PubMed  Google Scholar 

  24. Khanna S, Crues JV 3rd (2009) Complexities of MRI and false positive findings. Ann NY Acad Sci 1154:239–358

    Article  PubMed  Google Scholar 

  25. Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27(suppl):S21–S24

    Article  PubMed  Google Scholar 

  26. The Italian Guillain–Barré Study Group (1996) The prognosis and main prognostic indicators of Guillain–Barré syndrome. A multicentre prospective study of 297 patients. Brain 119:2053–2061

    Article  Google Scholar 

  27. van Doorn PA, Jacobs BC (2006) Predicting the course of Guillain–Barré syndrome. Lancet Neurol 5:991–993

    Article  PubMed  Google Scholar 

  28. Albertí MA, Alentorn A, Martínez-Yelamos S, Martínez-Matos JA, Povedano M, Montero J, Casasnovas C (2011) Very early electrodiagnostic findings in Guillain–Barré syndrome. J Peripher Nerv Syst 16:136–142

    Article  PubMed  Google Scholar 

  29. Albers JW, Donofrio PD, McGonagle TK (1985) Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 8:528–539

    Article  PubMed  CAS  Google Scholar 

  30. Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW (1986) An acute axonal form of Guillain–Barré polyneuropathy. Brain 109:1115–11126

    Article  PubMed  Google Scholar 

  31. Ropper AH, Wijdicks EF, Shahani BT (1990) Electrodiagnostic abnormalities in 113 consecutive patients with Guillain–Barré syndrome. Arch Neurol 47:881–887

    Article  PubMed  CAS  Google Scholar 

  32. van der Meché FG, Meulstee J, Kleyweg RP (1991) Axonal damage in Guillain–Barré syndrome. Muscle Nerve 14:997–1002

    Article  PubMed  Google Scholar 

  33. Triggs WJ, Cros D, Gominak SC, Zuniga G, Beric A, Shahani BT, Ropper AH, Roongta SM (1992) Motor nerve inexcitability in Guillain–Barré syndrome. The spectrum of distal conduction block and axonal degeneration. Brain 115:1291–1302

    Article  PubMed  Google Scholar 

  34. Fuller GN, Jacobs JM, Lewis PD, Lane RJ (1992) Pseudoaxonal Guillain–Barré syndrome: severe demyelination mimicking axonopathy. A case with pupillary involvement. J Neurol Neurosurg Psychiatry 55:1079–1083

    Article  PubMed  CAS  Google Scholar 

  35. Berciano J, Figols J, García A, Calle E, Illa I, Lafarga M, Berciano MT (1997) Fulminant Guillain–Barré syndrome with universal inexcitability of peripheral nerves: a clinicopathological study. Muscle Nerve 20:846–857

    Article  PubMed  CAS  Google Scholar 

  36. Massaro ME, Rodriguez EC, Pociecha J, Arroyo HA, Sacolitti M, Taratuto AL, Fejerman N, Reisin RC (1998) Nerve biopsy in children with severe Guillain–Barré syndrome and inexcitable motor nerves. Neurology 51:394–398

    Article  PubMed  CAS  Google Scholar 

  37. Brown WF, Feasby TE (1984) Conduction block and denervation in Guillain–Barré polyneuropathy. Brain 107:219–239

    Article  PubMed  Google Scholar 

  38. Hadden RD, Cornblath DR, Hughes RA, Zielasek J, Hartung HP, Toyka KV (1998) Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain–Barré Syndrome Trial Group. Ann Neurol 44:780–788

    Article  PubMed  CAS  Google Scholar 

  39. Berciano J, García A, Figols J, Muñoz R, Berciano MT, Lafarga M (2000) Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy. Neurology 55:552–559

    Article  PubMed  CAS  Google Scholar 

  40. Berciano J, García A, Berciano MT, Lafarga M (2012) Criteria for Guillain–Barré syndrome: additional insights from clinico-pathological studies. Clin Neurophysiol. doi:10.1016/j.clinph.2012.06.022. (Epub ahead of print)

  41. Davis DV, Coupland RE (1967) Gray’s anatomy. Described and applied (34th edn). Longmans, London, pp 1217–1243

  42. Gallardo E, García A, Combarros O, Berciano J (2006) Charcot-Marie-Tooth disease type 1A duplication: spectrum of clinical and magnetic resonance imaging features in leg and foot muscles. Brain 129:426–437

    Article  PubMed  Google Scholar 

  43. McDonald CM, Carter GT, Fritz RC, Anderson MW, Abresch RT, Kilmer DD (2000) Magnetic resonance imaging of denervated muscle: comparison to electromyography. Muscle Nerve 23:1431–1434

    Article  PubMed  CAS  Google Scholar 

  44. Viddeleer AR, Sijens PE, van Ooyen PM, Kuypers PD, Hovius SE, Oudkerk M (2012) Sequential MR Imaging of denervated and reinnervated skeletal muscle as correlated to functional outcome. Radiology 264:522–530

    Article  PubMed  Google Scholar 

  45. Sinclair CD, Morrow JM, Miranda MA, Davagnanam I, Cowley PC, Mehta H, Hanna MG, Koltzenburg M, Yousry TA, Reilly MM, Thornton JS (2012) Skeletal muscle MRI magnetisation transfer ratio reflects clinical severity in peripheral neuropathies. J Neurol Neurosurg Psychiatry 83:29–32

    Article  PubMed  CAS  Google Scholar 

Download references

Conflicts of interest

The authors declare no competing interests.

Ethical standard

This study has been approved by the appropriate ethics committee and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to José Berciano.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sedano, M.J., Canga, A., de Pablos, C. et al. Muscle MRI in severe Guillain–Barré syndrome with motor nerve inexcitability. J Neurol 260, 1624–1630 (2013). https://doi.org/10.1007/s00415-013-6845-y

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue date:

  • DOI: https://doi.org/10.1007/s00415-013-6845-y

Keywords

Morty Proxy This is a proxified and sanitized view of the page, visit original site.