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.


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Hughes RA, Cornblath DR (2005) Guillain–Barré syndrome. Lancet 366:1653–1666
van Doorn PA, Ruts L, Jacobs BC (2008) Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 7:939–950
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
Ropper AH (1986) Severe acute Guillain–Barré syndrome. Neurology 36:429–432
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
Rajabally YA, Uncini A (2012) Outcome and its predictors in Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 83:711–718
Miller RG, Peterson GW, Daube JR, Albers JW (1998) Prognostic value of electrodiagnosis in Guillain–Barré syndrome. Muscle Nerve 11:769–774
Koltzenburg M, Yousry T (2007) Magnetic resonance imaging of skeletal muscle. Curr Opin Neurol 20:595–599
Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM (2008) MRI appearance of muscle denervation. Skelet Radiol 37:397–404
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
Uetani M, Hayashi K, Matsunaga N, Imamura K, Ito N (1993) Denervated skeletal muscle: MR imaging work in progress. Radiology 189:511–515
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
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
Bendszus M, Koltsenburg M (2001) Visualization of denervated muscle by gadolinium-enhanced MRI. Neurology 57:1708–1711
Bendszus M, Koltzenburg M, Wessig C, Solymosi L (2002) Sequential MR imaging of denervated muscle: experimental study. AJNR Am J Neuroradiol 23:1427–1431
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
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
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
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
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
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
Lewis RA (2007) Neuropathies associated with conduction block. Curr Opin Neurol 20:525–530
Cornblath DR (1990) Electrophysiology in Guillain–Barré syndrome. Ann Neurol 27(suppl):S17–S20
Khanna S, Crues JV 3rd (2009) Complexities of MRI and false positive findings. Ann NY Acad Sci 1154:239–358
Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27(suppl):S21–S24
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
van Doorn PA, Jacobs BC (2006) Predicting the course of Guillain–Barré syndrome. Lancet Neurol 5:991–993
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
Albers JW, Donofrio PD, McGonagle TK (1985) Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 8:528–539
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
Ropper AH, Wijdicks EF, Shahani BT (1990) Electrodiagnostic abnormalities in 113 consecutive patients with Guillain–Barré syndrome. Arch Neurol 47:881–887
van der Meché FG, Meulstee J, Kleyweg RP (1991) Axonal damage in Guillain–Barré syndrome. Muscle Nerve 14:997–1002
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
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
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
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
Brown WF, Feasby TE (1984) Conduction block and denervation in Guillain–Barré polyneuropathy. Brain 107:219–239
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
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
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)
Davis DV, Coupland RE (1967) Gray’s anatomy. Described and applied (34th edn). Longmans, London, pp 1217–1243
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
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
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
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
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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.
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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
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DOI: https://doi.org/10.1007/s00415-013-6845-y

