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Published in: Journal of Neurology 6/2013

01-06-2013 | Original Communication

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

Authors: María J. Sedano, Ana Canga, Carmen de Pablos, José M. Polo, José Berciano

Published in: Journal of Neurology | Issue 6/2013

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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.
Literature
2.
go back to reference van Doorn PA, Ruts L, Jacobs BC (2008) Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 7:939–950PubMedCrossRef van Doorn PA, Ruts L, Jacobs BC (2008) Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 7:939–950PubMedCrossRef
3.
go back to reference 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 39:17–28PubMedCrossRef 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 39:17–28PubMedCrossRef
5.
go back to reference Sedano MJ, Calleja J, Canga E, Berciano J (1994) Guillain–Barré syndrome in Cantabria, Spain. An epidemiological and clinical study. Acta Neurol Scand 89:287–292PubMedCrossRef Sedano MJ, Calleja J, Canga E, Berciano J (1994) Guillain–Barré syndrome in Cantabria, Spain. An epidemiological and clinical study. Acta Neurol Scand 89:287–292PubMedCrossRef
6.
go back to reference Rajabally YA, Uncini A (2012) Outcome and its predictors in Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 83:711–718PubMedCrossRef Rajabally YA, Uncini A (2012) Outcome and its predictors in Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 83:711–718PubMedCrossRef
7.
go back to reference Miller RG, Peterson GW, Daube JR, Albers JW (1998) Prognostic value of electrodiagnosis in Guillain–Barré syndrome. Muscle Nerve 11:769–774CrossRef Miller RG, Peterson GW, Daube JR, Albers JW (1998) Prognostic value of electrodiagnosis in Guillain–Barré syndrome. Muscle Nerve 11:769–774CrossRef
8.
go back to reference Koltzenburg M, Yousry T (2007) Magnetic resonance imaging of skeletal muscle. Curr Opin Neurol 20:595–599PubMedCrossRef Koltzenburg M, Yousry T (2007) Magnetic resonance imaging of skeletal muscle. Curr Opin Neurol 20:595–599PubMedCrossRef
9.
go back to reference Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM (2008) MRI appearance of muscle denervation. Skelet Radiol 37:397–404CrossRef Kamath S, Venkatanarasimha N, Walsh MA, Hughes PM (2008) MRI appearance of muscle denervation. Skelet Radiol 37:397–404CrossRef
10.
go back to reference 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 45:659–667PubMedCrossRef 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 45:659–667PubMedCrossRef
11.
go back to reference Uetani M, Hayashi K, Matsunaga N, Imamura K, Ito N (1993) Denervated skeletal muscle: MR imaging work in progress. Radiology 189:511–515PubMed Uetani M, Hayashi K, Matsunaga N, Imamura K, Ito N (1993) Denervated skeletal muscle: MR imaging work in progress. Radiology 189:511–515PubMed
12.
go back to reference 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 187:213–218PubMed 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 187:213–218PubMed
13.
go back to reference 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 35:1077–1085PubMedCrossRef 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 35:1077–1085PubMedCrossRef
14.
go back to reference Bendszus M, Koltsenburg M (2001) Visualization of denervated muscle by gadolinium-enhanced MRI. Neurology 57:1708–1711CrossRef Bendszus M, Koltsenburg M (2001) Visualization of denervated muscle by gadolinium-enhanced MRI. Neurology 57:1708–1711CrossRef
15.
go back to reference Bendszus M, Koltzenburg M, Wessig C, Solymosi L (2002) Sequential MR imaging of denervated muscle: experimental study. AJNR Am J Neuroradiol 23:1427–1431PubMed Bendszus M, Koltzenburg M, Wessig C, Solymosi L (2002) Sequential MR imaging of denervated muscle: experimental study. AJNR Am J Neuroradiol 23:1427–1431PubMed
16.
go back to reference 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 24:1283–1289PubMed 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 24:1283–1289PubMed
17.
go back to reference 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 185:254–261PubMedCrossRef 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 185:254–261PubMedCrossRef
18.
go back to reference 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 37:1111–1117CrossRef 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 37:1111–1117CrossRef
19.
go back to reference 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 14:54–63PubMedCrossRef 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 14:54–63PubMedCrossRef
20.
go back to reference 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 259:1111–1116PubMedCrossRef 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 259:1111–1116PubMedCrossRef
21.
go back to reference 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 6:589–594PubMedCrossRef 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 6:589–594PubMedCrossRef
22.
23.
go back to reference Cornblath DR (1990) Electrophysiology in Guillain–Barré syndrome. Ann Neurol 27(suppl):S17–S20PubMedCrossRef Cornblath DR (1990) Electrophysiology in Guillain–Barré syndrome. Ann Neurol 27(suppl):S17–S20PubMedCrossRef
24.
go back to reference Khanna S, Crues JV 3rd (2009) Complexities of MRI and false positive findings. Ann NY Acad Sci 1154:239–358PubMedCrossRef Khanna S, Crues JV 3rd (2009) Complexities of MRI and false positive findings. Ann NY Acad Sci 1154:239–358PubMedCrossRef
25.
go back to reference Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27(suppl):S21–S24PubMedCrossRef Asbury AK, Cornblath DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27(suppl):S21–S24PubMedCrossRef
26.
go back to reference 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 119:2053–2061CrossRef 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 119:2053–2061CrossRef
27.
go back to reference van Doorn PA, Jacobs BC (2006) Predicting the course of Guillain–Barré syndrome. Lancet Neurol 5:991–993PubMedCrossRef van Doorn PA, Jacobs BC (2006) Predicting the course of Guillain–Barré syndrome. Lancet Neurol 5:991–993PubMedCrossRef
28.
go back to reference 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 16:136–142PubMedCrossRef 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 16:136–142PubMedCrossRef
29.
go back to reference Albers JW, Donofrio PD, McGonagle TK (1985) Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 8:528–539PubMedCrossRef Albers JW, Donofrio PD, McGonagle TK (1985) Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 8:528–539PubMedCrossRef
30.
go back to reference Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW (1986) An acute axonal form of Guillain–Barré polyneuropathy. Brain 109:1115–11126PubMedCrossRef Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW (1986) An acute axonal form of Guillain–Barré polyneuropathy. Brain 109:1115–11126PubMedCrossRef
31.
go back to reference Ropper AH, Wijdicks EF, Shahani BT (1990) Electrodiagnostic abnormalities in 113 consecutive patients with Guillain–Barré syndrome. Arch Neurol 47:881–887PubMedCrossRef Ropper AH, Wijdicks EF, Shahani BT (1990) Electrodiagnostic abnormalities in 113 consecutive patients with Guillain–Barré syndrome. Arch Neurol 47:881–887PubMedCrossRef
32.
go back to reference van der Meché FG, Meulstee J, Kleyweg RP (1991) Axonal damage in Guillain–Barré syndrome. Muscle Nerve 14:997–1002PubMedCrossRef van der Meché FG, Meulstee J, Kleyweg RP (1991) Axonal damage in Guillain–Barré syndrome. Muscle Nerve 14:997–1002PubMedCrossRef
33.
go back to reference 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 115:1291–1302PubMedCrossRef 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 115:1291–1302PubMedCrossRef
34.
go back to reference 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 55:1079–1083PubMedCrossRef 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 55:1079–1083PubMedCrossRef
35.
go back to reference 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 20:846–857PubMedCrossRef 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 20:846–857PubMedCrossRef
36.
go back to reference 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 51:394–398PubMedCrossRef 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 51:394–398PubMedCrossRef
37.
go back to reference Brown WF, Feasby TE (1984) Conduction block and denervation in Guillain–Barré polyneuropathy. Brain 107:219–239PubMedCrossRef Brown WF, Feasby TE (1984) Conduction block and denervation in Guillain–Barré polyneuropathy. Brain 107:219–239PubMedCrossRef
38.
go back to reference 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 44:780–788PubMedCrossRef 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 44:780–788PubMedCrossRef
39.
go back to reference Berciano J, García A, Figols J, Muñoz R, Berciano MT, Lafarga M (2000) Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy. Neurology 55:552–559PubMedCrossRef Berciano J, García A, Figols J, Muñoz R, Berciano MT, Lafarga M (2000) Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy. Neurology 55:552–559PubMedCrossRef
40.
go back to reference 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) 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.
go back to reference Davis DV, Coupland RE (1967) Gray’s anatomy. Described and applied (34th edn). Longmans, London, pp 1217–1243 Davis DV, Coupland RE (1967) Gray’s anatomy. Described and applied (34th edn). Longmans, London, pp 1217–1243
42.
go back to reference 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 129:426–437PubMedCrossRef 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 129:426–437PubMedCrossRef
43.
go back to reference McDonald CM, Carter GT, Fritz RC, Anderson MW, Abresch RT, Kilmer DD (2000) Magnetic resonance imaging of denervated muscle: comparison to electromyography. Muscle Nerve 23:1431–1434PubMedCrossRef McDonald CM, Carter GT, Fritz RC, Anderson MW, Abresch RT, Kilmer DD (2000) Magnetic resonance imaging of denervated muscle: comparison to electromyography. Muscle Nerve 23:1431–1434PubMedCrossRef
44.
go back to reference 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 264:522–530PubMedCrossRef 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 264:522–530PubMedCrossRef
45.
go back to reference 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 83:29–32PubMedCrossRef 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 83:29–32PubMedCrossRef
Metadata
Title
Muscle MRI in severe Guillain–Barré syndrome with motor nerve inexcitability
Authors
María J. Sedano
Ana Canga
Carmen de Pablos
José M. Polo
José Berciano
Publication date
01-06-2013
Publisher
Springer-Verlag
Published in
Journal of Neurology / Issue 6/2013
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-013-6845-y

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