Skip to main content
Top
Published in: BMC Neurology 1/2014

Open Access 01-12-2014 | Research article

Deterioration after corticosteroids in CIDP may be associated with pure focal demyelination pattern

Authors: Filip Eftimov, Marinus H Liesdek, Camillus Verhamme, Ivo N van Schaik

Published in: BMC Neurology | Issue 1/2014

Login to get access

Abstract

Background

In the PREDICT study, a randomised controlled trial comparing dexamethasone with prednisolone in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), almost a quarter of patients deteriorated soon after starting treatment. The primary objective of this post-hoc analysis was to test the hypothesis that a focal demyelination pattern is associated with early deterioration after corticosteroid treatment and to explore whether various clinical characteristics are associated with deterioration after corticosteroid treatment.

Methods

Clinical outcome was categorised into early deterioration and non-early deterioration. A neurophysiologist blinded for treatment outcome scored electrophysiological data into following categories: pure focal versus non-focal distribution of demyelination and no/minor versus moderate/severe sensory involvement. Additionally, we compared electrophysiological and clinical baseline parameters, with emphasis on previously reported possible associations.

Results

Early deterioration was found in 7 out of 33 patients (21%). Ten patients had pure focal distribution of demyelination, of whom 5 had early deterioration; 23 patients had non-focal distribution, of whom 2 had early deterioration (p = 0.02). Higher mean median nerve sensory nerve conduction velocity (SNCV) was found in patients with early deterioration compared to patients with non-early deterioration (52.6 and respectively 40.8 m/s, p = 0.02).

Conclusion

Pure focal distribution of demyelination and lesser sensory electrophysiological abnormalities may be associated with early deterioration in CIDP patients treated with corticosteroids.
Appendix
Available only for authorised users
Literature
1.
go back to reference Hughes R, Bensa S, Willison H, Van den BP, Comi G, Illa I, Nobile-Orazio E, van Doorn PA, Dalakas M, Bojar M, Swan A: Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001, 50: 195-201. 10.1002/ana.1088.CrossRefPubMed Hughes R, Bensa S, Willison H, Van den BP, Comi G, Illa I, Nobile-Orazio E, van Doorn PA, Dalakas M, Bojar M, Swan A: Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001, 50: 195-201. 10.1002/ana.1088.CrossRefPubMed
2.
go back to reference van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, van Doorn PA, van Schaik IN: European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010, 17: 356-363. 10.1111/j.1468-1331.2009.02930.x.CrossRefPubMed van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, Koski CL, Leger JM, Nobile-Orazio E, Pollard J, Sommer C, van Doorn PA, van Schaik IN: European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010, 17: 356-363. 10.1111/j.1468-1331.2009.02930.x.CrossRefPubMed
3.
go back to reference van Schaik IN, Eftimov F, van Doorn PA, Brusse E, Van den Berg LH, van der Pol WL, Faber CG, van Oostrom JC, Vogels OJ, Hadden RD, Kleine BU, van Norden AG, Verschuuren JJ, Dijkgraaf MG, Vermeulen M: Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol. 2010, 9: 245-253. 10.1016/S1474-4422(10)70021-1.CrossRefPubMed van Schaik IN, Eftimov F, van Doorn PA, Brusse E, Van den Berg LH, van der Pol WL, Faber CG, van Oostrom JC, Vogels OJ, Hadden RD, Kleine BU, van Norden AG, Verschuuren JJ, Dijkgraaf MG, Vermeulen M: Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial. Lancet Neurol. 2010, 9: 245-253. 10.1016/S1474-4422(10)70021-1.CrossRefPubMed
4.
go back to reference Slee M, Selvan A, Donaghy M: Multifocal motor neuropathy: the diagnostic spectrum and response to treatment. Neurology. 2007, 69: 1680-1687. 10.1212/01.wnl.0000277697.55288.d0.CrossRefPubMed Slee M, Selvan A, Donaghy M: Multifocal motor neuropathy: the diagnostic spectrum and response to treatment. Neurology. 2007, 69: 1680-1687. 10.1212/01.wnl.0000277697.55288.d0.CrossRefPubMed
5.
go back to reference Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, Jacobs J: Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1994, 57: 778-783. 10.1136/jnnp.57.7.778.CrossRefPubMedPubMedCentral Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, Jacobs J: Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1994, 57: 778-783. 10.1136/jnnp.57.7.778.CrossRefPubMedPubMedCentral
6.
go back to reference Viala K, Renie L, Maisonobe T, Behin A, Neil J, Leger JM, Bouche P: Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004, 127: 2010-2017. 10.1093/brain/awh222.CrossRefPubMed Viala K, Renie L, Maisonobe T, Behin A, Neil J, Leger JM, Bouche P: Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004, 127: 2010-2017. 10.1093/brain/awh222.CrossRefPubMed
7.
go back to reference van Asseldonk JT, Van den Berg LH, Van Den Berg-Vos RM, Wieneke GH, Wokke JH, Franssen H: Demyelination and axonal loss in multifocal motor neuropathy: distribution and relation to weakness. Brain. 2003, 126: 186-198. 10.1093/brain/awg019.CrossRefPubMed van Asseldonk JT, Van den Berg LH, Van Den Berg-Vos RM, Wieneke GH, Wokke JH, Franssen H: Demyelination and axonal loss in multifocal motor neuropathy: distribution and relation to weakness. Brain. 2003, 126: 186-198. 10.1093/brain/awg019.CrossRefPubMed
8.
go back to reference Busby M, Donaghy M: Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. J Neurol. 2003, 250: 714-724. 10.1007/s00415-003-1068-2.CrossRefPubMed Busby M, Donaghy M: Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. J Neurol. 2003, 250: 714-724. 10.1007/s00415-003-1068-2.CrossRefPubMed
9.
go back to reference Kuwabara S, Misawa S, Mori M, Tamura N, Kubota M, Hattori T: Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases. J Neurol Neurosurg Psychiatry. 2006, 77: 66-70. 10.1136/jnnp.2005.065441.CrossRefPubMedPubMedCentral Kuwabara S, Misawa S, Mori M, Tamura N, Kubota M, Hattori T: Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases. J Neurol Neurosurg Psychiatry. 2006, 77: 66-70. 10.1136/jnnp.2005.065441.CrossRefPubMedPubMedCentral
10.
go back to reference Rajabally YA, Narasimhan M, Chavada G: Electrophysiological predictors of steroid-responsiveness in chronic inflammatory demyelinating polyneuropathy. J Neurol. 2008, 255: 936-938. 10.1007/s00415-008-0687-z.CrossRefPubMed Rajabally YA, Narasimhan M, Chavada G: Electrophysiological predictors of steroid-responsiveness in chronic inflammatory demyelinating polyneuropathy. J Neurol. 2008, 255: 936-938. 10.1007/s00415-008-0687-z.CrossRefPubMed
11.
go back to reference Sghirlanzoni A, Solari A, Ciano C, Mariotti C, Fallica E, Pareyson D: Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients. Neurol Sci. 2000, 21: 31-37. 10.1007/s100720070116.CrossRefPubMed Sghirlanzoni A, Solari A, Ciano C, Mariotti C, Fallica E, Pareyson D: Chronic inflammatory demyelinating polyradiculoneuropathy: long-term course and treatment of 60 patients. Neurol Sci. 2000, 21: 31-37. 10.1007/s100720070116.CrossRefPubMed
12.
go back to reference Franssen H, Vermeulen M, Jennekens FG: Diagnostic criteria for neuromuscular disorders. Chronic Inflammatory Neuropathies. Edited by: Emery AEH. 1997, London: Royal Society Medical Press Franssen H, Vermeulen M, Jennekens FG: Diagnostic criteria for neuromuscular disorders. Chronic Inflammatory Neuropathies. Edited by: Emery AEH. 1997, London: Royal Society Medical Press
13.
go back to reference Eftimov F, Vermeulen M, van Doorn PA, Brusse E, van Schaik IN: Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012, 78: 1079-1084. 10.1212/WNL.0b013e31824e8f84.CrossRefPubMed Eftimov F, Vermeulen M, van Doorn PA, Brusse E, van Schaik IN: Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012, 78: 1079-1084. 10.1212/WNL.0b013e31824e8f84.CrossRefPubMed
14.
go back to reference Nobile-Orazio E, Cocito D, Jann S, Uncini A, Beghi E, Messina P, Antonini G, Fazio R, Gallia F, Schenone A, Francia A, Pareyson D, Santoro L, Tamburin S, Macchia R, Cavaletti G, Giannini F, Sabatelli M: Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012, 11: 493-502. 10.1016/S1474-4422(12)70093-5.CrossRefPubMed Nobile-Orazio E, Cocito D, Jann S, Uncini A, Beghi E, Messina P, Antonini G, Fazio R, Gallia F, Schenone A, Francia A, Pareyson D, Santoro L, Tamburin S, Macchia R, Cavaletti G, Giannini F, Sabatelli M: Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropathy: a randomised controlled trial. Lancet Neurol. 2012, 11: 493-502. 10.1016/S1474-4422(12)70093-5.CrossRefPubMed
15.
go back to reference Burke D, Kiernan MC, Bostock H: Excitability of human axons. Clin Neurophysiol. 2001, 112: 1575-1585. 10.1016/S1388-2457(01)00595-8.CrossRefPubMed Burke D, Kiernan MC, Bostock H: Excitability of human axons. Clin Neurophysiol. 2001, 112: 1575-1585. 10.1016/S1388-2457(01)00595-8.CrossRefPubMed
16.
go back to reference Park SB, Lin CS, Burke D, Kiernan MC: Activity-dependent conduction failure: molecular insights. J Peripher Nerv Syst. 2011, 16: 159-168. 10.1111/j.1529-8027.2011.00358.x.CrossRefPubMed Park SB, Lin CS, Burke D, Kiernan MC: Activity-dependent conduction failure: molecular insights. J Peripher Nerv Syst. 2011, 16: 159-168. 10.1111/j.1529-8027.2011.00358.x.CrossRefPubMed
17.
go back to reference Nordsborg N, Goodmann C, McKenna MJ, Bangsbo J: Dexamethasone up-regulates skeletal muscle maximal Na+, K + pump activity by muscle group specific mechanisms in humans. J Physiol. 2005, 567: 583-589. 10.1113/jphysiol.2005.087031.CrossRefPubMedPubMedCentral Nordsborg N, Goodmann C, McKenna MJ, Bangsbo J: Dexamethasone up-regulates skeletal muscle maximal Na+, K + pump activity by muscle group specific mechanisms in humans. J Physiol. 2005, 567: 583-589. 10.1113/jphysiol.2005.087031.CrossRefPubMedPubMedCentral
18.
go back to reference Iijima M, Yamamoto M, Hirayama M, Tanaka F, Katsuno M, Mori K, Koike H, Hattori N, Arimura K, Nakagawa M, Yoshikawa H, Hayasaka K, Onodera O, Baba M, Yasuda H, Saito T, Nakazato M, Nakashima K, Kira J, Kaji R, Oka N, Sobue G: Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Neurology. 2005, 64: 1471-1475. 10.1212/01.WNL.0000158680.89323.F8.CrossRefPubMed Iijima M, Yamamoto M, Hirayama M, Tanaka F, Katsuno M, Mori K, Koike H, Hattori N, Arimura K, Nakagawa M, Yoshikawa H, Hayasaka K, Onodera O, Baba M, Yasuda H, Saito T, Nakazato M, Nakashima K, Kira J, Kaji R, Oka N, Sobue G: Clinical and electrophysiologic correlates of IVIg responsiveness in CIDP. Neurology. 2005, 64: 1471-1475. 10.1212/01.WNL.0000158680.89323.F8.CrossRefPubMed
Metadata
Title
Deterioration after corticosteroids in CIDP may be associated with pure focal demyelination pattern
Authors
Filip Eftimov
Marinus H Liesdek
Camillus Verhamme
Ivo N van Schaik
Publication date
01-12-2014
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2014
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/1471-2377-14-72

Other articles of this Issue 1/2014

BMC Neurology 1/2014 Go to the issue