Research Article| Volume 324, ISSUE 1-2, P53-56, January 15, 2013

Long-term treatment of Lewis–Sumner syndrome with subcutaneous immunoglobulin infusions

Published:October 24, 2012DOI:


      Lewis–Sumner syndrome (LSS, synonymous multifocal acquired demyelinating sensory and motor neuropathy, MADSAM) is a dysimmune peripheral neuropathy responding to corticosteroids and intravenous immunoglobulins (IVIG) in the majority of patients. We report on the long term treatment (37 and 46 months respectively) of two LSS patients, who had initially responded to IVIG, with subcutaneous immunoglobulins (SCIg). Both were switched to SCIg since stabilization by IVIG could only be achieved with short treatment intervals, and one of them also suffered from recurrent transient ischemic attacks (TIAs) following IVIG related increased blood viscosity. Long-term use of SCIg was safe and well tolerated. Both patients were clinically stable with only mild to moderate fluctuations requiring SCIg dosage adaptions. No further ischemic events occurred, when the patient was switched to SCIg.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Lewis R.A.
        • Sumner A.J.
        The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies.
        Neurology. 1982; 32: 592-596
        • Whitesell J.
        Inflammatory neuropathies. Semin Neurol. 2010; 30: 356-364
        • Verschueren A.
        • Azulay J.P.
        • Attarian S.
        • Boucraut J.
        • Pellissier J.F.
        • Pouget J.
        Lewis–Sumner syndrome and multifocal motor neuropathy.
        Muscle Nerve. 2005; 31: 88-94
        • Viala K.
        • Renie L.
        • Maisonobe T.
        • et al.
        Follow-up study and response to treatment in 23 patients with Lewis–Sumner syndrome.
        Brain. 2004; 127: 2010-2017
        • Rajabally Y.A.
        • Chavada G.
        Lewis–Sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.
        Muscle Nerve. 2009; 39: 206-220
        • Saperstein D.S.
        • Amato A.A.
        • Wolfe G.I.
        • et al.
        Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis–Sumner syndrome.
        Muscle Nerve. 1999; 22: 560-566
        • Van den Bergh P.Y.
        • Hadden R.D.
        • Bouche P.
        • et al.
        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
        • Pouget J.
        • Verschueren A.
        • Azulay J.P.
        • Attarian S.
        Lewis and Sumner syndrome.
        Rev Neurol (Paris). 2001; 157: 1561-1564
        • Caress J.B.
        • Cartwright M.S.
        • Donofrio P.D.
        • Peacock Jr., J.E.
        The clinical features of 16 cases of stroke associated with administration of IVIg.
        Neurology. 2003; 60: 1822-1824
        • Caress J.B.
        • Hobson-Webb L.
        • Passmore L.V.
        • Finkbiner A.P.
        • Cartwright M.S.
        Case–control study of thromboembolic events associated with IV immunoglobulin.
        J Neurol. 2009; 256: 339-342
        • Dalakas M.C.
        High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events.
        Neurology. 1994; 44: 223-226
        • Dalakas M.C.
        Intravenous immunoglobulin in autoimmune neuromuscular diseases.
        JAMA. 2004; 291: 2367-2375
        • Dalakas M.C.
        • Clark W.M.
        Strokes, thromboembolic events, and IVIg: rare incidents blemish an excellent safety record.
        Neurology. 2003; 60: 1736-1737
        • Dacci P.
        • Riva N.
        • Scarlato M.
        • et al.
        Subcutaneous immunoglobulin therapy for the treatment of multifocal motor neuropathy: a case report.
        Neurol Sci. 2010; 31: 829-831
        • Eftimov F.
        • Vermeulen M.
        • de Haan R.J.
        • van den Berg L.H.
        • van Schaik I.N.
        Subcutaneous immunoglobulin therapy for multifocal motor neuropathy.
        J Peripher Nerv Syst. 2009; 14: 93-100
        • Harbo T.
        • Andersen H.
        • Hess A.
        • Hansen K.
        • Sindrup S.H.
        • Jakobsen J.
        Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy: a randomized, single-blinded cross-over trial.
        Eur J Neurol. 2009; 16: 631-638
        • Harbo T.
        • Andersen H.
        • Jakobsen J.
        Long-term therapy with high doses of subcutaneous immunoglobulin in multifocal motor neuropathy.
        Neurology. 2010; 75: 1377-1380
        • Koller H.
        • Schroeter M.
        • Feischen H.
        • Hartung H.P.
        • Kieseier B.C.
        Subcutaneous self-infusions of immunoglobulins as a potential therapeutic regimen in immune-mediated neuropathies.
        J Neurol. 2006; 253: 1505-1506
        • Lee D.H.
        • Linker R.A.
        • Paulus W.
        • Schneider-Gold C.
        • Chan A.
        • Gold R.
        Subcutaneous immunoglobulin infusion: a new therapeutic option in chronic inflammatory demyelinating polyneuropathy.
        Muscle Nerve. 2008; 37: 406-409
        • Berger M.
        Subcutaneous immunoglobulin replacement in primary immunodeficiencies.
        Clin Immunol. 2004; 112: 1-7
        • Kleyweg R.P.
        • van der Meche F.G.
        • Schmitz P.I.
        Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain–Barre syndrome.
        Muscle Nerve. 1991; 14: 1103-1109
        • Merkies I.S.
        • Schmitz P.I.
        • van der Meche F.G.
        • Samijn J.P.
        • van Doorn P.A.
        Clinimetric evaluation of a new overall disability scale in immune mediated polyneuropathies.
        J Neurol Neurosurg Psychiatry. 2002; 72: 596-601
        • Misbah S.A.
        • Baumann A.
        • Fazio R.
        • et al.
        A smooth transition protocol for patients with multifocal motor neuropathy going from intravenous to subcutaneous immunoglobulin therapy: an open-label proof-of-concept study.
        J Peripher Nerv Syst. 2011; 16: 92-97
        • Okuda D.
        • Flaster M.
        • Frey J.
        • Sivakumar K.
        Arterial thrombosis induced by IVIg and its treatment with tPA.
        Neurology. 2003; 60: 1825-1826
        • 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
        • Bouchard C.
        • Lacroix C.
        • Plante V.
        • et al.
        Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy.
        Neurology. 1999; 52: 498-503