Abstract
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.
Keywords
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References
- The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies.Neurology. 1982; 32: 592-596
- Inflammatory neuropathies. Semin Neurol. 2010; 30: 356-364
- Lewis–Sumner syndrome and multifocal motor neuropathy.Muscle Nerve. 2005; 31: 88-94
- Follow-up study and response to treatment in 23 patients with Lewis–Sumner syndrome.Brain. 2004; 127: 2010-2017
- Lewis–Sumner syndrome of pure upper-limb onset: diagnostic, prognostic, and therapeutic features.Muscle Nerve. 2009; 39: 206-220
- Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis–Sumner syndrome.Muscle Nerve. 1999; 22: 560-566
- 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
- Lewis and Sumner syndrome.Rev Neurol (Paris). 2001; 157: 1561-1564
- The clinical features of 16 cases of stroke associated with administration of IVIg.Neurology. 2003; 60: 1822-1824
- Case–control study of thromboembolic events associated with IV immunoglobulin.J Neurol. 2009; 256: 339-342
- High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events.Neurology. 1994; 44: 223-226
- Intravenous immunoglobulin in autoimmune neuromuscular diseases.JAMA. 2004; 291: 2367-2375
- Strokes, thromboembolic events, and IVIg: rare incidents blemish an excellent safety record.Neurology. 2003; 60: 1736-1737
- Subcutaneous immunoglobulin therapy for the treatment of multifocal motor neuropathy: a case report.Neurol Sci. 2010; 31: 829-831
- Subcutaneous immunoglobulin therapy for multifocal motor neuropathy.J Peripher Nerv Syst. 2009; 14: 93-100
- Subcutaneous versus intravenous immunoglobulin in multifocal motor neuropathy: a randomized, single-blinded cross-over trial.Eur J Neurol. 2009; 16: 631-638
- Long-term therapy with high doses of subcutaneous immunoglobulin in multifocal motor neuropathy.Neurology. 2010; 75: 1377-1380
- Subcutaneous self-infusions of immunoglobulins as a potential therapeutic regimen in immune-mediated neuropathies.J Neurol. 2006; 253: 1505-1506
- Subcutaneous immunoglobulin infusion: a new therapeutic option in chronic inflammatory demyelinating polyneuropathy.Muscle Nerve. 2008; 37: 406-409
- Subcutaneous immunoglobulin replacement in primary immunodeficiencies.Clin Immunol. 2004; 112: 1-7
- Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain–Barre syndrome.Muscle Nerve. 1991; 14: 1103-1109
- Clinimetric evaluation of a new overall disability scale in immune mediated polyneuropathies.J Neurol Neurosurg Psychiatry. 2002; 72: 596-601
- 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
- Arterial thrombosis induced by IVIg and its treatment with tPA.Neurology. 2003; 60: 1825-1826
- Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases.J Neurol Neurosurg Psychiatry. 2006; 77: 66-70
- Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy.Neurology. 1999; 52: 498-503
Article info
Publication history
Published online: October 24, 2012
Accepted:
September 28,
2012
Received in revised form:
September 20,
2012
Received:
October 24,
2011
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.