Abstract
We reviewed the clinical, electrophysiological an laboratory findings, plus the therapeutics and evolution of patients with motor-dominant Chronic inflammatory demyelinating polyneuropathy (CIDP) and compared them with those of other CIDP patients. Among 12 consecutive CIDP patients, we identified five patients with motor-dominant CIDP. The five patients with motor-dominant CIDP initially presented with weakness of the upper limbs. Cervical magnetic resonance imaging (MRI) examinations of the patients with motor-dominant CIDP showed that the most affected lesions are the cervical nerve roots and brachial plexus. The clinical course of these patients was relapsing-remitting, and they improved markedly after treatment by intravenous immunoglobulin (IVIg) infusion or plasmapheresis. However, they did not improve in response to corticosteroid therapy during the acute phase of relapses. The relapses frequently occurred within 2 years, but rarely occurred after that. The score in the modified Rankin disability scale (mRDS) at the last follow-up period was statistically lower for the patients with motor-dominant CIDP than for the other CIDP patients (P < 0.002). The characteristic clinical features, responsiveness to treatment, and prognosis suggest that motor-dominant CIDP is a distinct subtype of CIDP, with a specific immunological background. Repeated IVIg therapy is required to maintain the motor functions of patients with motor-dominant CIDP. We consider that treatment for recurrence prevention as an alternative to IVIg therapy is very important for patients with motor-dominant CIDP.
References
Sander HW, Latov N (2003) Research criteria for defining patients with CIDP. Neurology 60:8–15
Sabatelli M, Media F, Mignogna T, Lippi G, Quaranta L, Tonali P (2001) Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol 248:772–777
Busby M, Donaghy M (2003) Chronic dysimmune neuropathy: A subclassification based upon the clinical features of 102 patients. J Neurol 250:714–724
Misra UK, Kalita J, Yadav RK (2007) A comparison of clinical atypical with typical chronic inflammatory demyelinating polyradiculoneuropathy. Eur Neurol 58:100–105
Joint Task Force of the EFNS and the PNS (2005) European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of paraproteinemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. J Peripher Nerv Syst 10:220–228
van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J (1988) Interobserver agreement for the assessment of handicap in stroke patients. Stroke 19:604–607
Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K, Mitsuma T, Sobue G (1999) Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. Brain 122:427–439
Hattori N, Misu K, Koike H, Ichimura M, Nagamatsu M, Hirayama M, Sobue G (2001) Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 184:57–63
Kimura A, Yoshino H, Yuasa T (2005) Chronic inflammatory demyelinating polyneuropathy in a patient with hyperIgEaemia. J Neurol Sci 231:89–93
Nobile-Orazio E (1996) Multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 60:599–603
Federico P, Zochodne DW, Hahn AF, Brown WF, Feasby TE (2000) Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study. Neurology 55:1256–1262
Olney RK, Lewis RA, Putnam TD, Campellone JV Jr, American Association of Electrodiagnostic Medicine (2003) Consensus criteria for the diagnosis of multifocal motor neuropathy. Mus Nerv 27:117–121
Kazatchkine MD, Kaveri SV (2001) Immunomodulation of autoimmune and inflammatory diseases with intravenous immune globulin. N Engl J Med 345:747–755
Dalakas MC (2004) Intravenous immunoglobulin in autoimmune neuromuscular diseases. JAMA 291:2367–2375
Bystryn JC, Jiao D (2006) IVIg selectively and rapidly decreases circulating pathogenic autoantibodies in pemphigus vulgaris. Autoimmunity 39:601–607
Acknowledgments
The authors thank Dr. Y. Oki and Dr. G. Sobue for the pathological examination of sural nerve biopsy results. This research was partially supported by a Japanese Health and Labor Sciences Research Grant for Research on Psychiatry and Neurological Diseases and Mental Health (H21-022), and a Grant-in-Aid for Scientific Research (C) (1690486) from the Japan Society for the Promotion of Science (JSPS).
Conflict of interest statement
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kimura, A., Sakurai, T., Koumura, A. et al. Motor-dominant chronic inflammatory demyelinating polyneuropathy. J Neurol 257, 621–629 (2010). https://doi.org/10.1007/s00415-009-5386-x
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00415-009-5386-x