Objectives: There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes.
Methods: We performed a retrospective chart review of MGUSN (n = 56) and CIDP (n = 67) patients. Data extracted included: demographics, neurological examination, and nerve conduction studies (NCS) at baseline and last visit. Clinical status was rated as 0 = worse, 1 = unchanged, 2 = stabilized after a declining course, or 3 = improved. The electrophysiology data were rated as 0 = worse, 1 = stable, or 2 = improved. Statistical analyses were performed using JMP (version 9.0.2 for Macintosh, from SAS).
Results: Seventy percent were males, aged 68.1 ± 12.6 years with neuropathy for 9.8 ± 6.8 years and follow-up of 4.0 ± 3.2 years. CIDP patients had more severe neuropathy, and were more likely to receive treatment and to respond. The clinical neuropathy status remained unchanged in 52.8 % of the MGUSN and 24.2 % of the CIDP patients, and stabilized in 7.6 % of MGUSN and 30.3 % of CIDP patients. IgM-MGUSN patients did not differ from other immunoglobulin subclasses in response to treatment. The clinical severity and the number of abnormal NCS parameters were greater in the demyelinating MGUSN in comparison to the axonal group.
Conclusion: MGUSN patients have less severe neuropathy than CIDP patients, but among the MGUSN patients the severity is greater in the demyelinating and the IgM groups. MGUSN patients may do well without treatment and exposure to potential adverse effects.
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http://dx.doi.org/10.1007/s00415-014-7357-0 | DOI Listing |
Hum Immunol
December 2024
From the Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, China. Electronic address:
We aim to investigate the proportion and function of regulatory T (Treg) cells, as well as mTORC activity in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. Peripheral blood mononuclear cells (PBMCs) from 15 CIDP and healthy controls (HC) were collected. Treg and responsive T (Tresp) cells were isolated.
View Article and Find Full Text PDFNeurol Int
December 2024
Department of Neurology, University of Texas Medical Branch, Galveston, TX 77555, USA.
Background: Hereditary Sensory Motor Neuropathy (HSMN) 1A and Multiple Sclerosis (MS) are distinct demyelinating disorders affecting the peripheral and central nervous systems, respectively. We present a case of simultaneous occurrence of both conditions, exploring the clinical presentation, diagnostic workup, and potential interplay between these diseases. Case presentation and clinical approach: A 49-year-old male with a history of optic neuritis presented with progressive numbness, weakness, and sensory loss in all extremities over four years.
View Article and Find Full Text PDFFront Neurol
December 2024
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Autoimmune nodopathy (AN) is a rare immune-mediated neuropathy characterized by autoantibodies against nodal or paranodal proteins. Patients with AN generally respond poorly to immunoglobulin therapy, and as a newly defined condition, there are currently no established treatment guidelines. Although rituximab shows potential as a therapeutic option, its high cost, limited availability, and the need for infusion monitoring hinder its use as a first-line treatment in many countries.
View Article and Find Full Text PDFBrain Nerve
January 2025
Department of Neurotherapeutics, Yamaguchi University School of Medicine.
The effectiveness of chronic inflammatory demyelinating polyneuropathy (CIDP) treatment is difficult to evaluate based on disease characteristics and treatment methods. The first basic concept of CIDP treatment is "to prevent undertreatment due to inadequate treatment and not overlook patients who can be saved." The second concept is "to prevent overtreatment by unnecessary treatment and discontinue excessive therapy for patients.
View Article and Find Full Text PDFBrain Nerve
January 2025
Department of Neurology, Dokkyo Medical University.
Three main treatments are available for management of chronic inflammatory demyelinating polyneuropathy (CIDP). Both induction and maintenance therapies should be considered for treatment of CIDP. Plasma exchange and intravenous immunoglobulin therapy are effective as induction treatments for CIDP, and corticosteroid administration, such as daily oral or pulse therapies, may be effective for both induction and maintenance.
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