Objective: To compare motor unit number index (MUNIX) values in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and healthy controls, to assess correlations between MUNIX and clinical assessments used in CIDP and to assess short-term changes in MUNIX in CIDP following intravenous immunoglobulins (IVIg).
Methods: MUNIX sum scores were calculated from three muscles in patients and healthy controls. CIDP patients also underwent a series of clinical assessments and completed the Overall Neuropathy Limitations Scale (ONLS) and the Rasch-built Overall-Disability Scale (R-ODS). Repeat assessments were performed in CIDP patients receiving IVIg and CIDP patients not on active treatment.
Results: MUNIX sum scores were significantly lower in CIDP patients than healthy controls (mean values 214.0 vs 516.9, respectively; p < 0.001). MUNIX sum scores correlated with clinical assessment of motor and sensory function and ONLS and R-ODS scores in CIDP patients. Significant short-term improvements were seen in MUNIX values on repeat testing following IVIg (188.3-226.4, p = 0.001), but not in CIDP patients not receiving IVIg.
Conclusions: MUNIX values show stronger correlation with commonly-used clinical assessments and disability scores than other routinely used electrophysiological parameters. Rapid improvements in MUNIX sum scores are seen following IVIg.
Significance: MUNIX sum score may provide an objective marker of response to IVIg.
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http://dx.doi.org/10.1016/j.clinph.2019.06.231 | 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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