Polyneuropathy and monoclonal gammopathy of undetermined significance (MGUS); update of a clinical experience

Matà, Sabrina; Torricelli, Sara; Barilaro, Alessandro; Grippo, Antonello; Forleo, Paolo; Mastio, Monica Del; Sorbi, Sandro

doi:10.1016/j.jns.2021.117335

Cited by 5 publications

(4 citation statements)

References 28 publications

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“…Several previous studies have shown that patients with PDN associated with anti‐MAG are not a homogenous group [ 17 , 18 , 19 ]. The differing clinical, antibody and neurophysiological characteristics of these patients are probable reasons for the variability in response to therapies and explain the discordant results of RTX reported for this disease.…”

Section: Discussionmentioning

confidence: 99%

“…PDN patients may be heterogeneous with regards to clinical appearance, anti-MAG level, EDX pattern [15], and response to therapy (poor response to intravenous immunoglobulin or corticosteroids for patients showing a typical anti-MAG pattern on EDX study) [16]. Furthermore, in some patients with PDN and a typical anti-MAG pattern, anti-MAG are undetectable, are detected at very low level or are not pathogenic [6,17,18]. This neuropathy may become very disabling, mainly as a result of long disease duration or when axonal damage and motor impairment occur [19], and the response to immunotherapies remains suboptimal.…”

Section: Introductionmentioning

confidence: 99%

“…PDN patients may be heterogeneous with regards to clinical appearance, anti‐MAG level, EDX pattern [ 15 ], and response to therapy (poor response to intravenous immunoglobulin or corticosteroids for patients showing a typical anti‐MAG pattern on EDX study) [ 16 ]. Furthermore, in some patients with PDN and a typical anti‐MAG pattern, anti‐MAG are undetectable, are detected at very low level or are not pathogenic [ 6 , 17 , 18 ].…”

Section: Introductionmentioning

confidence: 99%

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Efficacy of rituximab in anti‐myelin‐associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow‐up

Parisi

Dogliotti

Clerico

et al. 2022

Euro J of Neurology

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Background and purpose We evaluated the clinical and neurophysiological efficacy of rituximab (RTX) in a neurophysiologically homogeneous group of patients with monoclonal gammopathy and immunoglobulin M (IgM) anti‐myelin‐associated glycoprotein antibody (anti‐MAG) demyelinating polyneuropathy. Methods Twenty three anti‐MAG‐positive polyneuropathic patients were prospectively evaluated before and for 2 years after treatment with RTX 375 mg/m2. The Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale (INCAT‐ds), modified INCAT sensory score (mISS), Medical Research Council sum score, Patients’ Global Impression of Change scale were used, IgM levels were assessed and extensive electrophysiological examinations were performed before (T0) and 1 year (T1) and 2 years (T2) after RTX treatment. Results At T1 and T2 there was a significant reduction from T0 both in mISS and in INCAT‐ds, with a p value < 0.001 in the inferential Friedman's test overall analysis. Ulnar nerve Terminal Latency Index and distal motor latency significantly changed from T0 to T1 and in the overall analysis (p = 0.001 and p = 0.002), and ulnar nerve sensory nerve action potential (SNAP) amplitude was significantly increased at T2 from T1, with a p value < 0.001 in the overall analysis. Analysis of the receiver‐operating characteristic curves showed that a 41.8% increase in SNAP amplitude in the ulnar nerve at T2 from T0 was a fair predictor of a mISS reduction of ≥2 points (area under the curve 0.85; p = 0.005; sensitivity: 90.9%, specificity: 83.3%). Conclusions This study suggests that RTX is effective in patients with clinically active demyelinating anti‐MAG neuropathy over 2 years of follow‐up, and that some neurophysiological variables might be useful for monitoring this efficacy.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Efficacy of rituximab in anti‐myelin‐associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow‐up

Parisi

Dogliotti

Clerico

et al. 2022

Euro J of Neurology

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“…Polyneuropathies in MGUS patients encompass a heterogeneous group of nerve disorders lacking a clear classification, whereas anti-MAG neuropathy has the most homogeneous clinical and neurophysiological presentations. IgM paraproteins can bind to MAG in peripheral nerves, resulting in myelin uncompaction and consequent demyelinating polyneuropathy [1,2].…”

Section: Introductionmentioning

confidence: 99%

Prevalence and clinical profiles of anti‐myelin‐associated glycoprotein neuropathy in Japan: A nationwide survey study of 133 patients

Aotsuka,

Misawa,

Suichi

et al. 2024

Euro J of Neurology

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Background and purposeThe aim of this study was to determine the prevalence of anti‐myelin‐associated glycoprotein (MAG) neuropathy and the current status of such patients in Japan.MethodsWe conducted a nationwide survey in 2021 using established epidemiological methods. Questionnaires were sent to all neurology and pediatric neurology departments throughout Japan to identify patients with anti‐MAG neuropathy. An initial questionnaire was used to determine the number of patients, with a second one used to collect detailed clinical information.ResultsThe estimated number of patients with anti‐MAG neuropathy was 353, with a prevalence of 0.28 per 100,000 and an incidence of 0.05 per 100,000. The detailed clinical profiles of 133 patients were available. The median (range) age of onset was 67 (30–87) years, with a prominent peak in the age range 66–70 years, and the male‐to‐female ratio was 3.6. Most patients had distal sensory‐predominant polyneuropathy, and neuropathic pain (50%), or sensory ataxia (42%), while 18% had Waldenström's macroglobulinemia or multiple myeloma. Intravenous immunoglobulin was the most frequently used treatment (65%), but the response rate was <50%, whereas rituximab was given in 32% of patients, and 64% of these showed improvement. At the last visit, 27% of patients could not walk independently.ConclusionsThis study on anti‐MAG neuropathy provides updated insights into the epidemiology of this disease, clinical profiles, and treatment approaches in Japan. Rituximab therapy, used for only one‐third of the patients, demonstrated efficacy. During the final visit, a quarter of the patients were unable to walk independently. Further studies are warranted to determine the optimal management of this rare and intractable disorder.

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Monoclonal Gammopathy of Undetermined Significance: A Comprehensive Review

Kaur

Valisekka

Hameed

et al. 2023

Clinical Lymphoma Myeloma and Leukemia

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Polyneuropathy and monoclonal gammopathy of undetermined significance (MGUS); update of a clinical experience

Cited by 5 publications

References 28 publications

Efficacy of rituximab in anti‐myelin‐associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow‐up

Efficacy of rituximab in anti‐myelin‐associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow‐up

Prevalence and clinical profiles of anti‐myelin‐associated glycoprotein neuropathy in Japan: A nationwide survey study of 133 patients

Monoclonal Gammopathy of Undetermined Significance: A Comprehensive Review

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