A diagnostic score for anti‐myelin‐associated‐glycoprotein neuropathy or chronic inflammatory demyelinating polyradiculoneuropathy in patients with anti‐myelin‐associated‐glycoprotein antibody

Abstract

Background and purpose:A diagnostic score was developed to discriminate anti-myelinassociated-glycoprotein (MAG) neuropathy from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and applied it to patients with atypical anti-MAG neuropathy. Methods:The clinical and electrophysiological features of patients with a diagnosis of typical anti-MAG neuropathy were compared to those of patients with a diagnosis of CIDP. The association of each feature with the diagnosis was assessed in the two groups.Features showing a significant association with the diagnosis were included in a multivariable logistic regression model and adjusted odds ratios were estimated for each feature.A score ranging from 1 to 3 was applied to each feature based on the magnitude of the estimated odds ratios. The score was then applied to patients with a clinical diagnosis of CIDP who also had high anti-MAG antibody titers (CIDP-MAG).Results: Thirty-one anti-MAG neuropathy patients, 45 typical CIDP patients and 16 CIDP-MAG patients were included. Scores in anti-MAG antibody patients ranged from 1 to 5 and in CIDP patients from −7 to −1. Using the score, 4/16 CIDP-MAG patients were diagnosed to have anti-MAG neuropathy and 12/16 patients to have CIDP. Response to intravenous immunoglobulin in the CIDP-MAG patients classified as CIDP was similar to that of definite CIDP patients and higher than that of anti-MAG neuropathy patients. Conclusions:Our score allowed an accurate discrimination to be made, amongst patients with anti-MAG antibodies, of those affected by CIDP and the patients with anti-MAG neuropathy. This score may help proper treatment to be chosen for patients with anti-MAG antibodies with a CIDP-like presentation.

Therapeutic Outcomes and Electrophysiological Biomarkers in Anti-Myelin-Associated Glycoprotein Neuropathy: A Multicenter Cohort Study in South Korea

“…The distal conduction distances were 60, 80, and 85 mm for the median and ulnar, peroneal, and tibial nerves, respectively. 42 The average distal CMAP amplitude (average CMAP) was defined as the arithmetic mean of CMAP amplitudes among the four motor nerves (median, ulnar, tibial, and peroneal). All parameters except for TLI were converted to percentage values relative to their respective normal limits to adjust for minor differences between the inference ranges of the participating centers ( Supplementary Table 1 in the online-only Data Supplement).…”

Mutational Profile in 75 Patients With Anti–Myelin-Associated Glycoprotein Neuropathy

“… 14 , 15 All the patients had neurophysiologic evidence of distal acquired demyelinating symmetric neuropathy with low conduction velocities and markedly prolonged distal latencies and no conduction blocks. Patients with atypical forms of anti-MAG antibody neuropathy 16 were not included.…”

Paraproteinemia‐associated neuropathy with or without anti‐myelin‐associated glycoprotein antibody

“…When CIDP with high anti‐MAG antibody titers was classified by the cutoff, there was no difference in the response to IVIg between the CIDP with high anti‐MAG antibody titers patients classified as CIDP by the score and the patients with definite typical CIDP. This score might assist in proper treatment, especially IVIg, being chosen for patients with anti‐MAG antibodies with a CIDP‐like presentation 37 …”