Lozeron Pierre, Ribrag Vincent, Adams David, Brisset Marion, Vignon Marguerite, Baron Marine, Malphettes Marion, Theaudin Marie, Arnulf Bertrand, Kubis Nathalie
Service de Physiologie Clinique-Explorations Fonctionnelles, AP-HP, Hôpital Lariboisière, 2, rue Ambroise Paré, 75010, Paris, France.
INSERM UMR965, Université Paris Diderot, Sorbonne Paris Cité, Paris, France.
J Neurol. 2016 Sep;263(9):1761-70. doi: 10.1007/s00415-016-8187-z. Epub 2016 Jun 17.
To report the frequency of the different patterns of sensory and motor electrophysiological demyelination distribution in patients with anti-MAG neuropathy in comparison with patients with IgM neuropathy without MAG reactivity (IgM-NP). Thirty-five anti-MAG patients at early disease stage (20.1 months) were compared to 23 patients with IgM-NP; 21 CIDP patients and 13 patients with CMT1a neuropathy were used as gold standard neuropathies with multifocal and homogeneous demyelination, respectively. In all groups, standard motor and sensory electrophysiological parameters, terminal latency index and modified F ratio were investigated. Motor electrophysiological demyelination was divided in four profiles: distal, homogeneous, proximal, and proximo-distal. Distal sensory and sensorimotor demyelination were evaluated. Anti-MAG neuropathy is a demyelinating neuropathy in 91 % of cases. In the upper limbs, reduced TLI is more frequent in anti-MAG neuropathy, compared to IgM-NP. But, predominant distal demyelination of the median nerve is encountered in only 43 % of anti-MAG neuropathy and is also common in IgM-NP (35 %). Homogeneous demyelination was the second most frequent pattern (31 %). Concordance of electrophysiological profiles across motor nerves trunks is low and median nerve is the main site of distal motor conduction slowing. Reduced sensory conduction velocities occurs in 14 % of patients without evidence of predominant distal slowing. Simultaneous sensory and motor distal slowing was more common in the median nerve of anti-MAG neuropathy than IgM-NP. Electrophysiological distal motor demyelination and sensory demyelination are not a distinctive feature of anti-MAG reactivity. In anti-MAG neuropathy it is mainly found in the median nerve suggesting a frequent nerve compression at wrist.
报告抗MAG神经病患者与无MAG反应性的IgM神经病(IgM-NP)患者相比,感觉和运动电生理脱髓鞘分布的不同模式的频率。将35例处于疾病早期(20.1个月)的抗MAG患者与23例IgM-NP患者进行比较;分别将21例慢性炎症性脱髓鞘性多发性神经病(CIDP)患者和13例遗传性运动感觉神经病1型(CMT1a)神经病患者作为具有多灶性和均匀性脱髓鞘的金标准神经病。在所有组中,研究了标准运动和感觉电生理参数、终末潜伏期指数和改良F比率。运动电生理脱髓鞘分为四种类型:远端型、均匀型、近端型和近端-远端型。评估远端感觉和感觉运动脱髓鞘。91%的抗MAG神经病病例为脱髓鞘性神经病。在上肢,与IgM-NP相比,抗MAG神经病中终末潜伏期指数降低更为常见。但是,仅43%的抗MAG神经病出现正中神经主要为远端脱髓鞘,在IgM-NP中也很常见(35%)。均匀性脱髓鞘是第二常见的类型(31%)。运动神经干电生理类型的一致性较低,正中神经是远端运动传导减慢的主要部位。14%的患者出现感觉传导速度降低,无明显的主要远端减慢证据。抗MAG神经病的正中神经同时出现感觉和运动远端减慢比IgM-NP更常见。电生理远端运动脱髓鞘和感觉脱髓鞘不是抗MAG反应性的独特特征。在抗MAG神经病中,主要见于正中神经,提示腕部经常发生神经受压。