Department of Neurology, Tübingen University Hospital, Tübingen, Germany.
Hertie Institute for Clinical Brain Research and German Center of Neurodegenerative Diseases (DZNE), University of Tübingen, Tübingen, Germany.
Eur J Neurol. 2017 Sep;24(9):1125-1134. doi: 10.1111/ene.13344. Epub 2017 Jul 5.
High-resolution ultrasound is a valuable tool in supporting the diagnosis of multifocal motor neuropathy (MMN) but longitudinal data under therapy are lacking.
The change in peripheral nerve ultrasound pattern in patients with MMN was assessed over time. Patients with MMN received a thorough initial examination and follow-up over a period of 6-12 months using high-resolution ultrasound of the cervical roots and the nerves of the arms and legs, nerve conduction studies, Medical Research Council Sum Score (MRCSS) and Rotterdam Inflammatory Neuropathy Cause and Treatment Group (INCAT) score to evaluate changes under treatment. The Ultrasound Pattern Sum Score (UPSS) was used as standardized peripheral nerve ultrasound protocol.
Seventeen patients with MMN received initial examinations of whom 12 were successfully followed up. All patients with MMN showed at least localized but often multifocal peripheral nerve enlargement. An enlarged overall cross-sectional area as well as enlarged single fascicles (>3 mm²) in clinically and electrophysiologically affected (>90%) and unaffected (>70%) nerves were found. The UPSS did not correlate with clinical disability at both visits. However, the change in clinical disability (evaluated as difference in MRCSS) and the change in UPSS correlated significantly inversely (P = 0.004).
High-resolution sonography of peripheral nerves revealed multifocal nerve enlargement in MMN. Distinct enlargement patterns may support the diagnosis. Ultrasound findings did not correlate well with clinical severity or electrophysiological findings at initial presentation. As changes in UPSS correlated significantly with the clinical course in terms of muscle strength (MRCSS), sonographic assessment may represent a useful tool for therapeutic monitoring.
高分辨率超声是支持多灶性运动神经病(MMN)诊断的有价值的工具,但缺乏治疗过程中的纵向数据。
通过高分辨率颈椎根和手臂及腿部神经超声、神经传导研究、肌电图(MRCSS)和鹿特丹炎症性神经病病因和治疗组(INCAT)评分,评估 MMN 患者的外周神经超声模式随时间的变化。对 MMN 患者进行全面的初始检查,并在 6-12 个月的时间内进行随访,以评估治疗过程中的变化。采用超声模式总分(UPSS)作为标准化外周神经超声方案。
17 例 MMN 患者接受了初始检查,其中 12 例成功随访。所有 MMN 患者均至少存在局灶性但常为多灶性周围神经肿大。在临床和电生理上受影响(>90%)和不受影响(>70%)的神经中,发现整个横截面积增大,以及单个束增大(>3mm²)。在两次就诊时,UPSS 均与临床残疾无相关性。然而,临床残疾的变化(用 MRCSS 差值表示)与 UPSS 的变化呈显著负相关(P=0.004)。
周围神经的高分辨率超声显示 MMN 存在多灶性神经肿大。明显的肿大模式可能支持诊断。在初次就诊时,超声发现与临床严重程度或电生理发现相关性不佳。由于 UPSS 的变化与肌肉力量的临床病程(MRCSS)显著相关,超声评估可能是治疗监测的有用工具。