Cold paresis in multifocal motor neuropathy
Cold paresis in multifocal motor neuropathy
复制标题
多灶性运动神经病中的冷性麻痹
作者：
D. Straver;J. V. van Asseldonk;N. Notermans;J. Wokke;L. H. van den Berg;H. Franssen
在多灶性运动神经病变（MMN）的单个病例中报告了在寒冷期间增加的虚弱（冷性轻瘫）。这是出乎意料的，因为脱髓鞘是MMN的一个特征，脱髓鞘的症状在寒冷中会改善，而不是恶化。假设MMN的冷性轻瘫并不仅仅反映脱髓鞘，而可能表明存在具有永久性去极化轴突的炎性神经病变，其仅在常温下传导，但在低温下失效。我们研究了50例MMN患者、48例慢性炎症性脱髓鞘性多神经病变（CIDP）患者、35例进行性脊髓性肌萎缩（PSMA）患者和25例慢性特发性轴突多神经病变患者的冷性轻瘫症状。我们还调查了在温暖时虚弱增加的症状（热轻瘫）。冷性轻瘫比热性轻瘫更常见。冷性麻痹在MMN中最常见。多因素分析表明MMN患者报告冷性轻瘫的风险比CIDP或PSMA患者高4- 6倍。由于冷性麻痹与脱髓鞘不一致，MMN的病变可能涉及其他机制，而不仅仅是脱髓鞘。总之，冷性瘫的症状在周围神经系统疾病中很常见，尤其是MMN。这支持了上述假设。
Increased weakness during cold (cold paresis) was reported in single cases of multifocal motor neuropathy (MMN). This was unexpected because demyelination is a feature of MMN and symptoms of demyelination improve, rather than worsen, in cold. It was hypothesized that cold paresis in MMN does not reflect demyelination only, but may indicate the existence of inflammatory nerve lesions with permanently depolarized axons that only just conduct at normal temperature, but fail at lower temperatures. We investigated symptoms of cold paresis in 50 MMN patients, 48 chronic inflammatory demyelinating polyneuropathy (CIDP) patients, 35 progressive spinal muscular atrophy (PSMA) patients, and 25 chronic idiopathic axonal polyneuropathy patients. We also investigated symptoms of increased weakness during warmth (heat paresis). Cold paresis was reported more often than heat paresis. Cold paresis was most frequently reported in MMN. Multivariate analysis indicated that MMN patients had a 4- to 6-fold higher risk of reporting cold paresis than CIDP or PSMA patients. Because cold paresis is not consistent with demyelination, the lesions in MMN may involve other mechanisms than demyelination only. In conclusion, symptoms of cold paresis are common in peripheral nervous system disorders, particularly in MMN. This supports the above-described hypothesis.
Kaji R：“多灶性运动神经病和其他脱髓鞘性神经病中传导阻滞的生理学”肌肉神经.. 27(3)。
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