Neuromuscular Disease Group, Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX, Utrecht, The Netherlands.
J Neurol. 2011 Feb;258(2):212-7. doi: 10.1007/s00415-010-5712-3. Epub 2010 Aug 28.
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.
在单发性多发性运动神经病(MMN)病例中,有报道称在寒冷时(冷麻痹)会出现肌力减弱。这是出乎意料的,因为脱髓鞘是 MMN 的特征,而且脱髓鞘的症状在寒冷时会改善,而不是恶化。据推测,MMN 中的冷麻痹不仅反映脱髓鞘,还可能表明存在具有永久去极化轴突的炎症性神经病变,这些轴突仅在正常温度下传导,但在较低温度下则无法传导。我们调查了 50 例 MMN 患者、48 例慢性炎症性脱髓鞘性多发性神经病(CIDP)患者、35 例进行性脊肌萎缩症(PSMA)患者和 25 例慢性特发性轴索性神经病患者的冷麻痹症状。我们还调查了在温暖时(热麻痹)肌力增强的症状。冷麻痹的报告比热麻痹更为常见。冷麻痹在 MMN 中最为常见。多变量分析表明,MMN 患者比 CIDP 或 PSMA 患者报告冷麻痹的风险高 4 至 6 倍。由于冷麻痹与脱髓鞘不一致,MMN 中的病变可能涉及除脱髓鞘以外的其他机制。总之,冷麻痹的症状在周围神经系统疾病中很常见,尤其是在 MMN 中。这支持了上述假设。