Charcot‐Marie‐Tooth disease type 1A (CMT1A) is the most common type of hereditary neuropathy worldwide and diabetes mellitus (DM) is the most frequent cause of peripheral neuropathy in the Western world. CMT1A typically manifest as a predominant motor neuropathy, while, DM‐related neuropathy often manifests as a predominant sensory disorder. There are some evidences that CMT1A patients that also had DM had a more severe neuropathy. Although the real frequency and the underlying mechanisms related to this association has not yet been addressed in the literature. We sought to characterize the phenotypic variability of CMT1A patients with persistent high glucose levels (DM or impaired glucose tolerance [IGT]). Nineteen patients with CMT1A and DM (CMTdiab), seven with CMT1A and IGT (CMTintol) and 27 with CMT1A without comorbidities were analyzed. They were evaluated through clinical assessment, application of the following scales: visual analogue scale, McGill, CMTNS, SF‐36 and COMPASS 31 and electrophysiological studies. Patients CMTdiab had a more severe motor and sensory neuropathy, more intense autonomic symptoms and worse quality of life. Surprisingly, proximal weakness and temporal dispersion on nerve conduction studies are frequently observed in this group. Patients CMTintol also had a more severe neuropathy. Curiously, we observed that the association of CMT1A and glucose metabolism disorders (CMTglic) clustered in some families. Patients CMTglic develop a more severe neuropathy. As there is yet no cure to CMT1A, a strict blood sugar control may be a useful measure.

中文翻译：

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高血糖水平作为CMT1A患者的调节因子。

1A型Charcot‐Marie‐Tooth疾病（CMT1A）是世界范围内最常见的遗传性神经病，而糖尿病（DM）是西方世界最常见的周围神经病的病因。CMT1A通常表现为主要的运动神经病，而DM相关的神经病通常表现为主要的感觉障碍。有证据表明，同时患有DM的CMT1A患者的神经病变更为严重。尽管与这种关联有关的实际频率和潜在机制尚未在文献中得到解决。我们试图表征持续高血糖水平（DM或糖耐量降低[IGT]）的CMT1A患者的表型变异性。19例CMT1A和DM（CMT diab），7例CMT1A和IGT（CMT tol）和27例没有合并症的CMT1A进行了分析。通过临床评估，以下量表的应用对他们进行了评估：视觉模拟量表，McGill，CMTNS，SF‐36和COMPASS 31以及电生理研究。CMT糖尿病患者的运动和感觉神经病更为严重，自主神经症状更为严重，生活质量较差。出人意料的是，在该组中经常观察到近端无力和神经传导的时间分散。CMT患者也有较严重的神经病。奇怪的是，我们观察到CMT1A与葡萄糖代谢异常（CMT glic）的关联在某些家庭中聚集。患者发生CMT糖化更严重的神经病。由于尚未治愈CMT1A，因此严格控制血糖可能是一种有用的措施。