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Abstract：
Neurocutaneous melanosis (NCM) is a rare, non-hereditary neurocutaneous disorder characterized by excessive melanocytic proliferation in the skin and central nervous system. As no major studies have covered the incidence of NCM among Japanese patients with congenital melanocytic nevi (CMN), we prospectively investigated the incidence of NCM among Japanese patients who underwent initial treatment for CMN. The relationship of CMN and NCM was also investigated. Japanese pediatric patients with CMN under 1 year of age were included between January 2020 and November 2022, and all patients underwent brain MRI to check for NCM in this study. NCM lesions were most frequently seen in the amygdala, followed by the cerebellum, brainstem, and cerebral hemispheres. NCM was diagnosed on brain MRI in 31.6% of the 38 patients with CMN and in 25.0% of patients with no prior examination or treatment. Distribution and size of CMN, number of satellite nevi, rugosity and nodules were strongly associated with the existence of NCM, and these findings may guide a future registry study with a large cohort of CMN patients.
摘要：
神经皮肤黑变病（NCM）是一种罕见的，以皮肤和中枢神经系统黑素细胞过度增生为特征的非遗传性神经皮肤疾病。由于尚无主要研究涵盖日本先天性黑素细胞痣（CMN）患者中NCM的发生率，我们前瞻性调查了接受CMN初始治疗的日本患者中NCM的发生率.还研究了CMN与NCM的关系。在2020年1月至2022年11月期间，纳入了1岁以下的日本CMN儿科患者，所有患者都接受了脑部MRI检查，以检查NCM。NCM病变最常见于杏仁核，其次是小脑,脑干,和大脑半球。在38例CMN患者中，有31.6％的人在脑MRI上诊断出NCM，在没有先前检查或治疗的患者中，有25.0％的人在脑MRI上诊断出NCM。CMN的分布和大小，卫星Nevi的数量，粗糙性和结节与NCM的存在密切相关，这些发现可能会指导未来对大量CMN患者进行的注册研究。
