神经-皮肤黑色素沉着症(nsurocutaneuos melanosis)是一种少见疾患,其特征有多发或巨型先天性色素痣,合并软脑膜黑色素沉着。到1977年国外共报道68例,国内曾报道2例。我组报告一例。患者,12岁,自幼全身皮肤散在黑色素痣,随年龄增长而逐渐增多并长大。入院前两个月无明显诱因阵发性头疼,喷射状呕吐。 Neuro-skin melanosis (nsurocutaneuos melanosis) is a rare disorder characterized by multiple or giant congenital pigmented nevus with melanotic pigmentation of the pia mater. In 1977, 68 cases were reported abroad, and 2 cases were reported in China. One case was reported by my group. The patient, aged 12 years old, had scattered all over the body with melanin pigmentation, which gradually increased and grew with age. Two months before admission, there was no obvious cause of paroxysmal headache and jet-like vomiting.