Wen W.-H.WANG-TSO LEELin M.-I.Lin H.-C.Chiu H.-C.Shen Y.-Z.2021-06-112021-06-1120011608-8115https://www.scopus.com/inward/record.uri?eid=2-s2.0-0035035204&partnerID=40&md5=56d8cbd5fef553e2c97d16e1759c2175https://scholars.lib.ntu.edu.tw/handle/123456789/565490Neurocutaneous melanosis is a rare congenital syndrome characterized by the association of large or multiple congenital melanocytic nevi and benign or malignant melanotic tumors in the central nervous system. Patients with neurocutaneous melanosis usually have neurological symptoms early in life that progress rapidly due to the development of increased intracranial pressure or malignant melanoma. We report a 2-month-old female infant with multiple congenital melanocytic nevi and frequent seizure attacks. Magnetic resonance imaging of the brain demonstrated several regions compatible with melanotic deposits. During follow-up for one year, she had normal development and was seizure-free under the treatment of phenobarbital and valproic acid. We suggest that infants with large or multiple congenital melanocytic nevi should receive regular clinical check-up and brain imaging to exclude the possibility of central nervous system lesions.Congenital melanocytic nevus; Epilepsy; Neurocutaneous melanosis[SDGs]SDG3phenobarbital; valproic acid; article; case report; central nervous system; clinical feature; disease association; disease course; epilepsy; female; follow up; human; infant; intracranial pressure; melanocytic nevus; melanoma; melanosis; neurologic disease; nuclear magnetic resonance imaging; phakomatosis; seizure; Epilepsy; Female; Humans; Infant; Melanosis; Neurocutaneous Syndromesjournal article113550632-s2.0-0035035204