댄디 워커 증후군이 동반된 증후성 신경피부멜라닌증.

Neurocutaneous melanosis (NCM) is a rare nonheritable congenital phakomatosis. NCM is characterized by large or multiple (>3) congenital nevi that are associated with benign and/or malignant melanocytic tumors of the leptomeninges. Approximately 10% of symptomatic patients with NCM have associated Dandy Walker complex (DWC), which seems to have an extremely poor prognosis. We report the case of a 7 year-old patient with multiple congenital NCM associated with DWC, who experienced a transient improvement in neurological symptoms including mental status and hemiparesis after intravenous immunoglobulin (IVIG) therapy. We suggest that the administration of IVIG may play a role in the treatment of symptomatic NCM. [ABSTRACT FROM AUTHOR]