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KMID : 0391520160240030168
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Journal of the Korean Child Neurology Society
2016 Volume.24 No. 3 p.168 ~ p.173
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Symptomatic Neurocutaneous Melanosis Associated with Dandy Walker Complex
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Koh Young-Kwon
Joo Jung-Yeon
Lee Sung-Chan
Rho Young-Il
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Abstract
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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.
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KEYWORD
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Neurocutaneous melanosis, Dandy Walker complex, Intravenous immunoglobulins
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