Korean J Med.  2009 Aug;77(Suppl 1):S183-S187.

Rapidly progressing neurocutaneous melanosis NCM in a 49-year-old woman

• Kim HJ
• Kim MJ
• Kim YJ
• Han JH
• Huh CH
• Lee HS
• Lee JS

Abstract

Symptomatic neurocutaneous melanosis (NCM) rarely presents in adulthood. NCM is a rare congenital syndrome characterized by large or multiple congenital melanocytic nevi (CMN) combined with benign or malignant melanocytic proliferation in the leptomeninges. This syndrome is believed to be caused by an error in the morphogenesis of embryonal neuroectoderm. The prognosis of symptomatic NCM is extremely poor, even in the absence of malignancy. We describe a 49-year-old patient with a large CMN and numerous satellite nevi who presented with obstructive hydrocephalus followed by a rapid neurological deterioration over 4 months. The rarity of NCM in an adult hindered early diagnosis, and the diagnosis of NCM was made after an extensive work-up for other causes. In patients with acute neurologic symptoms and underlying CMN, the likelihood of NCM with or without leptomeningeal melanoma should be considered.

Keyword

Neurocutaneous melanosis; Congenital melanocytic nevus

MeSH Terms

Adult
Early Diagnosis
Female
Humans
Hydrocephalus
Melanoma
Melanosis
Middle Aged
Morphogenesis
Neural Plate
Neurocutaneous Syndromes
Neurologic Manifestations
Nevus
Nevus, Pigmented
Prognosis
Melanosis
Neurocutaneous Syndromes