J Korean Neurosurg Soc.  1975 Oct;4(2):405-408.

A Case of Neurocutaneous Melanosis

• Suck JS
• Kim JH
• Kim HJ
• Choi KS
• Sim BS

Abstract

In 1861, Rokitansky first reported a unusual syndrome compatible with neurocutaneous melanosis, but it was coined by Van Bogaert in 1948. Neurocutaneous melanosis is a very much rare and less well-known member of phakomatoses. A review of the published material showed that only 40 cases of this syndrome had been reported until 1968 by Fox. It has been defined as a congenital disease which is manifested by parallel development of benign melanotic pigmentation of central nervous system and pigmented nevi of the skin. We have recently experienced a case of neurocutaneous melanosis. A 23 years old female was admitted to our hospital with complaints of generalized cafe au lait spots and giant nevi on the back and the left thigh accompanying severe headache, vomiting and right hearing disturbance. Diagnostic studies did not suggest any intracranial space-taking lesion except for evident hydrocephalus. Under the impression of Von Recklinghausen disease with right acoustic neurinoma the posterior fossa exploration was performed but no space talking lesion was noted. Numberous dark black-brownish pimentation was spread over the leptomeninges of the cisterna magna, brain stem, and right cerebellum. The increased intracranial pressure signs were completely relieved by ventriculo-atrial shunt. On biopsy it was identified as leptomeningeal melanin pigmentation. Detail consideration of neueurocutaneous melanosis was introduced with review of ever reported cases.

MeSH Terms

Biopsy
Brain Stem
Cafe-au-Lait Spots
Central Nervous System
Cerebellum
Cisterna Magna
Female
Headache
Hearing
Humans
Hydrocephalus
Intracranial Pressure
Melanins
Melanosis*
Neurocutaneous Syndromes
Neurofibromatosis 1
Neuroma, Acoustic
Nevus
Nevus, Pigmented
Numismatics
Pigmentation
Skin
Thigh
Vomiting
Young Adult
Melanins