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[Neurocutaneous melanosis--a case report]

S Yoshioka1, H Miyayama, A Ishihara

  • 1Department of Neurosurgery, Kumamoto Municipal Hospital, Japan.

No to Shinkei = Brain and Nerve
|March 1, 1994
PubMed
Summary

Neurocutaneous melanosis, a rare condition, involves congenital melanocytic nevi and leptomeningeal tumors. Early detection via MRI and CSF cytology is crucial for infants with extensive nevi, as malignant progression can be rapid.

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Area of Science:

  • Neurology
  • Dermatology
  • Pediatrics

Background:

  • Neurocutaneous melanosis is a rare congenital disorder.
  • It is characterized by congenital melanocytic nevi and leptomeningeal tumors.

Observation:

  • A 14-month-old boy presented with vomiting and drowsiness.
  • He had multiple congenital melanocytic nevi, hydrocephalus, and leptomeningeal enhancement on MRI.
  • CSF cytology revealed abnormal pigmented cells.

Findings:

  • Diagnosis of malignant neurocutaneous melanosis was made.
  • Despite aggressive treatment, the patient died due to rapid leptomeningeal infiltration.
  • Postmortem revealed meningeal malignant melanomas and benign melanosis.

Implications:

  • Infants with large or multiple congenital melanocytic nevi require close monitoring, especially in the first two years.
  • Hydrocephalus or convulsions in these infants warrant MRI and CSF cytology for early detection of meningeal abnormalities.

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