[Neurocutaneous melanosis. Report of a case].

Abstract:

:The clinical, laboratorial and radiological features of a 3-month-old child with neurocutaneous melanosis are described. The patient was born with multiple disseminated benign cutaneous nevi, proven by skin-biopsy, and presented with drug-resistant seizures and psycho-motor retardation. Serial cerebrospinal fluid studies showed high protein and low glucose levels, with pleocytosis and malignant cells in the fluid. Pneumoencephalogram showed mild non-obstructive hydrocephalus. Immunological studies showed normal immunological activity at three months of age and very poor activity at one year. The patient died at 16 months of age, after the sudden onset of intracranial hypertension and meningeal signs. Immunotherapy and chemotherapy were suggested but not accepted by the family. Some comments are made based on this and other 43 cases described in the literature; the value of laboratory studies in detecting malignant transformaiton in the meninges in patients with benign skin nevi is stressed.

journal_name

Arq Neuropsiquiatr

authors

Brandt RA,Schwartzman JS,Naspitz C

doi

10.1590/s0004-282x1978000200008

subject

Has Abstract

pub_date

1978-06-01 00:00:00

pages

147-54

issue

2

eissn

0004-282X

issn

1678-4227

journal_volume

36

pub_type

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