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Acta Neurológica Colombiana

versão impressa ISSN 0120-8748

Resumo

RUIZ, Adriana Marcela; LIZARAZO, Diego; FANDINO, Rafael  e  LIZARAZO, Jairo. A case of neurocutaneous melanosis: the dark side of the giant congenital melanocytic nevus. Acta Neurol Colomb. [online]. 2020, vol.36, n.1, pp.34-38. ISSN 0120-8748.  http://dx.doi.org/10.22379/24224022275.

Neurocutaneous melanosis is a non-hereditary phakomatosis, characterized by the proliferation of melanocytes and melanin deposits in the central nervous system associated with a giant congenital melanocytic nevus or multiple melanocytic nevi satellites.

CLINICAL CASE.

A young woman with congenital giant melanocytic nevus and satelitosis, presented with intracraneal hypertension associated with communicating hydrocephalus. She was treated with cerebrospinal fluid shunt with partial improvement; but she worsened, presenting seizures and later cervical spinal cord involvement that led to prolonged mechanical ventilation and eventually death. MRI showed lesions in leptomeninges.

CONCLUSIONS.

Neurocutaneous melanosis should be considered in adult patients with congenital melanocytic nevus and clinical neurological manifestations.

Palavras-chave : Epilepsy; Hydrocephalus; Hyperthyroidism; Intracranial hypertension; Nevus; Melanosis (MeSH).

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