Melanose Neurocutânea com Invasão de Sistema Nervoso Central (SNC) Cerebral e Espinhal: relato de caso em mulher jovem

Neurocutaneous Melanose with Brain and Spinal Central Nervous System (CNS) Invasion: case report in young woman

• Guilherme Wandall (1)    
• Sheila Wayszceyk (1)    
• Chelin Auswaldt Steclan (2)    
• Arlindo Américo de Oliveira (3)    
• Michael Ricardo Lang (4)    
• Gabriel Hoher Peres (5)    
• Leonard Rocha Fonseca de Brito (6)    
• Filipe Laurindo Cabral (7)    

• https://doi.org/10.22290/jbnc.2023.340101
• J Bras Neurocirur 34(1): 119-123, 2023

Resumo

Palavras-chave

Abstract

Neurocutaneous melanosis is a rare syndrome that associates a cutaneous manifestation with the central nervous system. It can evolve benign or malignant progression, and when presented early in life it is associated with a high mortality rate. Therefore, considering it as a rare clinical case involving a young woman, this study aimed to describe a case of an 18-year-old female patient with cutaneous melanosis during her first and second childhood, and later evolving to neurocutaneous melanosis when she was young. All data here presented was obtained through findings in medical records; reports associated with neuroimaging and laboratory tests were considered. Neuroimaging findings enabled the diagnosis of acute hydrocephalus. After biopsy of the arachnoid from the prepontine cistern, the presence of melanocytic cells in the CNS was confirmed. After ventricular bypass procedures, even with immediate clinical improvement after the procedures, the patient subsequently evolved with tetraparesis and palliative care, culminating in death. Thus, the present report addresses the clinical history of a young woman with the rare form of neurocutaneous melanosis that progressed to the central nervous system of the brain and spinal cord. This description of rare conditions enriches knowledge and allows guiding other diagnostic and therapeutic approaches.

Keywords

References

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1 Medical Student, Universidade Regional de Blumenau – FURB, Blumenau (SC), Brazil.

2 PhD, Biologist, Neuromax Neurologia e Neurocirurgia; University of Contestado – NUPESC, Mafra (SC), Brazil.

3 MD, Neurosurgeon, Neuromax Neurologia e Neurocirurgia; São Vicente de Paulo Hospital – HSVP, Mafra (SC) and Santa Teresa University Hospital – HUST, Joaçaba (SC), Brazil.

4 MD, Neurosurgeon, Neuromax Neurologia e Neurocirurgia; São Vicente de Paulo Hospital – HSVP, Mafra (SC), Brazil.

5 MD, Neurosurgeon, Neuromax Neurologia e Neurocirurgia; Beatriz Ramos Hospital – HBR, Indaial (SC), Brazil.

6 MD, Neurosurgeon, Neuromax Neurologia e Neurocirurgia; São José Hospital – HSJ, Jaraguá do Sul (SC); IDOMED Estácio, Jaraguá do Sul (SC), Brazil.

7 MD, Neurosurgeon, Neuromax Neurologia e Neurocirurgia; Santo Antônio Hospital – HSA, Blumenau (SC), Brazil.

Received Jan 10, 2023

Accepted Feb 6, 2023