Journal of Neurology and Neuroscience

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Intracranial hypertension: Think of leptomeningeal melanocytosis

Ouardia Belarbi 1, Si Ahmed Hakim2, Hatteb Samira2, Daoudi Smail2

Leptomeningeal melanocytosis is a rare primary melanocytic lesion of the central nervous system. It is considered a severe condition with poor prognosis.

It is characterized by diffuse melanocytic infiltration of the leptomeninges. Seen almost exclusively in children with large congenital nevi and together the findings form a dermatologic syndrome known as neurocutaneous melanosis (NCM).

We report a rare and atypical case of a 45-year-old adulte male, with no evident congenital melanocytic lesions who presented visual manifestations without other clinical signs of intracranial hypertension, and were found to have leptomeningeal melanocytosis with fatal evolution.