Abstract

Abstract A 50-year-old male presented to the emergency room with a sudden onset right upper limb facial numbness and dysphasia, soon followed by full-recovery. Brain computed tomography revealed hyperdense lesions within the left frontal sulcus, highly suspicious of subarachnoid hemorrhage. T1-weighted magnetic resonance imaging showed multiple leptomeningeal enhancement in the left frontal area.The digital subtraction angiography indicated an absence of vascular lesions. One-month after, the lesions progressed in the occipital area, when left sixth nerve palsy developed. Multiple giant nevus over the legs, trunk, and scalp had attracted attention. Owing to the suspicious symptomatic leptomeningeal lesions, open biopsy was performed. Surprisingly, diffuse, thick, black-colored tissue infiltration of the subarachnoid space, involving prominent cortical vessels, was noted. Pathology confirmed meningeal melanocytosis. Neurocutaneous melanosis is characterized by giant congenital or multiple satellite nevi and melanocytic tumors of the leptomeninges. Early disease diagnosis is crucial. Once the patient develops neurological symptoms, prognosis could quickly worsen.

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