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Giant congenital nevus and chronic progressive ascending hemiparesis (Mills syndrome). Report of a case

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Abstract

We describe the case of a 20 year old boy with a giant congenital nevus who developed a chronic progressive ascending hemiparesis. The association of the pigmented lesion with a focal neurological deficit is pathognomonic for neurocutaneous melanosis complicated by a leptomeningeal melanoma. MR imaging at 1.5 tesla ruled out such a possiblity and showed a small aspecific pontine lesion along the route of the corticospinal tract. We discuss possible etiologies.

Sommario

Descriviamo it caso di un ragazzo di 20 anni con un nevo congenito gigante che ha sviluppato una emparesi ascendente progressiva cronica. L'associazione della lesione pigmentata ad un deficit neurologico focale è patognomonica della melanosi neurocutanea complicata da un melanoma leptomeningeo. L'imaging con Risonanza Magnetica a 1.5 Tesla ha permesso di escludere questa possibilità ed ha dimostrato una piccola ed aspecifica lesione pontina lungo il decorso del tratto corticospinale. Discutiamo quindi le possibili cause eziologiche.

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Frisoni, G.B., Gasparotti, R. & Di Monda, V. Giant congenital nevus and chronic progressive ascending hemiparesis (Mills syndrome). Report of a case. Ital J Neuro Sci 13, 259–263 (1992). https://doi.org/10.1007/BF02224400

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