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.
Similar content being viewed by others
References
Beal M.F., Richardson E.P.:Primary lateral sclerosis: a case report. Arch. Neurol. 38:630–633, 1986.
Dalla Volta G., Magoni M., Vangi D., Vignolo L.:Role of MRI in the diagnosis of Mills sydnrome. Ital. J. Neurol. Sci. 10:519–521, 1989.
Fox H.:Neurocutaneous melanosis. In: Vinken P.J. and Bruyn G.W. eds. Handbook of Clinical Neurology. The Phakomatoses; vol. 14. Amsterdam, North-Holland, pp. 414–428, 1972.
Guidetti V., Garcovich A., Giuffré R., Curatolo P.:Nevo gigante congenito e melanosi neurocutanea. Considerazioni su due casi clinici. Min. Ped. 30:1961–1972, 1978.
Grunnett M.L., Leicher C., Zimmerman A., Zalneraitis E., Barwick M.:Primary lateral sclerosis in a child. Neurology 39:1530–1532, 1989.
Leaney B.J., Rowe P.W., Klug G.L.:Neurocutaneous melanosis with hydrocephalus and syringomyelia. J. Neurosurg. 62:148–152, 1985.
Malin J.P., Poburski R., Reusche E.:Klinische Varianten der amyotrophen Lateralsklerose: hemiplegischer Typ der ALS und Mills-Syndrom. Ein kritischer Rückblick. Fortschr. Neurol. Psichiat. 54:101–105, 1986.
Mills C.K.: A case of unilateral progressive ascending paralysis, probably representing a new form of degenerative disease. J. Nerv. Ment. Dis. 27:195–200, 1900.
Reed W.B., Becker Sr S.W., Becker Jr S.W., Nickel W.R.:Giant pigmented nevi, melanoma and leptomeningeal melanocytosis. Arch. Derm. 91:100–119, 1965.
Younger D.S., Chou S., Hays A.P., Lang D.J., Emeson R., Brin M., Thompson H., Rowland L.P.:Primary lateral sclerosis: a clinical diagnosis reemerges. Arch. Neurol. 45:1304–1307, 1988.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/BF02224400