Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient.

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Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient.

An Bras Dermatol. 2017 Nov-Dec;92(6):870-873

Authors: Serafini NB, Serafini CB, Vinhas AS, Godinho MB

Abstract
Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots, multiple neurofibromas, and ephelides in skin fold areas, as well as hamartomatous lesions in the eyes, bones, glands, and central nervous system. Moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with important ischemic cerebrovascular events. Despite the rarity of this association in childhood, children diagnosed with neurofibromatosis type 1 and focal neurologic symptoms should be investigated for moyamoya syndrome. The present study reports the case of a pediatric patient with a rapidly progressive cerebrovascular accident and a late diagnosis of Neurofibromatosis type 1 associated with moyamoya disease.

PMID: 29364453 [PubMed - indexed for MEDLINE]

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