Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient
An. bras. dermatol
;
92(6): 870-873, Nov.-Dec. 2017. graf
Article
in English
| LILACS
| ID: biblio-887126
ABSTRACT
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Neurofibromatosis 1
/
Moyamoya Disease
Type of study:
Risk factors
Limits:
Child, preschool
/
Humans
/
Male
Language:
English
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2017
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Rio de Janeiro/BR
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