Neurofibromatosis: part 2 – clinical management

• Autor(a) principal: Batista,Pollyanna Barros
• Data de Publicação: 2015
• Outros Autores: Bertollo,Eny Maria Goloni, Costa,Danielle de Souza, Eliam,Lucas, Cunha,Karin Soares Gonçalves, Cunha-Melo,José Renan, Darrigo Junior,Luiz Guilherme, Geller,Mauro, Gianordoli-Nascimento,Ingrid Faria, Madeira,Luciana Gonçalves, Mendes,Hérika Martins, Miranda,Débora Marques de, Mata-Machado,Nikolas Andre, Morato,Eric Grossi, Pavarino,Érika Cristina, Pereira,Luciana Baptista, Rezende,Nilton Alves de, Rodrigues,Luíza de Oliveira, Sette,Jorge Bezerra Cavalcanti, Silva,Carla Menezes da, Souza,Juliana Ferreira de, Souza,Márcio Leandro Ribeiro de, Martins,Aline Stangherlin, Valadares,Eugênia Ribeiro, Vidigal,Paula Vieira Teixeira, Waisberg,Vanessa, Waisberg,Yehuda
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Arquivos de neuro-psiquiatria (Online)
• Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2015000600531
• topic: neurofibromatosis; neurofibromatosis type 1; neurofibromatosis type 2; schwannomatosis; Legius syndrome

Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.