Neurofibromatosen

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• 作者：Dr. A. Zimmer (1)
  
• 关键词：Phakomatosen ; Diagnosekriterien ; Bildbefunde ; Morbus Recklinghausen ; Schwannome ; Phacomatosis ; Diagnostic criteria ; Imaging ; Recklinghausen disease ; Schwannomas
• 刊名：Der Radiologe
• 出版年：2013
• 出版时间：December 2013
• 年：2013
• 卷：53
• 期：12
• 页码：1077-1083
• 全文大小：
• 作者单位：Dr. A. Zimmer (1)
  
  1. Klinik f Diagnostische und Interventionelle Neuroradiologie, Universittsklinikum des Saarlandes, Kirrberger Strae, 66421, Homburg/Saar, Deutschland
  
• ISSN：1432-2102

文摘

Neurofibromatosis type1 (NF1) and type2 (NF2) are hereditary autosomal dominant neurocutaneous disorders, the phacomatoses, characterized by the development of tumors derived from the cells of the peripheral nerve sheath and also includes schwannomatosis. Regarding the clinical and genetic aspects they are, however, two distinct entities which are described separately in the following review. In addition to the typical clinical presentation and diagnostic criteria, characteristic imaging findings are presented especially in terms of the role of imaging in the diagnosis, follow-up and assessment of prognostic aspects in these multisystemic disorders.