Neurofibromatosis type two with associated spinal schwannomas

Authors

  • Yourika Govender

Abstract

Neurofibromatosis (NF) is a rare genetic disorder characterized by the development of benign tumors in the nervous system. This pathology is classified into neurofibromatosis type one (NF1) and neurofibromatosis type two (NF2). Schwannomatosis is a newly recognized third form of NF. It is characterized by multiple schwannomas, without vestibular schwannomas which are diagnostic of NF2. This case report describes the role of imaging and the management of a patient diagnosed as NF2 who later developed multiple spinal schwannomas. NF2 occurs as a result of genetic defects caused by a mutation on a gene located on chromosome number 22. It is characterized by multiple schwannomas, meningiomas, and ependymomas. Multiple cranial nerve schwannomas, as well as bilateral acoustic neuromas or bilateral vestibular schwannomas, are the hallmark of the disease. Tumors of the brain and spine eventually occur in most patients with NF2.

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Published

2008-05-09

Issue

Vol. 46 No. 1 (2008)

Section

Case Reports

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