NF2.

Sagittal MR image of the spine demonstrating a T10-11 schwannoma in a patient with NF2.
Axial MR image demonstrating tumors in a patient with NF2 who presented with the classic bilateral eighth cranial nerve schwannomas that occur in this disease.

Discussion:

Neurofibromatosis type 2
-One of the phakomatoses (neuroectodermal disorders with skin and
CNS tumors; other of the more common phakomatoses include tuberous sclerosis, Von-hippel Lindau disease, Sturge-Weber

-Incidence 1:50,000 (much less common than NF-1)
-Autosominal dominant inheritance via chromsome 22 defect

Diagnostic criteria:
-Bilateral vestibular schwanommas are diagnostic
-First degree relative with NF-2 plus unilateral vestibular
schwanomma, meningioma, glioma, scwhanomma, neurofibroma
-Cutaneous manifestations are rare

Radiographic features:
-~100% have cns tumors
-Bilateral vestibular schwanomma is diagnostic
-Other cranial nerve schwanommas
-Meningiomas (can be multiple)
-Non-neoplastic intracranial calcifications (especially
choroid plexus, but can also be cortical)
-Spinal cord schwanommas and ependymomas

http://www.google.com.eg/imgres?imgurl=http://img.medscape.com/fullsize/migrated/522/319/nf522319.fig5.gif&imgrefurl=http://www.medscape.com/viewarticle/522319_4&usg=__7bIYNRkY_Pmcp7PtiS7MG5bYpdA=&h=551&w=400&sz=137&hl=ar&start=9&zoom=1&itbs=1&tbnid=JzdQZhb1WY45KM:&tbnh=133&tbnw=97&prev=/images%3Fq%3DNeurofibromatosis%2Btype%2B2%26hl%3Dar%26sa%3DG%26gbv%3D2%26tbs%3Disch:1