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Neurofibromatosis 2 : Bilateral Vestibular Schwannomas

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Comments:
This patient was a young female who presented with seizures, left upper limb weakness and loss of consciousness. The MRI (post-contrast T1 coronal section) shows heterogeneous, moderately enhancing bilateral masses in the cerebellopontine angle cisterns extending into the internal acoustic meatii, consistent with bilateral acoustic schwannomas. The patient also had multiple meningiomas which are partially seen in this section (see next slide for better view). Case courtesy of Dr Imran Ahmad Khan, Radiopaedia.org. From the case rID: 42833

Baser criteria for the diagnosis of NF2:

A. Bilateral acoustic schwannomas.
B. First-degree family relative with NF2 and unilateral vestibular schwannoma or any two of the following: meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacity.
C. Unilateral vestibular schwannoma and any two of the following: meningioma, schwannoma, glioma, neurofibroma, posterior subcapsular lenticular opacity.
D. Multiple meningiomas (two or more) and unilateral vestibular schwannoma or any two of the following: schwannoma, glioma, neurofibroma, cataract.

References:
Baser, M. E., Friedman, J. M., Joe, H., Shenton, A., Wallace, A. J., Ramsden, R. T., & Evans, D. G. R. (2011). Empirical development of improved diagnostic criteria for neurofibromatosis 2. Genetics in Medicine, 13(6), 576-581.
Ardern-Holmes, Simone, Gemma Fisher, and Kathryn North. "Neurofibromatosis Type 2: Presentation, Major Complications, and Management, With a Focus on the Pediatric Age Group." Journal of Child Neurology 32.1 (2017): 9-22.

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