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NEUROLOGY 2007;68:E14
© 2007 American Academy of Neurology
Resident and Fellow Section

Neurofibromatosis type 2

S. Sathornsumetee, MD, A. DesJardins, MD, FRCP(C), D. A. Reardon, MD, J. N. Rich, MD and J. J. Vredenburgh, MD

From the Preston Robert Tisch Brain Tumor Center (S.S., A.D., D.A.R., J.N.R., J.J.V.), Departments of Medicine (S.S., A.D., J.N.R., J.J.V.), Surgery (D.A.R., J.N.R.), and Neurobiology (J.N.R.), Duke University Medical Center, Durham, NC.

Address correspondence and reprint requests to Dr. Sith Sathornsumetee, The Preston Robert Tisch Brain Tumor Center, Department of Medicine, Duke University Medical Center, DUMC 3624, Durham, NC 27710; e-mail: satho001{at}mc.duke.edu

A 45-year-old woman developed a gradual onset of sensorineural hearing loss in the left ear in 1984. Head CT scan with contrast demonstrated bilateral cerebellopontine angle (CPA) enhancing masses. She underwent a resection of the left CPA tumor with pathology confirming schwannoma. Despite several resections, she had progressive hearing loss and she became deaf in 2003. Her brain MRI (figure) demonstrated bilateral vestibular schwannomas, which are sufficient for a definite diagnosis of neurofibromatosis type 2 (NF-2), according to the National Institutes of Health, Manchester, or National Neurofibromatosis Foundation diagnostic criteria.1 In addition, this patient also has multiple meningiomas, which are associated with NF-2.2 The patient has been treated with imatinib mesylate (Gleevec) plus hydroxyurea in a clinical trial for her progressive meningiomas. At her last follow-up visit, she was neurologically and radiographically stable after 12 months of treatment.


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  		Figure. T1-weighted MRI revealed large gadolinium-enhancing masses in bilateral cerebellopontine angles (large white arrows) with extension into the internal auditory canals (small white arrows), characteristic of vestibular schwannomas. Several extra-axial enhancing masses were present in the posterior fossa and the right middle cranial fossa (black arrows), consistent with meningiomas.

 

Footnotes

Disclosure: The authors report no conflicts of interest.

Received August 11, 2006. Accepted in final form November 27, 2006.

References

  1. Baser ME, Friedman JM, Wallace AJ, Ramsden RT, Joe H, Evans DG. Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology 2002;59:1759–1765.[Abstract/Free Full Text]
  2. Antinheimo J, Sankila R, Carpen O, Pukkala E, Sainio M, Jaaskelainen J. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology 2000;54:71–76.[Abstract/Free Full Text]




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