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Visual Loss Secondary to Increased Intracranial Pressure in Neurofibromatosis Type 2
Arch Ophthalmol. 1999;117:1650-1653.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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Neurofibromatosis type 2 (NF2), previously known as central neurofibromatosis, is defined clinically by the presence of bilateral acoustic nerve schwannomas and genetically by a mutation in the long arm of chromosome 22.1 Schwannomas of other cranial and spinal nerves are often present, as well as meningiomas. Patients typically have hearing loss in the second or third decade of life. About 50% of cases are inherited in an autosomal dominant fashion.
Among the many ophthalmic signs that have been described in NF2,1-4 papilledema has not been highlighted. We report 2 cases in which the dire consequences of sustained elevated intracranial pressures were not recognized in time to prevent visual loss.
Report of Cases
Case 1
A 31-year-old man reported a 2-year history of worsening bilateral hearing loss and dizziness in 1992. Bilateral sensorineural hearing was documented. Results of a neurological examination were otherwise normal. A magnetic resonance imaging (MRI) scan disclosed masses coating the cerebral convexities . . . [Full Text of this Article] Case 2 Comment
Reprints: Jonathan D. Trobe, MD, W. K. Kellogg Eye Center, 1000 Wall St, Ann Arbor, MI 48105 (e-mail: jdtrobe@umich.edu).
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