Radiological investigation of neurofibromatosis type 2
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The radiological findings in six patients fulfilling the criteria of neurofibromatosis type 2 (NF2) were reviewed. Subtle cutaneous lesions were found in three. All patients had bilateral acoustic schwannomas; two had small acoustic tumours and normal hearing. In these patients the presenting symptoms were caused by multiple intracranial meningiomas and spinal neurofibromas, respectively, whereas the remaining four patients presented with hearing loss. Two patients had other cranial nerve tumours. Three patients had rapidly growing multiple intracranial meningiomas; two had multiple spinal neurofibromas and one a spinal meningioma. NF2 is a rare disease with few cutaneous but frequent, typical radiological findings in the central nervous system. The presenting symptom is most commonly hearing loss due to acoustic schwannomas, although symptoms emanating from other intracranial or tumours are not uncommon. The discovery of multiple meningiomas or multiple spinal neurofibromas without cutaneous lesions should initiate a search for acoustic schwannomas even when the patient has normal hearing.
Key wordsNeurofibromatosis NF2 Meningioma, multiple MRI
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- 1.National Institutes of Health Consensus Development Conference (1988) Conference statement. Neurofibromatosis. Arch Neurol 45:575–578Google Scholar
- 2.Seizinger BR, Rouleau GA, Ozelius LJ et al (1987) Genetic linkage of von Recklinghausen neurofibromatosis to the nerve growth factor receptor gene. Cell 49:589–594Google Scholar
- 3.Barker D, Wright E, Nguyen K et al (1987) Gene for von Recklinghausen neurofibromatosis is in the pericentric region of chromosome 17. Science 236:1100–1102Google Scholar
- 4.Wertelecki W, Rouleau G, Superneau D et al (1988) Neurofibromatosis 2: clinical and DNA linkage studies of a large kindred. N Engl J Med 319:278–282Google Scholar
- 5.Rouleau G, Wertelecki W, Haines J et al (1987) Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature 329:246–248Google Scholar
- 6.Aoki S, Barkovich J, Nishimura K et al (1989) Neurofibromatosis types 1 and 2: cranial MR findings. Radiology 172:527–534Google Scholar
- 7.Bognanno J, Edwards M, Lee T, Dunn D, Roos K, Klatte E (1988) Cranial MR imaging in neurofibromatosis. AJR 151:381–388Google Scholar
- 8.Cammarata C, Deveikis J, Schellinger D, Patronas N, Stull M (1990) Neuroradiology case of the day. Neurofibromatosis 2. AJR 154:1337–1338Google Scholar
- 9.Martuza R, Eldridge R (1988) Neurofibromatosis 2. N Engl J Med 368:684–688Google Scholar
- 10.Wayman J, Dutcher P, Manzione J, Nelson C, Kido D (1989) Gadolinium-DTPA-enhanced magnetic resonance scanning in cerebellopontine angle tumors. Laryngoscope 99:1167–1170Google Scholar
- 11.Clifton A, Phelps P, Lloyd G (1991) The investigation of acoustic neuroma. Clin Radiol 44:232–235Google Scholar