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Andrea L. Halliday, Raymond A. Sobel and Robert L. Martuza

T he neurofibromatoses are genetic disorders that give rise to tumors of the nerve sheath, glia, and meninges. Neurofibromatosis type 1, previously known as von Recklinghausen's or peripheral neurofibromatosis, is characterized by multiple café-au-lait spots, neurofibromas, axillary freckling, and iris hamartomas. It is an autosomal-dominant disorder affecting about one in 4000 individuals with high penetrance but variable expression. 7, 10, 12, 14, 18, 21, 22 Neurofibromatosis type 2, previously known as bilateral acoustic or central neurofibromatosis, is

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D. Baldwin, T. T. King, E. Chevretton and A. W. Morrison

material on chromosome 22 is present in patients with unilateral tumors 24 and in those with bilateral tumors. 15, 23 These changes were also seen in meningiomas and spinal tumors occurring in patients with central neurofibromatosis, or neurofibromatosis type 2. 25 Although familial association has been widely investigated, 4, 6, 17, 28 only a minority of cases encountered clinically give a family history, as most cases are the result of a spontaneous mutation. Despite important advances in understanding the genetics of this disease and improvements in diagnostic

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stimulation during surgery Blair Calancie K. John Klose Sylvia Baier Barth A. Green June 1991 74 6 897 904 10.3171/jns.1991.74.6.0897 Nitrogen mustard, vincristine, procarbazine, and prednisone as adjuvant chemotherapy in the treatment of medulloblastoma Jeffrey P. Krischer Abdelsalem H. Ragab Larry Kun Tae H. Kim John P. Laurent James M. Boyett Cornelius J. Cornell Michael Link Annette Ridolfi Luthy Bruce Camitta June 1991 74 6 905 909 10.3171/jns.1991.74.6.0905 Bilateral cerebellopontine angle tumors in neurofibromatosis type 2 D. Baldwin T. T. King E

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Marcos Tatagiba, Madjid Samii, Cordula Matthies, Mowaffak El Azm and Robert Schonmayr

anatomically preserved. Of these, 14 “ears” were already deaf preoperatively and, in a further 25 cases, pre- and/or postoperative CT examinations were not adequate to permit evaluation of the bony labyrinth. In three patients with neurofibromatosis type 2, the bilateral acoustic neurinomas were removed with cochlear nerve preservation on both sides. Consequently, a total of 186 patients (189 “ears”) were evaluated in this study. All had functional preoperative hearing and adequate pre- and postoperative CT examinations. Fig. 1. Left: Artist's illustration showing

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Yoshiaki Takamiya, Robert M. Friedlander, Harold Brem, Amy Malick and Robert L. Martuza

Patients with neurofibromatosis type 2 present with multiple nervous system tumors including bilateral acoustic neuromas, spinal schwannomas, neurofibromas, neurofibrosarcomas (malignant schwannomas), ependymomas, and meningiomas; 20, 21, 23 these patients are ideal subjects for the development of preventive therapeutic modalities because patients with this disease can often be identified prior to the onset of tumor growth. 27 Despite advances in understanding the pathogenicity of neurofibromatosis, no effective way of treating or preventing the development of these

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Juha Jääskeläinen, Anders Paetau, Ilmari Pyykkö, Göran Blomstedt, Tauno Palva and Henry Troupp

the risk that the remnant may grow large enough to cause symptoms. 2, 5, 10, 12 Perhaps surgical teams advocating different strategies should store the facial appearances of their patients on videotape or disk; then, in another 10 or 15 years we will be able to compare results in different series, with some semblance of objectivity, in order to design an optimum treatment strategy. References 1. Baldwin D , King TT , Chevretton E , et al : Bilateral cerebellopontine angle tumors in neurofibromatosis type 2. J Neurosurg

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Madjid Samii, Matteo M. Migliori, Marcos Tatagiba and Ramesh Babu

. Fifteen patients who were diagnosed as having neurofibromatosis Type 2 were excluded from this study. Twelve patients had a solitary trigeminal schwannoma. There were four women and eight men, with an average age of 42 years (range 18 to 62 years). The clinical records, radiological examinations, and operative notes of these 12 patients were studied retrospectively. Pre- and postoperative computerized tomography (CT) was obtained in all patients and seven underwent four-vessel cerebral angiography. In the eight most recent cases, magnetic resonance (MR) imaging

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Akira Kuroki and Aage R. Møller

cochlear nuclei mainly lie beneath the lateral recess in the area of the triangle, which consists of the long axis of CN VIII, the long axis of CN IX, and the foramen of Luschka. The translabyrinthine approach to the foramen of Luschka has been used for implanting a device to stimulate the cochlear nuclei in deaf patients whose deafness is caused by neurofibromatosis type 2. 1 We found that this approach provides only a limited operative view to the foramen of Luschka, as has also been reported by Brackmann, et al. 1 Exposure of the lateral recess may be more

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Bernard George and Guillaume Lot

.25 0.25 ± 0.11  hourglass 2.53 ± 0.14 0.89 ± 0.26 surgical approach  midline posterior 2.48 ± 0.15 0.84 ± 0.26  lateral (posterior or 2.47 ± 0.23 0.53 ± 0.13   anterior) In this series, 12 patients exhibited signs of neurofibromatosis type 1 (von Recklinghausen's disease) and one had evidence of neurofibromatosis type 2. The mean age of this group with neurofibromatosis was 32 years and there were four women and eight men. The mean delay before diagnosis for this group was 16 months. The clinical signs

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Madjid Samii, Ramesh P. Babu, Marcos Tatagiba and Abolghassem Sepehrnia

the cases is outlined. Clinical Material and Methods Patient Population Between 1986 and 1992, 23 patients with schwannomas of the jugular foramen and hypoglossal canal were operated on at our institution. These 23 cases accounted for 1% of 2200 intracranial tumors, and 4% of 570 intracranial schwannomas resected during the same time period in our department. The ratio of jugular foramen schwannomas to acoustic schwannomas during this time was 1:24. Five patients who were diagnosed as having neurofibromatosis type 2 were excluded from this study, as were