A novel mutation of the GTP-cyclohydrolase I gene in a patient with hereditary progressive dystonia/dopa-responsive dystonia |
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Authors: | Y Imaiso T Taniwaki T Yamada T Yoshimura M Hirano S Ueno N Kaneda J Kira |
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Affiliation: | Department of Anesthesiology, Albany Medical College, New York 12208-3479, USA. robertk@mail.amc.edu |
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Abstract: | PURPOSE: To evaluate re-treatment with an additional course of low-dose irradiation in patients with progressive or recurrent choroidal neovascular membranes (CNVMs). MATERIALS AND METHODS: Ten patients who had received 14 Gy of external-beam radiation therapy in seven fractions for subfoveal CNVMs were found to have recurrent or persistent neovascularization at follow-up. They received an additional 15 Gy of radiation therapy administered in five daily fractions with a standard lens-sparing technique. Before reirradiation, visual acuity ranged from 20/80 to counting fingers. The median time between radiation courses was 6.5 months (range, 2-16 months). After re-treatment, the patients were followed up with angiography and visual field testing. The median follow-up was 18.5 months (range, 12-21 months). RESULTS: Eight of 10 patients (80%) maintained their visual acuity at 1 year and three of seven (43%) at 18 months. Visual acuity was stabilized in five of the 10 patients at their last follow-up. No acute or late side effects of irradiation were noted. CONCLUSION: Additional radiation therapy in selected patients with CNVMs who have failed to benefit from previous irradiation is well tolerated and appears to stabilize the disease process in a substantial proportion of these patients. |
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