Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"

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Atypical Optic Nerve Affection with Unexpected Outcome

Demmler A.1, Wabbels B. K.1, Bloß H. G.2, Woenckhaus M.3, Lorenz B.1
1Dept. of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, 2Dept. of Neurosurgery, 3Institute of Pathology, University of Regensburg

Purpose: Gliomas of the optic nerve account for about 1% of all intracranial neoplasms. In 85% to 90% children are affected, especially in association with neurofibromatosis type 1. In contrast, malignant optic nerve glioma in adults is very rare. Hoyt described the first cases in 1973. In his report the typical symptom was rapid vision loss in middle-aged men, initially with signs of optic neuritis. All patients deceased within one year. Until now, 40 cases of malignant optic nerve gliomas have been reported.
Results: A 61 year old woman presented with visual loss OD and lower altitudinal visual field defect. Within 8 days, visual acuity decreased from 0.3 to light perception. MRI showed intraorbital swelling of the right optic nerve. The unilateral affection, papilledema with haemorrhages, the altitudinal visual field defect combined with retrobulbar pain and the signs in the MRI seemed consistent with the diagnosis of optic neuritis. Two months later, proptosis, ptosis, exotropia and reduced ocular motility of the right eye had developed  compatible with a slowly progressive oculomotor nerve palsy. After a new MRI an optic nerve biopsy was undertaken. As rapid section diagnosis was suspicious of optic nerve gliom

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