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We present two cases of delayed optic nerve complications following proton beam radiotherapy. Patient 1 received radiotherapy after surgery for clival chordoma and developed sequential radiation optic neuropathy at 5 and 9 months. Patient 2 developed optic nerve hemorrhage post radiotherapy for optic nerve sheath meningioma. These two cases of blindness demonstrate the risks associated with proton beam radiotherapy.

We report a case of tuberculum sellae meningioma with optic tract edema. Contrary to a prior report on this topic, edema along the optic tract is not only seen in craniopharyngiomas but may be seen (although rarely) in other common parasellar tumors, as in our case of a tuberculum sellae meningioma. The pathogenesis of this edema in meningioma is controversial.

Traumatic chiasmal neuropathy results from injury to the face, sphenoid and clivus. Its pathogenesis remains enigmatic. Because of its close relationship to the pituitary gland, hypothalamus and internal carotid artery, a neuro-ophthalmic evaluation and imaging is needed in such cases. We present a patient who developed traumatic chiasmal syndrome after an automobile accident. Computed tomographic scan showed fracture of the sella turcica. A carotid angiography showed a traumatic pseudoaneurysm of the internal carotid at the base of the skull.

Background: Systemic lupus erythematosus (SLE) is a multisystemic inflammatory disease of unknown etiology. Physiopathology includes small vessel occlusion and auto-antibody production. Neuro-ophthalmic complications can be detected in 10-30% of SLE cases. Further, some patients can present with neuro-ophthalmic complications prior to diagnosis of SLE., Patients and Methods: We report seven cases of neuro-ophthalmic complications of SLE demonstrating lesions from the retina to the brain. The other possible manifestations of SLE will be reviewed., Results: Two cases were not yet diagnosed with SLE when neuro-ophthalmic disease occurred. Anti-double stranded DNA antibodies were detected at a very high titer, suggesting SLE, later confirmed by rheumatologists., Conclusions: Ophthalmologists should be aware of the unusual patient presenting with a neuroophthalmic disorder prior to the diagnosis of SLE. In such cases, autoantibodies should be sought. Antinuclear antibodies are very sensitive but non specific for SLE. Anti-double stranded DNA antibodies are specific for SLE and elevated titer should raise the suspicion of undiagnosed SLE.

Optic neuropathy resulting in permanent visual loss is an infrequent delayed complication of radiation therapy. Hyperbaric oxygen therapy (HBO) has been used to treat such a complication, but its efficacy is controversial. We report a patient who presented with radiation-induced optic neuropathy 17 months after irradiation for a left maxillary antrum melanoma. HBO fully reversed visual loss in the more recently involved eye, and slightly improved vision in the earlier affected eye.

Hodgkin's disease is a rare cause of infiltrative optic neuropathy, which typically evolves late in the disease course. We managed an unusual case of isolated optic neuropathy in a 21-year-old man occurring during clinical remission from Hodgkin's disease. Radiotherapy and treatment with high-dose systemic corticosteroids resulted in dramatic improvement in vision. Even without other evidence of recurrent disease, acute-onset optic neuropathy in a patient with a history of a lymphoproliferative disorder should raise the question of a reemergence of the malignancy.

Three patients with delayed radiation optic neuropathy after radiation therapy for parasellar neoplasms underwent magnetic resonance imaging. The affected optic nerves and chiasms showed enlargement and focal gadopentetate dimeglumine enhancement. The magnetic resonance imaging technique effectively detected and defined anterior visual pathway changes of radionecrosis and excluded the clinical possibility of visual loss because of tumor recurrence.

Six patients with optic nerve sheath meningiomas were studied with gadolinium-diethylenetriaminepentaacetic acid (Gd-DTPA)-enhanced magnetic resonance imaging (MRI) to evaluate intracranial extension. The intraorbital and intracranial tumors were isointense to cortical gray matter on T1-weighted studies without contrast in all patients. After Gd-DTPA, three patients showed mild enhancement of the intraorbital tumor, whereas five of six patients showed vivid enhancement of the intracranial tumor. In four cases, the intracranial extension could not be definitely seen on MRI without Gd-DTPA. Two patients had proton density and T2-weighted studies; in each case, the intraorbital tumor remained nearly isointense. The intracranial tumor was suggested in one patient on T2-weighted studies, but was poorly defined. Gadolinium-DTPA has proved valuable in detecting intracranial extension of optic nerve meningiomas that are not well visualized on standard MRI without contrast.

The ophthalmoscopic findings in a series of 14 eyes with radiation-induced optic neuropathy are described. Typically, the entity manifests acutely as disc swelling with surrounding exudate, hemorrhages, and subretinal fluid. Optic atrophy may ensue in the later stages. Similar to radiation retinopathy, radiation optic neuropathy also seems to occur secondary to vascular damage. In contrast to visual loss secondary to radiation retinopathy, the visual acuity in certain eyes with decreased vision attributable to radiation optic neuropathy may improve over a period of several months.

Sarcoid optic neuropathy without fundus lesions is unusual. We present a case of optic neuropathy with spontaneous remission in one eye that paralleled the clinical course of demyelinative acute optic neuritis. The fellow eye had disc swelling with normal visual acuity but with a large blind spot and distended optic nerve sheaths seen on ultrasonography. The diagnosis of sarcoidosis was made by characteristic chest roentgenographic findings and confirmed by skin biopsy.

We performed visual contrast sensitivity testing with Regan's low-contrast letter charts on 30 patients with optic neuropathies and good Snellen visual acuity. Results of this testing were compared with color vision and the presence of an afferent pupillary defect. Regan's low-contrast letter charts were found to be 93% sensitive for detecting subtle optic neuropathies, with the apparent false-negative findings being readily explainable. Color vision testing was abnormal in only 49% of eyes with known optic neuropathies.

Four patients with radiation-induced optic neuropathies were treated with hyperbaric oxygen. They had received radiation therapy for treatment of pituitary tumors, reticulum cell sarcoma, and meningioma. Two presented with amaurosis fugax before the onset of unilateral visual loss and began hyperbaria within 72 hours after development of unilateral optic neuropathy. Both had return of visual function to baseline levels. The others initiated treatment two to six weeks after visual loss occurred in the second eye and had no significant improvement of vision. Treatment consisted of daily administration of 100% oxygen under 2.8 atmospheres of pressure for 14-28 days. There were no medical complications of hyperbaria. While hyperbaric oxygen is effective in the treatment of radiation-induced optic neuropathy, it must be instituted within several days of deterioration in vision for restoration of baseline function.

We examined three patients who developed optic neuropathies while undergoing chronic hemodialysis. One patient developed severe bilateral deterioration of vision, but recovered totally four weeks after discontinuing deferoxamine chelation therapy. Another patient had bilateral visual loss associated with chronic papilledema of idiopathic increased intracranial pressure. A third showed atypically severe consecutive anterior ischemic optic neuropathy. The latter two patients showed little improvement with high-dose intravenous methylprednisolone therapy combined with more vigorous hemodialysis. These cases, in addition to those previously described, underscore the heterogeneity of optic nerve disease in patients with uremia.

The etiology of anterior ischemic optic neuropathy (AION), when not associated with giant cell arteritis, is usually unknown. Clinical, pathologic, and experimental studies have not determined a cause. The optic disc appearance in both the involved and normal fellow eye was studied in 51 patients with acute nonarteritic AION. The number of discs (both involved and fellow) without a physiologic cup was significantly greater than would be expected from normal population studies. The etiology of nonarteritic AION may be related to the anatomic configuration of the optic nerve.

Of 196 patients with anterior ischemic optic neuropathy, 169 had the nonarteritic form and 27 had the arteritic type. Visual acuities were 20/40 or better in 83 of 184 eyes with nonarteritic anterior ischemic optic neuropathy but only eight of 45 eyes with the arteritic type. We found systemic disease associations for hypertension and diabetes mellitus only for patients with nonarteritic anterior ischemic optic neuropathy who were between 45 and 64 years of age. After a mean follow-up period of five years, 92 nonarteritic patients showed no changes in the first affected eye; there was eventual involvement of the second eye in 20 patients.