Schilder's (1912) disease. Total cerebral blindness due to acute demyelination.

A 51-year-old woman became blind from involvement of both occipital lobes with a confluent lesion demonstrated by computed tomographic scan. Biopsy showed demyelination consistent with Schilder's 1912 variant of diffuse sclerosis. No cytoplasmic inclusions were found on electron microscopy.

Treatment of temporal arteritis with ocular involvement.

A 78-year-old white woman had catastrophic visual loss in one eye due to temporal arteritis. Despite treatment with doses of oral corticosteroids high enough to normalize the Westergren erythrocyte sedimentation rate, she experienced progressive retinal ischemia with visual loss in the second eye. The use of 1,000 mg of pulsed intravenous methylprednisolone every 12 hours restored her vision. Brief hospitalization of patients with arteritic ischemic optic neuropathy for treatment with intravenous methylprednisolone may offer a significant chance of visual recovery of the involved eye and provide optimal protection to the uninvolved eye.

Bilateral optic nerve sheath meningiomas.

A 35-year-old man was initially seen following the incidental discovery of swollen optic disc in both eyes. Over a subsequent 15-year period, the patient experienced an inexorable loss of vision in both eyes, characterized by normal neuroradiologic findings and the presence of opticocillary shunt vessels on both discs. A craniotomy ultimately demonstrated the presence of bilateral optic nerve sheath meningiomas that appeared to have arisen multifocally.

Ocular reticulum cell sarcoma.

In four of eight cases of reticulum cell sarcoma with ocular involvement diagnosis was made by craniotomy when the signs of a brain tumor developed. A fifth case had an isolated intraocular tumor. Of the other three, two were diagnosed by vitreous aspiration and one by cerebrospinal fluid cytology. Ocular reticulum cell sarcoma frequently accompanies or precedes brain involvement. In this form of the disease the ocular lesion is usually a tumor cell infiltrate of the retina. The associated retinochoroiditis leads to marked vitreous clouding and eventual retinal detachment and glaucoma may occur. On the other hand, when intraocular reticulum cell sarcoma occurs in association with the systemic lymph node visceral form of the disease, choroidal involvement is the rule. Vitreous aspiration may confirm the diagnosis at an early stage when clinical and laboratory evaluation reveal no disease outside the globe. The retinal tumor foci are radiosensitive and local radiation, although not curative, may restore visual acuity. Immunosuppressive therapy given in low doses for anti-inflammatory effect may exacerbate the disease.

Cryptogenic oculomotor nerve palsies in children.

We examined two cases of isolated, acquired, unremitting oculomotor palsies in children. The results of systemic, neurologic, and neuroradiologic investigations were normal. Both children were observed for more than two years and showed no additional signs or symptoms. Acquired isolated oculomotor palsies in some cases are not necessarily a harbinger of serious disease.

Autoimmune retrobulbar optic neuritis.

Three patients, all women, ranging in age from 26 to 44 years, had acute retrobulbar neuritis and laboratory evidence of a collagen vascular disorder. These patients were treated with intravenously administered "pulse" methylprednisolone, and visual acuity recovered in three of four involved eyes. Continued oral administration of prednisone and other immunosuppressive drugs was necessary to maintain vision.

Accommodation defect in Wilson's disease.

A 22-year-old man with Wilson's disease had blurred vision caused by a defect of accommodation that we believed to be supranuclear in origin.

Bilateral optic atrophy caused by chronic oral ingestion and topical application of hexachlorophene.

A 31-year-old woman ingested hexachlorophene (pHisoHex) over a ten-month period. She developed bilateral optic atrophy with no other systemic evidence of neurotoxicity. This cases supports pathologists' findings that the optic nerve and chiasm are particularly sensitive to the poisonous effects of hexachlorophene.

Disappearing opticociliary shunt vessels and pseudotumor cerebri.

In two patients opticociliary venous shunts occurred in association with pseudotumor cerebri. One patient underwent bilateral optic nerve sheath fenestrations, after which her papilledema resolved, and the opticociliary venous shunts were noted to be markedly decreased in caliber.

Pontine gaze paresis due to traumatic craniocervical hyperextension. Report of two cases.

Pontine gaze paresis is frequently due to tumor of the brain stem. Occasionally it may be caused by inflammation or ischemia. Two cases are reported, each with pontine gaze paresis and other signs of lower brain-stem injury, basal skull fractures, and second cervical vertebra fractures. This pattern of injuries is believed to be the result of craniocervical hyperextension with stretch injury to the brain stem at the junction of the medulla and pons.

Optic neuropathy associated with penicillamine therapy in a patient with rheumatoid arthritis.

Optic neuropathy developed in a patient with rheumatoid arthritis who had been receiving D-penicillamine for about 1 year. An associated finding included a 2+ positive antinuclear antibody test with a titer of 1:320. Optic disc swelling was resolved on high doses of intravenous steroids. The case resembles two previously reported cases of optic neuropathy which occurred in patients with Wilson's disease who were receiving penicillamine.

Acute oculomotor nerve palsy in childhood. Is arteriography necessary?

In the past, angiography was performed in all patients as part of the initial workup for isolated oculomotor paralysis, except patients older than 40 years with pupillary sparing. The pupil-sparing group was not subjected to angiography because of a low probability of cerebral aneurysm. It is believed that the case reported here constitutes the lower age limit (14 years) for documented, isolated oculomotor paralysis due to aneurysm. It is recommended that an angiogram not be a necessary part of the workup of patients 10 years old or younger.

Management of dysthyroid optic neuropathy.

The diagnosis of dysthyoid ophthalmopathy is based on clinical observation of characteristic ophthalmic abnormalities. Proper diagnosis and initiation of treatment should not be delayed because a patient is found to be euthyroid. Although visual problems in dysthyroid ophthalmopathy are uncommon this disorder can result in permanent severe visual loss if optic neuropathy is not detected early and managed properly. Oral corticosteroid therapy and surgical decompression both seem to have a place in the treatment of dysthyroid ophthalmopathy with optic neuropathy.

Amiodarone keratopathy.

Thirty-eight patients received an average of 325 mg of amiodarone per day (range, 100 to 600 mg/day) for an average period of 16 months (range, nine to 30 months). Visible corneal microdeposits developed in all patients. Ninety-five percent of our patients had grade I or grade II keratopathy with no effect on vision. Five percent (5%) had grade III keratopathy with loss of one line of visual acuity and experienced subjective blurring and colored halos. Although there was a relationship of the total cumulative dose of the drug to the density of the corneal microdeposits, there was great variability from patient to patient, which limited the usefulness of this relationship.

Pupil-sparing oculomotor palsies with internal carotid-posterior communicating artery aneurysms.

The charts of 84 patients admitted to the Neurosurgery Service of the Washington University Medical Center between January, 1960, and July, 1981, with aneurysms at or near the junction of the internal carotid and posterior communicating arteries (ICA-PoCA) were reviewed. Special emphasis was placed on the pupillary size and reactivity of 51 patients with oculomotor nerve involvement. Seven initially had normal pupils, representing 8% of the total group and 14% of those presenting with oculomotor palsies. In 4 of these patients pupillary involvement developed within 5 days, and in 1 pupillary involvement developed in 4 months. Pupillary sparing appears to be more common than previously appreciated in patients with ICA-PoCA aneurysms and oculomotor nerve involvement. Patients with acute somatic oculomotor paresis should be observed closely for at least one week for the development of pupillary involvement. Arteriography may be indicated more frequently than previously recommended.

Atypical oculomotor paresis.

A series of patients is presented with partial oculomotor palsies. The accompanying signs of neurologic dysfunction are indicative of a mesencephalic lesion. The peculiar anatomy of the third cranial nerve permits one to infer precise localization of the lesions.

Evaluation of the suprasellar cistern by computed tomography.

The normal suprasellar cistern is a five- or six-pointed, starshaped, fluid-filled structure as demonstrated by computerized axial tomography (CAT). At various levels the normal suprasellar cistern contains the major intracranial vessels and their anastomotic channels, the optic nerves, chiasm, and infundibular stalk. The existence of lesions, either intrinsic structures of or extrinsic structures contiguous to the suprasellar cistern can be detected by their effect on the normal anatomy of the suprasellar cistern or by filling defects produced when studied with metrizamide cisternography.

Optic neuritis in inflammatory bowel disease.

It is not unusual for various ocular diseases to be associated with inflammatory bowel disease. Ocular involvement includes the inflammatory entities of keratitis, conjunctivitis, episcleritis, orbital pseudotumor, iritis and neuroretinitis , as well as those states in which the etiology remains undefined, i.e., scleromalacia perforans, peripheral corneal ulceration and furrow, retinal artery occlusion, and optic neuropathy. We report five cases of optic neuropathy associated with ulcerative colitis to emphasize that this may be the sole ocular manifestation of inflammatory bowel disease.

Autoimmune optic neuropathy: evaluation and treatment.

Fourteen patients, 12 of whom were women, with an age range from 26 to 56 years, presented with progressive or recurrent optic neuropathy, despite conventional doses of corticosteroid, and laboratory evidence of collagen vascular disease. The visual loss was severe and most had an acuity less than 20/200. Megadose corticosteroid therapy improved the vision in 11 of the 12 patients. Continued oral prednisone and cytotoxic drugs were necessary to maintain vision in nine patients. Patients with autoimmune optic neuropathy must be differentiated from cases with idiopathic optic neuritis or multiple sclerosis to facilitate the appropriate therapy.