Norman J. Schatz

Clinical Profile And Long-Term Implications of Anterior Ischemic Optic Neuropathy

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.

Hypopituitarism following radiation therapy of pituitary adenomas

Pituitary function was evaluated before and after supervoltage radiotherapy for a pituitary adenoma in 35 patients; 22 had had prior surgical treatment, and 13 had not. For comparison, pituitary function was also followed concurrently in another 10 patients whose pituitary adenomas were treated surgically, but who did not receive subsequent radiotherapy. Following radiotherapy, deficiencies of adrenal, thyroid, and gonadal function developed in 67, 55, and 67 percent of the patients who had previously been treated surgically during a mean of 4.2 years of observation, and in 55, 15, and 50 percent of the patients who did not have prior surgery during the subsequent five years. In contrast, in patients who had previously been treated surgically and who did not receive radiation, deficiencies of these hormones developed in only 13, 13, and 0 percent during four years of observation. It is concluded that supervoltage radiotherapy for pituitary adenoma, especially when preceded by surgical treatment, frequently produces, during the ensuing four to five years, deficiencies of pituitary hormones that had been secreted normally prior to irradiation.

Radiation Optic Neuropathy

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.

Cerebrospinal fluid diversion procedures in pseudotumor cerebri

We reviewed the efficacy of CSF diversion for pseudotumor cerebri (PTC) in patients from six different institutions. Thirty-seven patients underwent a total of 73 lumboperitoneal shunts and nine ventricular shunts. Only 14 patients remained “cured” after a single surgical procedure. The average time between shunt insertion and shunt replacement was 9 months, although 64% of shunts lasted less than 6 months. Shunt failure (55%) and low-pressure headaches (21%) were the most common causes for reoperation. The vision of most patients improved (13) or stabilized (13) postoperatively. However, three who had initially improved subsequently lost vision. Six had a postoperative decrease in vision. Two patients improved in one eye but worsened postoperatively in the other. Four lost vision despite apparently adequate shunt function. Shunt failure with relapse of PTC occurred as late as 7 years after insertion. CSF diversion procedures have a significant failure rate as well as a high frequency of side effects.

Alzheimer's Disease with Prominent Visual Symptoms: Clinical and Metabolic Evaluation

The authors examined eight patients with dementia of the Alzheimers type (DAT), five with prominent visual symptoms early in the illness (VS) and three with no visual symptoms (NVS). Results of neuro-ophthalmologic examinations on VS patients showed relatively consistent abnormalities in figure copying, color vision tested by isochromatic plates, and stereopsis. Cerebral glucose metabolism determined by 18F-fluoro-2-deoxyglucose positron emission tomography (PET) was unchanged in primary visual cortex of VS and NVS patients compared with 12 normal volunteers of similar age and sex. Glucose metabolism in VS patients was decreased by 45 and 34% in left and right visual association cortex (P less than 0.01 and P less than 0.05, respectively) and 34 and 37% in left and right inferior parietal cortex (P less than 0.05) compared with controls; NVS patients had no significant metabolic alteration in these areas. Symptoms, physical examination, and metabolic imaging imply that these patients are a heterogenous but distinct clinical subgroup of DAT often with mild dementia who have visual symptoms due primarily to visual agnosia.

The Syndrome of Paradoxical Worsening of Dural-Cavernous Sinus Arteriovenous Malformations

Three patients with dural arteriovenous malformations contiguous with the cavernous sinus had marked worsening of their neuro-ophthalmic symptoms. Such clinical deterioration has been attributed traditionally to increased blood flow through the malformations into the superior ophthalmic vein. However, arteriography in all three patients and magnetic resonance imaging (MR) in two patients demonstrated thrombosis of the entire superior ophthalmic vein. All patients subsequently underwent spontaneous, complete resolution of symptoms. This report describes a clinical syndrome of paradoxical worsening of cavernous sinus malformations and indicates that MR will aid in the management of these vascular malformations.

Hyperbaric Oxygen in the Treatment of Radiation-induced Optic Neuropathy

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.

High-dose Methylprednisolone and Acetazolamide for Visual Loss in Pseudotumor Cerebri

We treated four patients who had acute, severe visual loss associated with pseudotumor cerebri with intravenous methylprednisolone (250 mg four times per day) for five days followed by an oral taper, in combination with acetazolamide and ranitidine. In addition to high-grade disk edema, one patient had serous detachment of both maculas and lipid deposition, one had a unilateral macular star, and one had a monocular branch retinal artery occlusion. These three patients experienced rapid and lasting improvement in visual acuity, visual field, papilledema, and symptoms. Vision of the fourth patient did not improve, requiring optic nerve sheath fenestration for chronic papilledema. Transient acne developed in one patient. This regimen is a safe, effective treatment of acute, severe visual loss associated with florid papilledema of pseudotumor cerebri. Lack of immediate improvement is an indication for optic nerve sheath decompression.

Delayed radiation injury to the retrobulbar optic nerves and chiasm : clinical syndrome and treatment with hyperbaric oxygen and corticosteroids

Thirteen patients with delayed radiation injury to the optic nerves and chiasm were treated with hyperbaric oxygen (HBO) and corticosteroids. These patients experienced painless, abrupt loss of vision in one (6 patients) or both (7 patients) eyes between 4 and 35 months after receiving radiation doses of at least 4500 cGy to the region of the chiasm. Diagnostic evaluation including neuro-imaging and lumbar puncture showed no recurrent tumor and no other cause for visual loss. No patients vision improved during treatment or follow-up lasting between 1 and 4 years. There were no serious complications of treatment.

The cupped disc: Who needs neuroimaging?☆

OBJECTIVE To determine the incidence of positive neuroradiologic studies in consecutive patients with glaucoma associated with normal intraocular pressure and to compare the psychophysical and clinical characteristics of these eyes with eyes with disc cupping associated with intracranial masses. DESIGN Retrospective case-controlled study. PARTICIPANTS Fifty-two eyes of 29 patients with glaucoma associated with normal intraocular pressure and 44 eyes of 28 control patients with compressive lesions were reviewed. INTERVENTION The medical records of consecutive glaucoma patients with normal intraocular pressure who underwent brain magnetic resonance imaging or computed tomography scanning as part of a diagnostic evaluation between January 1, 1985, and July 1, 1995, were reviewed. A masked reading of optic nerve photographs and visual fields was performed by one observer. A similar analysis was performed on a control group of consecutive patients with nonglaucomatous optic nerve cupping with known intracranial mass lesions. MAIN OUTCOME MEASURES The neuroradiologic findings, clinical characteristics, optic nerve head appearance, and patterns of visual field loss were compared between groups. RESULTS None of the patients diagnosed with glaucoma had neuroradiologic evidence of a mass lesion involving the anterior visual pathway. Compared to control subjects, patients with glaucoma were older (P = 0.0001), had better visual acuity (P = 0.002), greater vertical loss of neuroretinal rim tissue (P = 0.0001), more frequent optic disc hemorrhages (P = 0.01), less neuroretinal rim pallor (P = 0.0001), and more nerve fiber bundle visual field defects aligned at the horizontal midline (P = 0.0001). Visual acuity less than 20/40, vertically aligned visual field defects, optic nerve pallor in excess of cupping, and age younger than 50 years were 77%, 81%, 90%, and 93% specific for nonglaucomatous cupping associated with compressive lesions, respectively. CONCLUSIONS Anterior visual pathway compression is an uncommon finding in the neuroradiologic evaluation of patients with a presumptive diagnosis of normal-tension glaucoma. Younger age, lower levels of visual acuity, vertically aligned visual field defects, and neuroretinal rim pallor may increase the likelihood of identifying an intracranial mass lesion.