Nonarteritic ischemic optic neuropathy. The impact of tobacco use.

BACKGROUND: Numerous associations to anatomic variation and systemic vascular disease have been made to anterior ischemic optic neuropathy (AION) but exogenous agents have not been emphasized. The authors studied the effect that smoking had in the development of AION. The relevance of other intraocular and systemic vascular disease to AION also is discussed. METHODS: Over a 10-year period (January 1980-May 1990), nonarteritic AION was diagnosed in 148 patients, 137 of whom were included in this analysis. FINDINGS: Of the 137 patients identified with nonarteritic AION, 28 smokers were statistically younger, at 51 years of age, compared with 83 nonsmokers whose mean age was 64 years (P = 0.005). CONCLUSIONS: Cigarette smoking is an important risk factor in the development of AION. Cessation of smoking appears to reduce the risk of AION to that of the nonsmoking population.

Changes in adult metabolism of aflatoxin B1 in rats neonatally exposed to diethylstilbestrol. Alterations in alpha-class glutathione S-transferases.

Neonatal exposure of rats to xenobiotics has been shown to produce long-term alterations in hepatic enzyme activities and in levels of DNA adducts following carcinogen exposure. We exposed newborn male rats to diethylstilbestrol (DES), pregnenolone-16 alpha-carbonitrile, 7,12-dimethylbenz[a]anthracene or phenobarbital on days 1, 3 and 5 of age. At five months of age, males were injected with 1 mg/kg of [3H]aflatoxin B1 (AFB1), killed after 2 h and examined for AF-DNA adduction in the liver. Males neonatally exposed to DES showed a 35% decrease in DNA adduction levels. Analysis of the adducted DNA bases failed to show any changes in relative proportions of individual adducts in the DES samples compared to controls. Hepatic glutathione concentrations were unchanged. However, Western blot analysis of alpha-class glutathione S-transferases (alpha GST), enzymes known to inactivate the toxic AFB1-8,9-epoxide, showed a 2-fold increase in subunit levels in the DES-treated males, suggesting that the detoxifying activity of the cytosol may have been increased. To confirm this, in vitro tests were undertaken using butylated hydroxyanisole (BHA) induced mouse microsomes to activate [3H]AFB1 in the presence of treated cytosol and GSH. Analysis of metabolites by HPLC showed that DES-treated males formed 245% of the AFB-SG conjugate relative to vehicle controls. These results indicate that neonatal DES treatment resulted in long-term changes in basal alpha GST levels and suggest that these changes were responsible for lower levels of DNA adduction following adult exposure to AFB1.

Neuro-ophthalmic findings in progressive supranuclear palsy.

We studied 104 patients with progressive supranuclear palsy (PSP), 38 of whom were examined by both a neurologist and a neuro-ophthalmologist. Neuro-ophthalmic findings that may help differentiate PSP from Parkinson's disease include vertical supranuclear ophthalmoparesis and fixation instability. Eyelid abnormalities, particularly lid retraction, blepharospasm, and "apraxia" of eyelid opening and closure, were important distinguishing signs. Although downgaze palsy is felt to be the clinical hallmark of PSP, upgaze and downgaze were equally affected at the time of diagnosis in our patients.

Bilateral internuclear ophthalmoplegia in a patient with Wernicke's encephalopathy.

The most common cause of bilateral internuclear ophthalmoplegia is multiple sclerosis. Wernicke's encephalopathy has been reported as a cause of unilateral internuclear ophthalmoplegia but not of bilateral internuclear ophthalmoplegia. In this report, we present the case of a patient with a history of alcohol abuse and acute onset of bilateral internuclear ophthalmoplegia whose clinical course and diagnostic studies are most consistent with a diagnosis of Wernicke's encephalopathy.

Opsoclonus-myoclonus with Beckwith-Wiedemann syndrome and hepatoblastoma.

The association between Beckwith-Wiedemann syndrome and hepatoblastoma is well established and relatively commonplace. The occurrence of opsoclonus-myoclonus syndrome in individuals with occult neoplasia is also well documented. However, the development of opsoclonus-myoclonus syndrome in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma has not been reported previously. The list of underlying causes of opsoclonus-myoclonus syndrome should be expanded to include hepatoblastoma, particularly in any child with features suggestive of Beckwith-Wiedemann syndrome.

Familial hemifacial spasm.

Three male patients in successive generations of the same family developed acquired left-sided hemifacial spasm. Magnetic resonance imaging of the proband demonstrated a pontine vertebral artery anomaly near the root of the affected facial nerve. This family and a similar family that was recently presented illustrate several interesting features: (1) hemifacial spasm, from whatever cause, may be autosomal dominant; (2) the spasms involve the same side of the face in affected individuals within an affected family; and (3) the age of onset in familial cases may be younger than in nonfamilial cases.

Venous stasis retinopathy of stenotic or occlusive carotid origin.

Venous stasis retinopathy is an uncommon sign of carotid artery obstruction. It is found in those patients who have very severe stenosis, or complete occlusion, of the arterial lumen. Affected patients usually have advanced multivessel disease with compromise of both carotid arteries. The retinopathy is ipsilateral to the more severely compromised artery; it is caused by progressive, chronic hypoxic injury to retinal tissues, which causes an ophthalmoscopic picture of venous dilation and tortuosity, retinal hemorrhages, macular edema, and eventual neovascular proliferation. The retinal findings are quite similar to those found in diabetic retinopathy, except for unilaterality corresponding to the more obstructed artery and early onset in the retinal midzone rather than the posterior pole. The primary treatment is panretinal photocoagulation to ischemic areas, followed by either medical or surgical management of obstructive carotid disease or carotid endarterectomy alone.

Management of pregeniculate anterior visual pathway gliomas.

Gliomas of the pregeniculate anterior visual pathways comprise about 5% of all intracranial tumors that occur in the first decade of life. Gliomas are the most common primary optic nerve tumor. Diagnosing a glioma of the anterior visual pathways is much easier since the advent of computerized tomography (CT) and magnetic resonance imaging (MRI); the continuing problem is appropriate management. Improved guidelines for management are suggested based on a review of the literature and the authors' experience. These guidelines include both the need to characterize accurately the location of the tumor and its clinical and radiologic progression, and the imperative to employ active intervention only when there is a specific therapeutic goal that can be used for measuring the success of the treatment.

Intraosseous capillary hemangioma of the frontal bone.

A 31-year-old woman with a history of slowly progressive proptosis associated with blepharoptosis was found radiographically to have a lytic lesion of the frontal bone. Complete surgical excision of the bony mass was performed. Histopathologically the tumor was a capillary hemangioma. Follow-up information two years later showed that the patient was alive and well, without clinical evidence of recurrence. Plain x-ray films appear to be more reliable for diagnosis than computed tomography exhibiting the characteristic sunburst pattern of intraosseous hemangiomas.

Computed tomography and magnetic resonance imaging in the diagnosis of inflammatory disease of the optic nerve.

Enlargement of the optic nerve and chiasm as demonstrated by high-resolution CT scanning or magnetic resonance imaging is often assumed to indicate tumor. However, this is not always true. The mode of clinical presentation of the disease process must be correctly applied to the interpretation of the radiographic studies. In the case illustrated here, neuro-ophthalmologic evaluation prevented an unnecessary biopsy in a patient whose presentation was atypical for anterior visual pathway glioma and whose clinical course was otherwise compatible with optic neuritis.

Progressive blindness caused by metastatic occult signet-ring cell gastric carcinoma.

We described a metastatic signet-ring cell gastric adenocarcinoma in a 60-year-old woman who complained of progressive visual loss. The discovery of signet-ring cells in the cerebrospinal fluid established the diagnosis of mucus-secreting adenocarcinoma. Histologic study of an ulcerated lesion in the lesser curvature of the stomach established the diagnosis of primary gastric adenocarcinoma. When confronted by a patient with impaired function of the cerebral cortex, cranial nerves, and spinal nerve roots that results in progressive neurologic deterioration, leptomeningeal carcinomatosis should be considered and repeated cytologic examinations of spinal fluid should be obtained.

Chiasmal apoplexy: hemorrhage from a cryptic vascular malformation in the optic chiasm.

We describe three patients with acute chiasmal visual field loss caused by hemorrhage within an intrachiasmal arteriovenous malformation. One patient had five episodes; vision improved three times without treatment and twice after surgical decompression. Surgical intervention restored vision in two of the three patients. The role for surgery is indefinite, but may be indicated if the visual defect remains static for 1 week or progresses. Decompression is likely to be most effective if done early. The approach to the optic chiasm should be transcranial, not transsphenoidal. A radiologically normal sella turcica favors the diagnosis of chiasmal apoplexy in the acute chiasmal syndrome.

Nuclear magnetic resonance diagnosis of an anaplastic astrocytoma.

A patient presented with an 8-month history of a progressive left homonymous visual field deficit, left hemiparesis, and a left thalamocortical sensory deficit that was not detectable by repeated conventional neurodiagnostic evaluations. Proton nuclear magnetic resonance (NMR) imaging revealed a right parietal lesion characterized by a prolonged T2 (spin-spin relaxation time). At surgery, the mass proved to be an anaplastic astrocytoma. NMR appears to be more sensitive than x-ray computerized tomography scanning in some patients with malignant gliomas and offers the clinician an additional probe with which to evaluate these patients.

Epithelioid angiosarcoma of the orbit presenting as Tolosa-Hunt syndrome. A clinicopathologic case report with review of the literature.

The clinical diagnosis of Tolosa-Hunt syndrome was first considered in a 66-year-old man with facial pain and diplopia. A complete neuroradiologic evaluation as well as an oncologic work-up yielded normal results. Several courses of oral prednisone provided no significant benefit. Within a year the patient became clinically worse and a CT scan disclosed an abnormal area of enhancement at the left orbital apex. An orbital exploration was performed elsewhere and a histologic diagnosis of myositis was obtained. Because of further worsening the patient was re-evaluated 3 months later and a CT scan showed a mass in the left orbital apex and superior orbital fissure. A second orbital exploration was performed and a sausage-shaped mass encompassing the optic nerve was excised. By light microscopy a poorly differentiated malignant tumor was infiltrating the orbital tissues with areas of intra- and perineural invasion. The tumor cells were arranged in strands and tubules with a definite tendency to form lumens that often contained red blood cells. Electron microscopic studies disclosed features consistent with a neoplasm of endothelial cell origin displaying a polarized basal lamina and rare micropinocytotic vesicles on the luminal side. The presence of multiple, slender microvilli and sometimes tonofilaments as well as desmosomes were interpreted as epithelioid metaplasia of an angiosarcoma.

Managing menacing meningioma.

The role of irradiation therapy in the management of meningiomas involving the anterior visual system is under active clinical investigation. This report documents the value of radiotherapy in a patient with a histologically verified psammomatous tuberculum sellar meningioma and presents a 15-year postsurgical follow-up. The patient experienced a return of vision from 11/200 to 20/60 in his only seeing eye following 5,000 rads to the intracanicular and intracranial portions of right optic nerve. He is able to practice his profession 3 years following irradiation therapy. The classification of optic nerve involvement by meningioma is discussed.