Search

We examined two patients who awoke with profound bilateral visual loss after operations under general anesthesia. Their fundi, initially normal, later showed bilateral optic atrophy. Neither patient showed other neurologic deficits, although one demonstrated radiologic evidence of a small cerebral infarction in the deep white matter. These patients probably suffered intraoperative infarction of the retrobulbar segments of both optic nerves, producing posterior ischemic optic neuropathy. Profound systemic hypotension may have been a contributing factor in one patient, the use of the pump-oxygenator in the other, and anemia in both.

We examined three patients with classic findings of familial dysautonomia (Riley-Day syndrome) whose visual impairment was associated with optic atrophy. The presence of an optic atrophy in familial dysautonomia is indicative of central nervous system involvement, at least in these cases. Each of these patients was first noted to have visual impairment after the first decade. The late onset of optic atrophy may partly explain its apparent rarity. Since the life span of patients with familial dysautonomia is increasing, optic atrophy may be more commonly recognized in the future.

• We describe a simple device used to compare the sense of brightness in the two eyes. An investigation of brightness-sense with this device in normal persons and patients with optic nerve disease, maculopathies, cataract, and factitious visual impairment shows that brightness testing can provide useful information supplementary and complementary to conventional clinical and laboratory investigations. Impairment of brightness-sense was a consistent finding among patients with optic neuropathies and, in some cases, brightness testing appeared to be more sensitive than other techniques in establishing the presence of disease. Brightness-sense was also used to monitor the course of the patient's illness. Abnormalities were also found among some patients with unilateral maculopathies, but significant intereye differences in brightness-sense were not encountered among normal persons or patients with cataracts. Patients with factitious visual loss gave variable responses, and the nature of their disorder could be identified by the inconsistency of their responses.

• Impaired visual function in Alzheimer's disease (AD) could result from either precortical or cortical lesions, or both. In a parallel psychophysical study of visual function in AD, we found that contrast sensitivity function, color vision, stereoacuity, and back-ward masking were impaired relative to the performance of age-matched control subjects, whereas performance on a critical flicker fusion test was normal. The intent of the present study was to determine whether abnormalities of the retinocalcarine pathway contribute to visual dysfunction. We performed neuro-ophthalmological examinations on 38 patients with AD; from this group, 25 received additional psychophysical testing and 13 underwent electrophysiological testing. Clinical neuro-ophthalmological examinations, full-field electroretinograms, focal electroretinograms, and pattern visual evoked potentials were normal in all patients tested. There was no evidence of retinocalcarine abnormality specific to AD. We conclude that the visual impairment experienced by some patients with AD primarily results from involvement of the visual association cortices rather than from precortical damage, at least before the end stage of the disease.

• Contrary to statements in the literature, youth does not seem to confer protection from visual loss in pseudotumor cerebri. We examined five children and adolescents who suffered permanent loss of visual acuity or field in association with this disorder. All of them had impairment at the time of diagnosis, and two of the five developed further visual failure while under observation. Children and adolescents with pseudotumor should be kept under the same close ophthalmic surveillance as has been advocated for adults with this disease.

• Altitudinal visual field defects were discovered in four young women on routine eye or general physical examinations. This led to extensive investigations because three of them were falsely presumed to have acquired disorders of the central nervous system. In each case the defect appeared to result from congenital hypoplasia of the optic nerve associated with maternal diabetes mellitus. Congenital optic nerve hypoplasia should be included in the differential diagnosis of visual field defects even when they are discovered in adults. Hypoplasia consequent to maternal diabetes mellitus is apt to be associated with excellent visual acuity and is usually an isolated defect. The female offspring of diabetic mothers may be at greater risk than the male offspring for the development of this anomaly.

• Five young men had painless, progressive, bilateral loss of central vision with late optic atrophy. One had a family history of Leber's optic neuropathy. After being functionally blind for four to 21 months, spontaneous improvement began leading to remarkable recovery of vision in all patients. We propose that these are cases of Leber's optic neuropathy in which an unusual capacity for recovery may have been related to a lack of microvascular abnormalities in the fundi.

• Nine patients with the syndrome of juvenile diabetes mellitus and optic atrophy exemplified the wide range of manifestations of this syndrome. The disease may occur as a recessively inherited or sporadic disorder and tends to have multisystem involvement. Hearing loss, diabetes insipidus, and evidence of cerebellar or central vestibulo-ocular dysfunction are common.

Bilateral uveitis was the initial manifestation of a limited form of Wegener granulomatosis in a 47-year-old man. Diagnosis was established by biopsy of a pulmonary nodule. Subsequently, evidence of lesions developed in the patient's central and peripheral nervous systems, but renal lesions were absent. Pulmonary lesions regressed on therapy with azathioprine and prednisone. Blindness ultimately resulted from retinal phlebitis, vitreous hemorrhages, and neovascular glaucoma.(JAMA 233:868-871, 1975)

In four of six patients with clinical optic chiasmal neuritis, MRI demonstrated abnormalities of the chiasm. Optic chiasmal neuritis may be the initial manifestation of multiple sclerosis, a reflection of established CNS demyelination, or an isolated clinical finding.

Three untreated patients with presumed chiasmal gliomas had spontaneous visual improvement, although serial magnetic resonance imaging in two of the patients showed the lesions to be unchanged. Two patients had evidence of neurofibromatosis. Three other patients with similar disease courses have been documented in the medical literature. These cases demonstrate that conservative management of optic chiasmal gliomas may sometimes be associated with favorable outcomes.

• This report summarizes the details of 33 personal cases of the important but uncommon entity of posttraumatic optic neuropathy, seen during an 11-year interval. Victims were predominantly young males, and bicycles proved the most common source of injury. Severity of visual loss correlated poorly with level of consciousness and presence of craniofacial fractures. Optic canal fractures were found in only seven of the patients. Vision improved in five of 25 untreated patients, one of four treated with corticosteroids, and three of four treated with corticosteroids and transethmoidal decompression of the optic canal. No patients lost vision after their initial examination. Two of six initially blind eyes regained some vision. Management of these injuries is controversial. Hospitals should designate physician teams that should develop and implement a treatment plan for traumatic optic neuropathies.

• Only four cases of chaismal syndromes caused by arteriovenous malformations (AVMs) have been reported. We have examined two patients with chiasmal dysfunction caused by an AVM. In one patient, the AVM was suspected only after angiography. However, the presence of an angioma of the lip might have been a clue to the diagnosis. In the other patient, seizures and pulsating proptosis led to the correct diagnosis before angiography was performed. This patient also had episodes of transient bilateral blindness, presumably caused by postictal states or by periodic shunting with ischemia (chiasmal steal).

• A 32-year-old black woman with systemic lupus erythematosus went rapidly blind due to severe bilateral retinal vasculitis. The mechanism appeared to be total arrest of the retinal circulation by thrombosis of most of the retinal vasculature including major arterioles.(Arch Ophthalmol 95:794-797, 1977)

• Color blindness developed in five patients apparently because of lesions in the posterior portions of both cerebral hemispheres. Three of them also had symptoms of prosopagnosia. The lesions were neoplastic in two and vascular in three of the patients. It would appear that bilateral, Inferior, occipital lobe lesions may be responsible both for acquired cerebral dyschromatopsia and prosopagnosia. Evidence from experimental investigations in primates suggests that the areas of the cerebral hemispheres analogous to those Involved in these patients, may be specialized for the processing of colored stimuli.(Arch Ophthalmol 95:121-128, 1977)

OBJECTIVE: Spontaneous recovery of a sixth nerve palsy is thought to rule out a neoplastic origin. We reviewed cases of sixth nerve palsy that improved without treatment but that ultimately proved to be caused by a tumor at the base of the skull. DESIGN: Case series. SETTING: Hospital-based, neuro-ophthalmology referral practice. PATIENTS: Seven patients with an age range from 7 to 61 years had sixth nerve palsy secondary to a slow-growing neoplasm at the skull base. MAIN OUTCOME MEASURES: Return of lateral rectus function and resolution of diplopia without intervention. RESULTS: Seven patients with sixth nerve palsy caused by skull base tumors experienced spontaneous improvement of their deficit. Recovery time ranged from 1 week to 18 months. No patient was diabetic or had evidence of vascular disease. In one patient, the palsy improved once prior to becoming a fixed deficit, and spontaneous improvement occurred on two to five occasions in the other patients. CONCLUSION: Spontaneous recovery of a sixth nerve palsy can occur in the presence of an extramedullary compression by a tumor at the base of the brain. Possible mechanisms for recovery include remyelination, axonal regeneration, relief of transient compression (eg, resorption of hemorrhage), restoration of impaired blood flow, slippage of a nerve previously stretched over the tumor, or immune responses to the tumor.

The right optic nerves of three cynomolgus monkeys were frozen under direct visualization after a modified Kröhnlein operation. Left optic nerves were subjected to an identical procedure without freezing. Postoperatively, visual evoked responses (VERs) could not be obtained by stimulating right eyes, while electroretinograms from both eyes and VERs obtained by stimulating left eyes were normal. Extensive lesions which were found in the right optic nerves of all animals were histologically similar to those obtained by freezing in the brain.

Ischemic optic neuropathy developed in five eyes of four patients with optic nerve drusen. Two of the patients were in their 20s, a decade when idiopathic ischemic optic neuropathy is rare. This argues against a chance concurrence of drusen and idiopathic ischemic optic neuropathy. Infarction of the distal portion of the optic nerve in patients with drusen may result from mechanical distortion of blood vessels in the laminar and prelaminar regions.

We examined six patients who developed blurring or oscillopsia as a result of downbeat nystagmus while being treated with lithium carbonate. Of these six plus six previously described similar patients, all but two developed downbeat nystagmus insidiously as an isolated disorder in the setting of otherwise satisfactory therapeutic control, without clinical or biochemical evidence of acute lithium intoxication. Only six of these 12 patients were able either to reduce or to stop taking lithium, and in only two of these six did the downbeat nystagmus improve or remit.

Tobacco amblyopia, once an important cause of bilateral optic neuropathy, has become so rare in the United States that some investigators doubt its existence. However, we treated two men who appeared to have this disorder. These two patients demonstrate that tobacco amblyopia can develop without malnutrition, alcoholism, or disordered vitamin B12metabolism. Both patients recovered, one with cessation of smoking and the other with intramuscular administration of hydroxocobalamin despite continued smoking.

Two patients showed inability to voluntarily close the eyes. They had normal reflex closure and slept with their eyes closed. Comprehension was normal. Both had bilateral frontal lesions. This symptom is probably indicative of fundamental motor dysfunction, rather than apraxia as has previously been suggested.

Sodium cyanide intoxication of rats produced demyelinative and necrotic lesions in the corpora callosa in 70% and the optic nerves in 20%. The optic neuropathy was bilateral, focal, and retrobulbar. All animals with optic neuropathies had marked callosal lesions. Chiasmal involvement was rare. Certain features of experimental cyanide intoxication negate the possibility that cyanide is responsible for human optic nerve diseases.

The injection of 750 units of α-chymotrypsin into the posterior chamber of the rhesus monkey eye resulted frequently in sustained elevation of the intraocular pressure. Retinal and neural degeneration, typical of that encountered in spontaneous human and experimental animal glaucoma, was found. Central corneal degeneration was frequent and led to perforation. Atrophy of the ciliary body was a striking finding in monkeys that developed glaucoma, and was present as early as three days after instillation of the enzyme. It is suggested that the pressure elevation was the consequence of ciliary body damage.

INTRODUCTION Since alkaline phosphatase was first reported in the retina by deConciliis in 1934,1 much study has gone into the chemical and histochemical demonstration of various phosphatases in the eye. Until recently most attention was directed to acid and alkaline phosphatase. In the past few years interest has shifted to certain "specific" phosphatases whose optimal activity occurs within or close to the physiological range of tissue pH and whose substrates are known to play important roles in intermediary metabolism.Various types of adenosine triphosphatase (ATPase) have been demonstrated in retina, ciliary body, iris sphincter, choroid, corneal epithelium, corneal endothelium, and lens capsule and epithelium.2 In certain of these loci the enzyme is thought to be concerned with cation transport. 5′-Nucleotidase has been detected in the choroid and retina.3 Yet there is little histochemical evidence for the localization of these enzymes in the eye. In 1957 Wachstein and

Neuroglia have been recognized in the retina since 1851 when Müller described the cells that bear his name and have been studied most vigorously in recent times with various silver methods. These cells exhibit metabolic1 and ultrastructural2 similarities to neuroglia in the brain. Despite these similarities they bear little outward resemblance to conventional astroglia or oligodendroglia which constitute the chief components of glia in the central nervous system. The present study was undertaken in an effort to determine the neuroglial composition of the retina as revealed by several staining methods. METHOD Studies were made on 29 human retinas, both normal and pathologic, and on normal retinas from monkey, calf, rat, cat, mouse, rabbit, and toadfish. The eyes were fixed in Cajal's formol-ammonium bromide or transferred to that fixative after initial immersion in neutral formalin. The mid-periphery and far-periphery of each retina were studied. The papillary and peripapillary regions

In the 12-year-period, 1958 to 1969, there was a 202% increase in reported cases of primary and secondary syphilis in the United States and, as a consequence, congenital syphilis in children under 1 year of age rose by 117% during the ten years from 1960 to 1969. In the same decade, congenital syphilis in children under 10 years of age rose by 168%. At the Boston City Hospital Skin Clinic, 271 patients with late congenital syphilis were examined from January 1967 through June 1969, and the presence or absence of 14 common stigmata were carefully checked. Of these, five are major signs and, for all practical purposes, are diagnostic of congenital syphilis. The other nine stigmata are minor signs, and of themselves are not diagnostic of congenital syphilis.

In the past decade attention has been focused upon the Mariana Islands of the western Pacific as an area of particular interest to neurologists and epidemiologists. The prevalence of amyotrophic lateral sclerosis among the indigenous Chamorro population is unusually high.1-4 Recent reports have described the clinical,5 pathological,6 and electroencephalographical7 features of another endemic neurological disease of Guam, "the parkinsonism-dementia complex." It is the purpose of this communication to describe the epidemiological characteristics of this latter disorder. THE MARIANAS GROUP AND ITS PEOPLE The Marianas form a chain of 15 volcanic and coral islands between 13 and 21 degrees N. latitude at 145 degrees E. longitude (Fig. 1). Guam, a territory of the United States, is the largest and most southern of the group. It is located 5,100 miles west-southwest of San Francisco and 1,600 miles south of Tokyo. It lies within the Tropic of Cancer, and

INTRODUCTION For the past 10 years the National Institute of Neurological Diseases and Blindness, through its Epidemiology Branch, has maintained a center for the study of the various neurological diseases that occur among the indigenous population (Chamorros) of the Island of Guam. The stability the population of the island, the high quality of medical care available, the small size of the island, and the relatively large sibships are proven assets to the epidemiologist working on Guam. This natural epidemiological laboratory has already provided much important data regarding several degenerative diseases of the nervous system. The prevalence of amyotrophic lateral sclerosis (ALS) has been shown to be 100 times that encountered anywhere else in the world.1-4 Recent surveys have established the presence of a unique, highly prevalent degenerative disease—the parkinsonism-dementia complex—manifested by dementia, extrapyramidal and pyramidal tract signs, and evidence of involvement of lower motor neurons.5 There is clinical

Arteriovenous malformations within or adjacent to the brain stem produce unpredictable signs and symptoms mimicking other vascular diseases, tumors, and multiple sclerosis. Two cases with prominent ocular findings illustrate the ways in which these rare lesions present clinically and evoke local and distant neuro-ophthalmic changes.

A young woman with erythema nodosum demonstrated the clinical and fluorescein angiographie findings of acute posterior multifocal placoid pigment epitheliopathy. The possibility of a relationship between the two disorders is raised. The fluorescein angiograms seemed suggestive of choroidal vascular disease rather than of a primary pigment epitheliopathy.