Prospective analysis of macular and optic disc changes after non-arteritic anterior ischemic optic neuropathy.

PURPOSE: To prospectively analyse macular and optic disc changes after the occurrence of non-arteritic anterior ischemic optic neuropathy (NAION) and study possible predictors of final visual outcome. METHODS: Patients with NAION underwent a complete ophthalmic examination, including spectral-domain optical coherence tomography of the macula and optic nerve head. The examination was repeated 1, 3, 6, 9 and 12 months after onset. Final visual prognosis was evaluated by visual field (VF) and best-corrected visual acuity (BCVA) at the final visit. Data within the NAION group were analysed over the course of the disease and compared to a disease-free control group at each visit. RESULTS: Twenty-two eyes with NAION and 43 eyes from a control group were included. The retinal nerve fiber layer (RNFL) was significantly thicker in NAION eyes than controls at presentation (P=0.00), and significantly decreased during the next 3 months after presentation (P=0.02). The ganglion cell+inner plexiform layer (GCIPL) was thinner in the NAION group throughout the course of the disease (all P<0.05). Although the acute NAION eyes had significantly lower cup/disc ratios and higher neuroretinal and disc sizes (all P=0.00), there were no significant differences between groups from the third month onwards (all P>0.05). The best predictors of BCVA and VF were GCIPL at 3 months of follow-up (r2=0.32; P=0.03) and RNFL at 6 months of follow-up (r2=0.41; P=0.01) respectively. CONCLUSIONS: RNFL and optic disc changes occur during the first 3 months after the onset of NAION, whereas GCIPL is affected soon after the onset of symptoms. GCIPL and RNFL are useful predictors of final visual outcome.

Optical coherence tomography of the retinal nerve fibre layer in mild papilloedema and pseudopapilloedema.

AIMS: To determine the degree to which optical coherence tomography (OCT) can distinguish differences in retinal nerve fibre layer (RNFL) thickness between eyes with mild papilloedema, pseudopapilloedema, and normal findings. METHODS: 13 patients with mild papilloedema, 11 patients with congenitally crowded optic nerves, and 17 normal subjects underwent neuro-ophthalmic examination, automated visual field testing, and fundus photography. Spinal fluid pressure measurements were obtained in a subgroup of five patients with pseudopapilloedema and 11 patients with mild papilloedema. Circular OCT scans using a diameter of 3.38 mm surrounding the optic disc were performed in each eye of patients and subjects. Fundus photographs were analysed by two observers who diagnosed crowding or papilloedema and graded amounts of swelling. Findings were assessed by descriptive statistics and variance analysis. RESULTS: RNFL thickness was greater in the superior and inferior quadrants and showed a high degree of correlation between each group of patients and subjects. A statistically significant difference was found in mean RNFL thickness between both groups of patients with optic disc swelling and normal subjects. However, there was not a statistical difference in mean nerve fibre layer thickness between patients with papilloedema and those with congenitally crowded optic nerves. CONCLUSIONS: OCT demonstrates measurable differences in nerve fibre layer thickness between normal subjects and patients with either papilloedema or pseudopapilloedema. However, OCT does not appear to differentiate between those individuals with congenitally crowded optic nerves and those with mild papilloedema caused by increased intracranial pressure.

Metamorphopsia and palinopsia. Association with periodic lateralized epileptiform discharges in a patient with malignant astrocytoma.

A patient with a right parietal glioma developed palinopsia and various forms of metamorphopsia that were temporally related to the electrographic presence of periodic lateralized epileptiform discharges. These symptoms occurred in an alert woman with normal visual fields. The literature shows that epileptic events can produce these symptoms. We believe that continuous repetitive electrical stimulation caused our patient's unusual symptoms by altering the association pathways through which visual data are experienced. To our knowledge, the association between periodic lateralized epileptiform discharges and metamorphopsia or palinopsia has heretofore not been reported.

Parasellar and orbital apex syndrome caused by aspergillosis.

Patients with orbital aspergillosis commonly present with unilateral proptosis and associated sinus disease. However, neither of these manifestations was observed in a 62-year-old woman who had an orbital apex syndrome with visual loss, complete ophthalmoplegia, and corneal hypoesthesia. Pathologic examination of specimen from a granulomalike mass removed at left frontotemporal craniotomy showed branching hyphae with the characteristic appearance of Aspergillus.

Orbital myositis and giant cell myocarditis.

A 65-year-old woman developed progressive, bilateral ophthalmoplegia, with thickened extraocular muscles on CT. One month later, a cardiac arrhythmia led to her death. Pathologically, the extraocular and skeletal muscles showed diffuse mononuclear cell inflammation, while the heart contained granulomatous myositis. This patient's syndrome of idiopathic, orbital myositis and giant cell myocarditis may be a distinct nosologic entity.

Periodic alternating gaze deviation in infancy.

Periodic alternating gaze is a rarely reported phenomenon. We have observed two cases that are unique in their early onset at birth and infancy. Multiple congenital defects of the posterior cranial fossa were present on MRI in both cases. A prominent abnormality shared by both was absence of normal structures in the region of the inferior cerebellar vermis. Periodic alternating gaze appears to be associated with pathologic changes in the hindbrain in these and other reported cases.

Intermittent downbeat nystagmus due to vertebral artery compression.

Downbeat nystagmus (DBN) uncommonly occurs as a transient phenomenon, and it rarely occurs in patients with cerebrovascular disease. We observed a patient with intermittent DBN and lightheadedness due to transient obstruction of his dominant vertebral artery when he turned his head to his left side. Surgical removal of an osteophyte at the site of the angiographically demonstrated lesion relieved his symptoms.

Ocular and pericardial involvement in Legionnaires' disease.

Legionnaires' disease can exhibit protean extrapulmonary manifestations. Pericardial involvement is rare and has been described in three case reports. A patient is described with Legionnaires' disease and pericardial and ocular involvement, an entity that has not been reported previously. This patient was successfully treated with intravenous erythromycin with resolution of his pericardial effusion and ophthalmologic findings.

Cancer-associated retinopathy in a patient with non-small-cell lung carcinoma.

Cancer-associated retinopathy (CAR) is a paraneoplastic syndrome most often associated with small-cell lung carcinoma (SCLC), and it has been reported in patients with other malignancies. Antibodies against recoverin, a 23-kDa protein, have been found in patients with CAR suggesting an autoimmune phenomenon. Herein is the first report of a patient with non-small-cell lung cancer (NSCLC) in whom anti-recoverin antibodies were detected in the serum. Steroid therapy, chemotherapy, and radiation therapy did not help the patient's vision. Progressive loss of vision in patients with lung cancer should, potentially, be tested for CAR.

Orbital color Doppler imaging in carotid occlusive disease.

OBJECTIVE: To define orbital circulation abnormalities identified by color Doppler imaging in patients with severe carotid occlusive disease. PATIENTS: Twenty-four patients referred to a hospital-based neuro-ophthalmology service with hemodynamically significant carotid occlusive disease (> 75% stenosis) were prospectively studied. Eight had signs of ocular ischemic syndrome; 12 of the 24 patients underwent endarterectomy. MAIN OUTCOME MEASURES: Peak systolic velocity of the central retinal, posterior ciliary, and ophthalmic artery and pulsatility indexes as determined by color Doppler imaging. METHODS: Color Doppler imaging was performed using a 7.5-MHz probe. Both eyes were studied in all patients and carotid duplex imaging was obtained. RESULTS: All patients with hemodynamically significant carotid occlusive disease had lower mean peak systolic velocities in the central retinal, posterior ciliary, and ophthalmic arteries and higher pulsatility indexes compared with normal control patients. Endarterectomy improved peak systolic velocities. Reversal of ophthalmic flow direction as a separate variable was unassociated with altered mean central retinal or posterior ciliary artery flow velocities. Patients with ocular ischemic syndrome may have similar orbital color Doppler imaging findings compared with patients with severe carotid occlusive disease without overt manifestations of chronic ocular ischemia. CONCLUSION: Orbital circulation is highly adaptable even when faced with severe compromise in proximal blood flow.

Botulin ophthalmoplegia. Clinical and oculographic observations.

Two patients with botulin ophthalmoplegia had rapid quivering eye motions during attempts to refixate laterally placed objects; these abnormal eye movements occurred in conjunction with a disjunctive limitation of range of eye movements. Oculographic examination showed that the quivering motions were composed of multiple hypometric saccades, many of which had subnormal and stuttering velocities. By blocking acetylcholine release into myoneural junctions, botulin toxin seems to limit the duration of saccadic burst innervation reaching ocular muscle.

The clinical value of negative temporal artery biopsy specimens.

The clinical course of patients with signs and symptoms suggestive of temporal arteritis but with negative temporal artery biopsy specimens was evaluated. Ninety-one patients undergoing a biopsy formed the basis of this study. Of these, 63 patients had no evidence of arteritis on biopsy, and 28 patients had biopsy specimens showing granulomatous inflammation. False-negative findings from biopsy specimens occurred in 5% of patients who had the disease. Cancer was the final diagnosis in 21% of patients with negative biopsy specimens v 3% of patients with temporal arteritis. Various chronic systemic inflammatory diseases were found in 16% of patients with negative biopsy specimens, while none of the patients with temporal arteritis had additional systemic inflammatory diseases. Patients with proven arteritis were notably older and had higher ESRs than patients without the disease. However, no laboratory test or frequently observed symptom or sign noted on initial examination, considered alone or in combination with other findings, had diagnostic sensitivity or specificity as high as temporal artery biopsy.

Intravenous digital subtraction angiography and its role in ocular vascular disease.

One hundred patients with ocular occlusive vascular disease were studied by intravenous digital subtraction angiography (IV DSA). In 17 patients with transient monocular blindness, 65% showed ipsilateral carotid atheroma. The degree of carotid stenosis was greater than 70% in eight of the 17 patients. Severe unilateral or bilateral stenosis was demonstrated in 70% of ten patients with chronic retinal artery obstruction, ie, venous stasis retinopathy or orbital hypoxia. An ipsilateral carotid artery source of emboli or substantial degree of stenosis was documented in 50% of 48 patients with acute retinal artery obstruction. Little or no carotid disease was found in 72% of 25 patients with retinal vein obstruction. The greatest value of IV DSA is in the management of patients with transient monocular blindness.

Corneal and conjunctival effects of monobenzone in patients with vitiligo.

Monobenzone (the monobenzyl ether of hydroquinone [Benoquin]) is used topically by patients with extensive vitiligo to depigment their remaining normally pigmented skin. A patient who had been applying the drug for one year had an anterior linear deposition of pigment in both corneas. Of 15 additional patients with vitiligo, 11 of whom were using monobenzone, acquired conjunctival melanosis in two patients and pingueculae in three may have been related to monobenzone use. Light and electron microscopy of one corneal epithelial scraping and 12 conjunctival biopsy specimens revealed pleomorphic, single-membrane-limited intracytoplasmic inclusions within the corneal epithelium and within the epithelium, fibrocytes, histiocytes, and vascular endothelium of the conjunctiva. The ultrastructural aspects of these inclusions suggested that they are residual bodies containing lipid and lipofuscin.

Optical coherence tomography demonstrates subretinal macular edema from papilledema.

OBJECTIVE: To evaluate macular changes in eyes with papilledema from increased intracranial pressure using optical coherence tomography (OCT). METHODS: Fifty-five patients with papilledema seen during 1998 and 1999 were studied with OCT of the optic nerve and retinal nerve fiber layer. Nineteen of these also had OCT of the macula during periods of acute, subacute, or recurrent papilledema and were evaluated in detail for this report. RESULTS: Seven patients had OCT evidence of subretinal fluid involving the macula. All had some reduction in visual acuity. The subretinal fluid appeared to arise from the peripapillary region, and all showed some improvement in central vision as the fluid resolved. CONCLUSIONS: Subretinal fluid accumulations can cause decreased visual acuity in patients with papilledema. Optical coherence tomography can demonstrate subretinal fluid and can be used to follow the course of this important visual complication of papilledema.

Ocular involvement associated with chronic Epstein-Barr virus disease.

Ocular involvement with acute Epstein-Barr virus infection is usually limited to a transient follicular conjunctivitis, although other lesions have been reported. Chronic Epstein-Barr virus infection has recently gained attention, but ocular manifestations have not been emphasized. We describe three patients with chronic infection with prominent ocular involvement. Bilateral uveitis was noted in all patients, ranging from an anterior uveitis that was responsive to steroids to a severe panuveitis with vitritis, cataract, optic disc swelling, and macular edema. In one patient, topical acyclovir ointment resulted in a substantial decrease in the inflammatory reaction when added to systemic acyclovir therapy. Another patient displayed a keratitis that resolved with topical steroid therapy. Cataract and vitreous surgery were also beneficial in the management of these patients.

Food shortages and an epidemic of optic and peripheral neuropathy in Cuba.

From late 1991 to mid-1993, cases of optic neuropathy of unknown etiology, which first appeared in unusual numbers in a western province of Cuba, spread and multiplied throughout the island. The dominant symptoms changed, becoming increasingly those of peripheral neuropathy. Incidence rates peaked in April 1993. An estimated 50,000 cases were reported. The majority were adult men and women (aged about 25-65), with comparatively few children or elderly people being affected. The cause has yet to be delineated. However, food shortages and radical changes in diet resulting from the longstanding US trade embargo and the recent loss of Eastern Europe as Cuba's trading partner have compromised nutritional status, especially B-vitamin sufficiency, and appear to be related to the neuropathic illnesses. In April 1993, the Cuban government began distributing vitamin supplements to every citizen. Causal hypotheses include tobacco-alcohol or "nutritional" amblyopia; cyanide toxicity from cassava; toxic legumes introduced as supplements to scarce flour; other toxins, for example pesticides, or a "blue mold" on tobacco; enterovirus; and a hereditary enzyme deficiency in affected persons. None of these factors appears to be present in all cases, but it is generally believed that an interaction of some toxin or toxins, in combination with nutritional deficiency, is likely to be the major cause.

Bilateral visual loss in a child with disc swelling.

A nine-year-old girl developed periorbital pain followed by sequential visual loss over a 3-day period. The patient recovered vision to a 20/40 level over a 5-month period of time. The differential diagnosis and evaluation in this presentation is discussed. The patient is believed to have had an idiopathic or parainfectious optic neuritis.

Ophthalmoplegia associated with AIDS.

A 34-year-old man with AIDS was admitted to the hospital with a one-week history of cough, chest pain, and fever. Radiography revealed a cavitating left upper lobe lesion. Two weeks later he developed a headache associated with a contrast enhancing lesion in the right parietal lobe. The patient had a progressive downhill course, developing atrioventricular block and hypernatremia. Neuro-ophthalmologically, there was a mild facial droop, "hand motions" vision with presumed bilateral cytomegalic inclusion retinitis, and signs of a mesencephalic syndrome, including lid retraction. Discussions center on the differential diagnosis of the central nervous system disease and the obligative recommendations the neuro-ophthalmologist must be willing to make.

Papilledema: its recognition and relation to increased intracranial pressure.

This has been a review on the subject of papilledema from a clinical, pathologic and experimental point of view. Terminology has been clarified. The term papilledema should be reserved for those patients with optic disc edema caused by increased intracranial pressure. The forms of papilledema which are identifiable are: 1. Early (incipient) form which develops into an acute type and then into a fully developed form; 2. Fully developed form, characterized by obscured disc margins, hemorrhages and ischemic infarcts; 3. Chronic papilledema which may persist (the name "vintage" papilledema may be applied to those patients affected in varying degrees up to several years); and 4. Chronic atrophic papilledema which, as the name implies, illustrates a form we do not see as frequently as in years past. It is the type we are now trying to prevent and in this effort we are fortunately achieveing some success. The importance of the intracranial expanding lesions and the influence of the rapidity of elevation and duration of intracranial hypertension on the optic nerve has been considered, based on the clinical and experimental work to date. The pathogenesis of papilledema has been considered in some detail. A unified whole is difficult to arrive at in such a complex situation. Recent advances in our understanding of anatomic, mechanical (sheath space pressure), tissue and vascular pressure as related to increased intracranial pressure have been described. A hydrostatic mechanism brings these factors together in a reasonable, although admittedly not completely proven concept of a mechanism for the development of papilledema. Clarification of terminology and clinical appearance of the various forms of disc edema related to intracranial pressure are of practical value in the diagnosis and management of each patient we see with this clinical entity.