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PurposeTo estimate the prevalence of abnormalities in visual function and ocular structures associated with the long-term use of tamoxifen citrate.MethodsA single-masked, cross-sectional study involving multiple community and institutional ophthalmologic departments was conducted with a volunteer sample of 303 women with breast cancer currently taking part in a randomized clinical trial to determine the efficacy of tamoxifen (20 mg/day) in preventing recurrences. Participants included women who had never been on drug (n=85); women who had taken tamoxifen for an average of 4.8 years, then been off the drug for an average of 2.7 years (n=140); and women who had been on tamoxifen continuously for an average of 7.8 years (n=78). Women were evaluated by questionnaire, psychophysical testing, and clinical examination to determine any abnormalities in visual function and the comparative prevalences of corneal, lens, retinal, and optic nerve pathology.ResultsThere were no cases of vision-threatening ocular toxicity among the tamoxifen-treated participants. Compared with nontreated participants, the tamoxifen-treated women had no differences in the activities of daily vision, visual acuity measurements, or other tests of visual function except for color screening. Intraretinal crystals (odds ratio [OR]=3.58, P=.178) and posterior subcapsular opacities (OR=4.03, P=.034) were more frequent in the tamoxifen-treated group.ConclusionsWomen should have a thorough baseline ophthalmic evaluation within the first year of initiating tamoxifen therapy and receive appropriate follow-up evaluations.

Background: Studies suggest that weight gain is a prominent risk factor for recurrence of papilledema in idiopathic intracranial hypertension (IIH). Given this information, the significant weight gain that occurs during pregnancy, and the fact that pharmacologic therapy is many times discontinued, raises concerns for worsening edema and vision loss. To examine the impact of pregnancy weight gain on IIH, a retrospective chart review of patients with IIH and pregnancy was performed. Compared with previous studies, we 1) quantified findings with optical coherence tomography (OCT) and Humphrey visual field (HVF) data, 2) Included baseline data before pregnancy, 3) determined excess pregnancy weight gain using body mass index-adjusted weight gain goals, and 4) correlated worsening in IIH symptoms with changes in papilledema., Methods: Charts were reviewed for patients with diagnoses of IIH who had at least 2 visits with neuro-ophthalmology during pregnancy. Thirteen patients met inclusion criteria. Data were compared from baseline visits before pregnancy, pregnancy visits, and postpregnancy visits., Results: Comparisons of HVF mean deviation (MD), OCT retinal nerve fiber layer (RNFL), and Max OCT RNFL during pregnancy were not significant compared with baseline ( P = 0.51, 0.41, and 0.25). Three patients were found to have increased papilledema during pregnancy (Max Avg OCT RNFL of 152.5, 129, and 123.5 μm) of which 2 developed new reproducible mild visual field defects (HVF ∆MD -1.78 and -4.49). All patients showed more than the 6% weight gain, typically observed in recurrent IIH. Eleven patients gained more than their weight from initial diagnosis. Eight patients had excess pregnancy weight gain. Six patients discontinued pharmacologic therapy for IIH., Conclusions: Weight gain seems to carry a lower risk in IIH patients when associated with pregnancy. This is suggested by the high rate of stable or even decreased disc edema in patients despite medication discontinuation and excess pregnancy weight gain. We postulate these findings may be related to changes in weight distribution or endocrine changes during pregnancy., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Although visual symptoms are common following concussion, quantitative measures of visual function are missing from concussion evaluation protocols on the athletic sideline. For the past half century, rapid automatized naming (RAN) tasks have demonstrated promise as quantitative neuro-visual assessment tools in the setting of head trauma and other disorders but have been previously limited in accessibility and scalability. The Mobile Interactive Cognitive Kit (MICK) App is a digital RAN test that can be downloaded on most mobile devices and can therefore provide a quantitative measure of visual function anywhere, including the athletic sideline. This investigation examined the feasibility of MICK App administration in a cohort of Division 1 college football players. Participants (n = 82) from a National Collegiate Athletic Association (NCAA) Division 1 football team underwent baseline testing on the MICK app. Total completion times of RAN tests on the MICK app were recorded; magnitudes of best time scores and between-trial learning effects were determined by paired t-test. Consistent with most timed performance measures, there were significant learning effects between the two baseline trials for both RAN tasks on the MICK app: Mobile Universal Lexicon Evaluation System (MULES) (p < 0.001, paired t-test, mean improvement 13.3 s) and the Staggered Uneven Number (SUN) (p < 0.001, mean improvement 3.3 s). This study demonstrated that the MICK App can be feasibly administered in the setting of pre-season baseline testing in a Division I environment. These data provide a foundation for post-injury sideline testing that will include comparison to baseline in the setting of concussion., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Background: Applying oncoplastic techniques to breast conservation therapy is believed to improve cosmetic and oncologic outcomes, compared with standard breast conservation therapy alone. This study aimed to perform a comprehensive review of the literature comparing outcomes of oncoplastic breast conservation therapy (BCT + R) with that of standard breast conservation therapy alone (BCT). A secondary objective was to compare these results to outcomes after oncoplastic breast conservation therapy performed at our institution (BCT + r)., Methods: A literature search was performed in PubMed using key words, "oncoplastic," "partial breast reconstruction," and "breast conservation therapy." Case reports, case series, and studies with fewer than 10 patients and studies that did not report re-excision rates were excluded. A retrospective chart review was performed from 2011 to 2017 of all cases of oncoplastic breast conservation therapy performed at our institution by a single 2-surgeon team consisting of 1 breast surgeon and 1 plastic surgeon. Outcomes were assessed by comparing re-excision rates between the 3 comparison groups (BCT, BCT + R, BCT + r)., Results: The BCT group was made of 5965 patients (22 articles), and the BCT + R group comprised 2564 patients (41 articles). Re-excision rates in the BCT + R group were lower (4.0%) than the BCT group (17.2%, P = 0.0001). One hundred seventy-two patients comprised the BCT + r group and underwent oncoplastic breast conservation therapy during the study period at our institution. The re-excision rate in the BCT + r group was 1.7% and was significantly lower than the BCT group (P = 0.0001) and lower but not significantly different from the BCT + R group (P = 0.2113)., Conclusions: Oncoplastic breast conservation therapy leads to lower re-excision rates compared with standard breast conservation therapy. Oncoplastic breast conservation therapy may improve oncologic outcomes compared with standard breast conservation therapy by allowing for more extensive resection without compromising aesthetic results.

Background: Medical photography has become an important component of the evaluation and management of patients across many specialties. It is increasingly utilized in contemporary practice with modern smartphones and enhanced digital media. Photography can enhance and improve treatment plans and communication between providers and patients. Additionally, photography supplements education, research, and marketing in both print and social media. Ethical and medicolegal standards for medical photography, specifically for patients with breast disease, have not been formally developed to guide medical providers., Purpose: To provide guidelines for breast care physicians using medical photography, the Ethics Committee of the American Society of Breast Surgeons presents an updated review of the literature and recommendations for ethical and practical use of photography in patient care., Methods: An extensive PubMed review of articles in English was performed to identify studies and articles published prior to 2018 investigating the use of medical photography in patient care and the ethics of medical photography. After review of the literature, members of the Ethics Committee convened a panel discussion to identify best practices for the use of medical photography in the breast care setting. Results of the literature and panel discussion were then incorporated to provide the content of this article., Conclusion: The Ethics Committee of the American Society of Breast Surgeons acknowledges that photography of the breast has become an invaluable tool in the delivery of state-of-the-art care to our patients with breast disease, and we encourage the use of this important medium. Physicians must be well informed regarding the concerns associated with medical photography of the breast to optimize its safe and ethical use in clinical practice.

Background: This analysis was performed to assess the impact of early intervention following prospective surveillance using bioimpedance spectroscopy (BIS) to detect and manage breast cancer-related lymphedema (BCRL)., Methods: From 8/2010 to 12/2016, 206 consecutive patients were evaluated with BIS. The protocol included pre-operative assessment with L-Dex as well as post-operative assessments at regular intervals. Patients with L-Dex scores >10 from baseline were considered to have subclinical BCRL and were treated with over-the-counter (OTC) compression sleeve for 4 weeks. High-risk patients were defined as undergoing axillary lymph node dissection (ALND), receiving regional nodal irradiation (RNI), or taxane chemotherapy. Chronic BCRL was defined as the need for complex decongestive physiotherapy (CDP)., Results: Median follow-up was 25.9 months. Overall, 17% of patients had one high-risk feature, 8% two, and 7% had three. 9.8% of patients were diagnosed with subclinical BCRL with highest rates seen following ALND (23 vs. 7%, p = 0.01). Development of subclinical BCRL was associated with ALND and receipt of RNI. At last follow-up, no patients (0%) developed chronic, clinically detectable, BCRL. Subset analysis was performed of the 30 patients undergoing ALND. Median number of nodes removed was 18 and median number of positive nodes was 2. 77% received taxane chemotherapy, 62% axillary RT, and 48% had elevated BMI. Overall, 86% of patients had at least one additional high-risk feature, 70% at least two, and 23% had all three. Seven patients (23%) had abnormally elevated L-Dex scores at some point during follow-up. To date, none has required CDP., Conclusions: The results of this study support prospective surveillance utilizing BIS initiated pre-operatively with subsequent post-operative follow-up measurements for the detection of subclinical BCRL. Intervention triggered by subclinical BCRL detection with an elevated L-Dex score was associated with no cases progressing to chronic, clinically detectable BCRL even in very high-risk patients.

In the original publication of the article, under the heading, Study limitations in the Discussion section, the second paragraph, fifth sentence was published incorrectly as "Patients with an L-Dex increase of ≥ 5.5 undergo…sleeve for 4 weeks".

Objective: To evaluate the prevalence and clinical profile of patients with biopsy-proven arteritic anterior ischemic optic neuropathy presenting with preserved visual acuity of 20/40 or better and those with an initial poor visual acuity of 20/50 or worse through a retrospective chart review., Results: Nine of 37 patients with arteritic anterior ischemic optic neuropathy presented with a preserved visual acuity of 20/40 or better in the affected eye. All patients with preserved visual acuity had initial visual field defects that spared the central field. All 37 patients immediately received high-dose corticosteroid therapy. Visual acuity worsened by > 2 lines in one of nine patients (11%) with preserved visual acuity, with a corresponding progression of visual field constriction., Conclusion: Although preserved visual acuity of 20/40 or better has traditionally been associated with the nonarteritic form of anterior ischemic optic neuropathy, giant cell arteritis should still be strongly considered, especially if they have giant cell arteritis systemic symptoms.

Current diagnosis and monitoring of sports-related concussion rely on clinical signs and symptoms, and balance, vestibular, and neuropsychological examinations. Conventional brain imaging often does not reveal abnormalities. We sought to assess if the longitudinal change of functional and structural connectivity of the default-mode network (DMN) can serve as a potential biomarker. Eight concussed Division I collegiate football student-athletes in season (one participated twice) and 11 control subjects participated in this study. ImPACT (Immediate Post-Concussion Assessment and Cognitive Testing) was administered over the course of recovery. High-resolution three dimensional T1-weighted, T2*-weighted diffusion-tensor images and resting-state functional magnetic resonance imaging (rs-fMRI) scans were collected from each subject within 24 h, 7±1 d and 30±1 d after concussion. Both network based and whole-brain based functional correlation analyses on DMN were performed. ImPACT findings demonstrated significant cognitive impairment across multiple categories and a significant increase of symptom severity on Day 1 following a concussion but full recovery by 6.0±2.4 d. While the structural connectivity within DMN and gross anatomy appeared unchanged, a significantly reduced functional connectivity within DMN from Day 1 to Day 7 was found in the concussed group in this small pilot study. This reduction was seen in eight of our nine concussion cases. Compared with the control group, there appears a general trend of increased DMN functional connectivity on Day 1, a significant drop on Day 7, and partial recovery on Day 30. The results of this pilot study suggest that the functional connectivity of DMN measured with longitudinal rs-fMRI can serve as a potential biomarker to monitor the dynamically changing brain function after sports-related concussion, even in patients who have shown clinical improvement.

A 37-year-old woman presented with an anterior optic neuropathy related to Bartonella henselae. Twenty-nine days after symptom onset, a partial thickness macular hole developed in the involved eye. Fundus photography and optical coherence tomography confirmed the conversion to a full-thickness macular hole in 2 months. Macular hole as a complication of cat scratch disease is a rare entity, with 2 prior reported cases in children. The development of a macular hole following cat scratch disease can appear without the clinical picture of multiple white chorioretinal lesions, macular star, or vitritis.

Statins have recently been shown to exert neuronal protection in ischemic stroke. Reactive oxygen species, specifically superoxide formed during the early phase of reperfusion, augment neuronal injury. NADPH oxidase is a key enzyme for superoxide production. The present study tested the hypothesis that atorvastatin protects against cerebral infarction via inhibition of NADPH oxidase-derived superoxide in transient focal ischemia. Transient focal ischemia was created in halothane-anesthetized adult male Sprague-Dawley rats (250-300 g) by middle cerebral artery occlusion (MCAO). Atorvastatin (Lipitor, 10 mg/kg sc) was administered three times before MCAO. Infarct volume was measured by triphenyltetrazolium chloride staining. NADPH oxidase enzymatic activity and superoxide levels were quantified in the ischemic core and penumbral regions by lucigenin (5 microM)-enhanced chemiluminescence. Expression of NADPH oxidase membrane subunit gp91(phox) and membrane-translocated subunit p47(phox) and small GTPase Rac-1 was analyzed by Western blot. NADPH oxidase activity and superoxide levels increased after reperfusion and peaked within 2 h of reperfusion in the penumbra, but not in the ischemic core, in MCAO rats. Atorvastatin pretreatment prevented these increases, blunted expression of membrane subunit gp91(phox), and prevented translocation of cytoplasmic subunit p47(phox) to the membrane in the penumbra 2 h after reperfusion. Consequently, cerebral infarct volume was significantly reduced in atorvastatin-treated compared with nontreated MCAO rats 24 h after reperfusion. These results indicate that atorvastatin protects against cerebral infarction via inhibition of NADPH oxidase-derived superoxide in transient focal ischemia.

Background: Participants enrolled in the Optic Neuritis Treatment Trial have been observed for more than a decade to assess the relationship between optic neuritis and the development of clinically definite multiple sclerosis., Objective: To assess neurologic disability 10 to 12 years after an initial episode of optic neuritis., Design: Longitudinal follow-up of a clinical trial., Setting: Fourteen Optic Neuritis Treatment Trial clinical centers performed standardized neurologic examinations, including an assessment of neurologic disability., Participants: One hundred twenty-seven patients who had developed clinically definite multiple sclerosis., Main Outcome Measures: Functional Systems Scale and Expanded Disability Status Scale., Results: The disability of most patients was mild, with 65% of patients having an Expanded Disability Status Scale score lower than 3.0. The degree of disability appeared to be unrelated to whether the baseline magnetic resonance imaging scan was lesion-free or showed lesions (P =.51). Among patients with baseline lesions, the degree of disability was unrelated to the number of lesions that were present on the scan (P =.14). Two patients died owing to severe multiple sclerosis, one of whom had no lesions revealed on the baseline scan., Conclusion: Most patients who develop clinically definite multiple sclerosis following an initial episode of optic neuritis will have a relatively benign course for at least 10 years.

Purpose: To assess visual function more than 10 years after an episode of optic neuritis in patients enrolled in the Optic Neuritis Treatment Trial., Design: Longitudinal follow-up of a randomized clinical trial., Methods: Vision testing included measures of visual acuity, contrast sensitivity, and visual field. Quality of life was assessed with the National Eye Institute Visual Function Questionnaire., Results: Examinations were completed on 319 patients. In most patients, visual function test results in the eyes that experienced optic neuritis at study entry ("affected eyes") were normal or only slightly abnormal after 9.9 to 13.7 years. Visual acuity in the affected eyes was >or=20/20 in 74%, 20/25 to 20/40 in 18%, <20/40 to 20/200 in 5%, and <20/200 in 3%. On average, visual function was worse in patients with multiple sclerosis (MS) than in those without MS. Recurrent optic neuritis in either eye occurred in 35% of patients. Such attacks were more frequent in patients with MS (P <.001). The National Eye Institute Visual Function Questionnaire scores were lower when visual acuity was abnormal and when MS was present., Conclusions: Most patients retained good to excellent vision more than 10 years after an attack of optic neuritis. Recurrences were more frequent in patients with MS.

Objective: To identify factors associated with a high and low risk of developing multiple sclerosis after an initial episode of optic neuritis., Methods: Three hundred eighty-eight patients who experienced acute optic neuritis between July 1, 1988, and June 30, 1991, were followed up prospectively for the development of multiple sclerosis. Consenting patients were reassessed after 10 to 13 years., Results: The 10-year risk of multiple sclerosis was 38% (95% confidence interval, 33%-43%). Patients (160) who had 1 or more typical lesions on the baseline magnetic resonance imaging (MRI) scan of the brain had a 56% risk; those with no lesions (191) had a 22% risk (P<.001, log rank test). Among the patients who had no lesions on MRI, male gender and optic disc swelling were associated with a lower risk of multiple sclerosis, as was the presence of the following atypical features for optic neuritis: no light perception vision; absence of pain; and ophthalmoscopic findings of severe optic disc edema, peripapillary hemorrhages, or retinal exudates., Conclusions: The 10-year risk of multiple sclerosis following an initial episode of acute optic neuritis is significantly higher if there is a single brain MRI lesion; higher numbers of lesions do not appreciably increase that risk. However, even when brain lesions are seen on MRI, more than 40% of the patients will not develop clinical multiple sclerosis after 10 years. In the absence of MRI lesions, certain demographic and clinical features seem to predict a very low likelihood of developing multiple sclerosis. This natural history information is a critical input for estimating a patient's 10-year multiple sclerosis risk and for weighing the benefit of initiating prophylactic treatment at the time of optic neuritis or other initial demyelinating events in the central nervous system.

Background: Migraineurs with visual aura are highly susceptible to illusions and visual distortion and are particularly sensitive to a pattern of regularly spaced parallel lines or stripes., Purpose: To determine whether the high degree of susceptibility to illusions and visual distortion in migraineurs with aura is associated with hyperneurological activity of the occipital cortex., Methods: In order to investigate any relationships among neuronal activity, spatial frequency of square-wave gratings, and self-described visual distortion, we investigated the neuronal and psychophysical responses to square-wave gratings in migraineurs with visual aura and in nonheadache controls., Results: Square-wave gratings provoked various types of visual distortion and illusions and induced a hyperneuronal response in the visual cortex of migraineurs with visual aura, a response that strongly depended upon the stimulus spatial frequency., Conclusion: The hyperneuronal activity of the occipital cortex is consistent with general cortical hyperexcitability in migraine.

A 67-year-old woman with a history of chronic headache and recent removal of two squamous cell lesions from her forehead presented with left facial pain and diplopia. A diagnosis of Tolosa-Hunt syndrome was made based on clinical presentation and imaging studies. When the patient did not respond to steroids, further studies were done, including biopsy, which revealed perineural spread of squamous cell carcinoma.

Each year brings new scientific knowledge that builds on itself in a geometric fashion. Ocular motility basic and clinical neurosciences continue to advance with this accelerating pace. The years 1997 and 1998 brought new knowledge to the motility world. This review focuses on the clinical advances within this realm. Part I of this review appeared in the June 2000 (20:2) issue.

A retrospective chart review was performed for identification of patients with isolated internuclear ophthalmoplegia (INO) postcardiac catheterization from two neuro-ophthalmology units. Of the 110 patients with a diagnosis of INO who were evaluated during the observation period, five patients (4.5%) demonstrated relatively isolated INO occurring in the perioperative period of a cardiac endovascular procedure. These five patients underwent diagnostic catheterization alone (three patients), balloon angioplasty (one patient), or stent placement (one patient). All patients improved, with resolution of diplopia in primary position after a mean period of 82 days. The occurrence of INO in the postcardiac catheterization setting is not uncommon, and it appears to be related to dorsal pontine ischemia. The pontomesencephalic medial longitudinal fasciculus is supplied by small-caliber perforating end-arteries from the basilar trunk, which increases selective vulnerability of this area. Cardiac catheterization may precipitate microemboli involving these vessels, leading to internuclear ophthalmoplegia.

Although optic nerve enhancement may be seen in magnetic resonance imaging of radiation-induced ischemic optic neuropathy, similar enhancement in ischemic optic neuropathy has not been previously reported in the English-language neuroophthalmologic literature. We report three cases of optic nerve enhancement in biopsy-proven arteritic ischemic optic neuropathy. Clinicians should consider giant cell arteritis in the differential diagnosis of an optic neuropathy with optic nerve enhancement on magnetic resonance imaging.

In acute monosymptomatic optic neuritis, treatment with oral prednisone alone should be avoided. Therapy with intravenous methylprednisolone (1 g/day for 3 days) followed by 11 days of oral prednisone (1 mg/kg with a short taper) should be considered instead. This is particularly true if a patient considers accelerated visual recovery to be particularly urgent or if magnetic resonance imaging (MRI) demonstrates three or more signal abnormalities consistent with demyelination. In patients without a diagnosis of clinically definite multiple sclerosis (CDMS), an MRI should be considered to assess the prognosis for developing multiple sclerosis (MS) and to eliminate other causes of optic neuropathy. Foregoing an MRI or steroid treatment is an acceptable option. Chest x-ray, blood tests, and lumbar puncture are not necessary in evaluating patients with typical clinical features of optic neuritis. These tests may be appropriate, however, for patients who are about to undergo corticosteroid therapy, which could complicate or mask an unrecognized condition.

The predictive value of CSF oligoclonal banding for the development of clinically definite MS (CDMS) within 5 years after optic neuritis was assessed in 76 patients enrolled in the Optic Neuritis Treatment Trial. The presence of oligoclonal bands was associated with the development of CDMS (p = 0.02). However, the results suggest that CSF analysis is only useful in the risk assessment of optic neuritis patients when brain MRI is normal and is not of predictive value when brain MRI lesions are present at the time of optic neuritis.

Neuro-imaging is an essential part of the evaluation in patients with neuro-ophthalmologic disorders. Over the last two decades enormous advances in this area have been made allowing noninvasive evaluation of the orbit and brain. The idea of using nuclear magnetic resonance technology to produce images rather than the ionizing radiation of computed tomography (CT) began to emerge clinically in the late 1970s and early 1980s. Although the quality of early magnetic resonance imaging (MRI) scans was not much better than CT images, by the early 1990s, it became obvious that MRI had particular strength in identifying lesions in the posterior fossa, and demyelinating plaques. With advances in magnetic strength, computer software, surface coils, contrast medium, and more attention to the basic physics of magnet technology, the clarity of MR images improved dramatically. Recent advances in CT scanning (spiral and three-dimensional CT) and MRI (functional MRI and cine MRI) continue to affect significant changes in the discipline of neuro-ophthalmology. Furthermore, advances in MR angiography promise to allow excellent and noninvasive analysis of the cerebral vasculature. This review highlights the recent advances in neuro-imaging.

The transient pattern electroretinogram (PERG) was recorded from 16 patients with acute optic neuritis and from 13 patients with acute non-arteritic anterior ischemic optic neuropathy (AION). All patients were tested within 35 days from the the onset of visual symptoms and all had significant central visual field abnormalities in their affected eyes as quantified by automated perimetry. Analysis of the PERGs showed that the amplitude of the N95 peak was abnormally reduced for each eye affected with AION while it remained normal in optic neuritis. No significant alteration in P50 amplitude was observed in either condition. The loss of N95 amplitude in AION was highly correlated with the average depth of visual field loss (in decibels) within a radius of 10 degrees of fixation. These results suggest that PERG could be used early in the course of optic neuropathy to distinguish optic neuritis from AION in those cases for which the diagnosis is still uncertain after the clinical examination.

The peak latency of the pattern-reversal visual evoked potential is a sensitive measure of conduction delay in the optic nerve caused by demyelination. Despite its clinical utility, the pattern-reversal visual evoked potential has not previously been used in multicenter clinical trials, presumably because of difficulty in standardizing conditions between centers. To establish whether the pattern-reversal visual evoked potential could be adequately standardized for use as a measure in multicenter therapeutic trials for optic neuropathy or multiple sclerosis, stimulus and recording variables were equated at four centers and pattern-reversal visual evoked potentials were recorded from 64 normal subjects and 15 patients with resolved optic neuritis. Results showed equivalent latency and amplitude data from all centers, suggesting that stimulus and recording variables can be satisfactorily standardized for multicenter clinical trials. N70 and P100 peak latencies and N70-P100 interocular amplitude difference were sensitive measures of resolved optic neuritis.

Background: Optic neuritis is often the first clinical manifestation of multiple sclerosis, but little is known about the effect of corticosteroid treatment for optic neuritis on the subsequent risk of multiple sclerosis., Methods: We conducted a multicenter study in which 389 patients with acute optic neuritis (and without known multiple sclerosis) were randomly assigned to receive intravenous methylprednisolone (250 mg every six hours) for 3 days followed by oral prednisone (1 mg per kilogram of body weight) for 11 days, oral prednisone (1 mg per kilogram) alone for 14 days, or placebo for 14 days. Neurologic status was assessed over a period of two to four years. The patients in the first group were hospitalized for three days; the others were treated as outpatients., Results: Definite multiple sclerosis developed within the first two years in 7.5 percent of the intravenous-methyl-prednisolone group (134 patients), 14.7 percent of the oral-prednisone group (129 patients), and 16.7 percent of the placebo group (126 patients). The adjusted rate ratio for the development of definite multiple sclerosis within two years in the intravenous-methylprednisolone group was 0.34 (95 percent confidence interval, 0.16 to 0.74) as compared with the placebo group and 0.38 (95 percent confidence interval, 0.17 to 0.83) as compared with the oral-prednisone group. The beneficial effect of the intravenous-steroid regimen appeared to lessen after the first two years of follow-up. Signal abnormalities on magnetic resonance imaging (MRI) of the brain were a strong indication of risk for the development of definite multiple sclerosis (adjusted rate ratio in patients with three or more lesions, 5.53; 95 percent confidence interval, 2.41 to 12.66). The beneficial effect of treatment was most apparent in patients with abnormal MRI scans at entry., Conclusions: In patients with acute optic neuritis, treatment with a three-day course of high-dose intravenous methylprednisolone (followed by a short course of prednisone) reduces the rate of development of multiple sclerosis over a two-year period.

Objective: Changes in the brain on magnetic resonance images are common in patients with optic neuritis even when there is no other clinical evidence of multiple sclerosis. The current study was designed to determine systematically the prevalence of brain abnormalities on magnetic resonance images in the patients entered into the Optic Neuritis Treatment Trial., Design: Prospective multicenter clinical trial., Setting: Referral centers., Patients and Methods: Brain magnetic resonance images from 418 patients with acute optic neuritis (77% women; mean age, 32.0 years) were evaluated at a central reading center with the use of a standardized classification system (ranging from 0 for normal to IV for most extensive changes)., Results: Of the scans, 40.9% were classified as grade 0, 10.8% as grade I, 9.1% as grade II, 6.7% as grade III, and 32.5% as grade IV. For patients with isolated (monosymptomatic) optic neuritis, 26.7% had two or more lesions., Conclusions: We found a lower prevalence of brain magnetic resonance imaging abnormalities in isolated optic neuritis than previous studies have reported. This likely is due to our study having a higher degree of standardization of patient inclusion criteria, which limited patient selection bias.

Recent work has suggested that the N95 peak of the transient pattern electroretinogram (PERG) may be a more sensitive indicator of the late stages of retinal function prior to optic nerve activation than the P50 peak. In this report, we show that a new measure of N95 amplitude, based on digital filtering methods to identify a non-linear baseline before measurement, greatly reduced the amplitude variation in a population of 50 normal subjects when compared with two other plausible measures. We then used that new measure to follow the time course of N95 amplitudes in 12 optic neuritis patients. It was found that maintenance of a normal N95 amplitude at 6 months after onset of optic neuritis was always associated with excellent clinical recovery as measured by visual fields, acuity, presence or absence of an afferent pupil and optic atrophy, and contrast sensitivity (CS). Loss of N95 amplitude to below laboratory limits of normal was associated with abnormalities in these indicators of visual function. This study supports the idea that the N95 peak represents retinal ganglion cell function.

The pattern electroretinogram (PERG) is a small electrical response of the retina to a reversing checkerboard pattern, usually less than 6 microV in amplitude. Unfortunately, the PERG can be obscured by artifacts such as blinks, eye movements, poor fixation, and amplifier saturation. Amplitude criterion artifact rejection systems found on commercial signal averagers eliminate large amplitude artifacts but are insensitive to small amplitude artifacts associated with amplifier saturation. Such saturation often occurs for several recording sweeps after large amplitude signals such as eye blinks are rejected. The presence of post-saturation artifacts complicates clinical PERG analysis. In this paper we describe procedures to remove these small amplitude artifacts from the PERG. These include computer selection of inputs for averaging and use of tracings with small input numbers to approximate PERG amplitudes. These procedures greatly reduce the variability of PERG amplitudes in the normal population, making PERG amplitude a more reliable clinical measure.

Background and Methods: The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period., Results: Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did not differ from that in the placebo group. In addition, the rate of new episodes of optic neuritis in either eye was higher in the group receiving oral prednisone, but not the group receiving intravenous methylprednisolone, than in the placebo group (relative risk for oral prednisone vs. placebo, 1.79; 95 percent confidence interval, 1.08 to 2.95)., Conclusions: Intravenous methylprednisolone followed by oral prednisone speeds the recovery of visual loss due to optic neuritis and results in slightly better vision at six months. Oral prednisone alone, as prescribed in this study, is an ineffective treatment and increases the risk of new episodes of optic neuritis.

We have reviewed the charts of 45 neuro-ophthamological patients diagnosed with 79 monocular visual field or visual acuity losses secondary to non-organic etiology. Our aim was to determine the percentage of patients that have improvement in vision. As part of the protocol, all patients had magnetic resonance images, pattern visual evoked potentials, and flash electroretinography in addition to complete neuro-ophthalmological examinations. A single physician performed both the initial and follow-up examinations of all patients. Thirty-three percent of these patients had visual field defects only, 62% had both visual field defects and visual acuity defects, and 5% had only visual acuity defects. After organic disease was ruled out, all were given a timetable for recovery and clear reassurance regarding their prognoses for visual recovery. Seventy-eight percent of these patients showed improvement or were normal, while 22% showed no improvement. Younger patients without obvious psychiatric disorder had better prognoses than older patients.

Breast cancer metastatic to the eye is a common entity occurring in up to 30% of women with metastatic disease. The prevalence of this lesion is not appreciated because of the dominant clinical picture of metastases occurring in other organs. The diagnosis should be suspected in any women with a history of breast cancer and any visual symptoms, particularly metamorphopsia and scotomata. A thorough ophthalmologic evaluation, aided by ultrasonography, computed tomography, or magnetic resonance scanning, usually confirms the diagnosis. Early treatment with radiation therapy can alleviate symptoms and control local disease. The recognition and treatment of this disorder is important in maximizing the quality of life in patients with metastatic breast cancer, especially because newer treatment regimens prolong survival and thereby increase the chances for ocular metastasis.

Sixteen normal subjects and three patients with optic neuritis were studied to determine the effect of decreased retinal illumination on simultaneously recorded pattern electroretinograms (PERG) and visual-evoked potentials (VEP). Using neutral-density filters (NDF), it was found that linear modeling is an excellent fit for VEP/PERG amplitudes and latencies as log functions of retinal illumination, both for individual eyes and averages of pooled data. Within narrow statistical limits, regression slopes show that mean PERG B-wave and VEP P100 latencies are affected almost identically by decreased illumination, leaving the mean retinocortical time (RCT) virtually unchanged. However, mean B-wave amplitude was greatly reduced at retinal illuminations at which P100 amplitude was unaffected. Of clinical significance was that these latency and amplitude effects were found in each eye tested, whether normal or pathologic. In particular, the RCT in normal subjects was never found to be statistically abnormal due to decreased retinal illumination, and it faithfully represented the optic nerve lesion in the patients with optic neuritis. This result was applied to a population of eight patients with uncomplicated cataracts. The significance of these results is discussed.

We studied 20 patients with acute optic neuritis prospectively for 12 months. Visual fields, color vision, and VEPs to 15' checks were initially abnormal in all patients. Visual acuity was abnormal in 90% and contrast sensitivity in 95% of patients. We devised a graded visual impairment scale (GVIS) to include all visual functions tested. Complete recovery of visual function occurred in 65% of cases. Recovery in the majority of patients was rapid and complete within the first 2 months. In some patients, improvement continued over 6 months. The initial classification on the GVIS was significantly correlated with the final outcome. Patients initially classified as having moderate visual impairment recovered completely or improved to near normal vision. Sixty percent of patients initially classified as total or severe blindness had permanent visual impairment. VEP latency remained prolonged in 19 patients, even when their vision had returned to normal, and is a reliable indicator of resolved optic neuritis.

This study compared visual evoked potentials (VEP) and contrast sensitivity (CS) as methods of detecting visual loss in 15 women with acute onset of pseudotumor cerebri (PTC). A total of 24 out of 30 eyes (14 of 15 patients) had a visual disturbance. Neuro-ophthalmologic examination demonstrated abnormal Goldmann visual fields in 13 of 30 eyes. CS was abnormal in 18 eyes; VEP was abnormal in five eyes. Nine eyes had visual loss detected by CS only, five by perimetry only, and one had the VEP as the sole abnormality. CS was a valuable adjunct to perimetry in PTC whereas VEP was rarely helpful.

We report a 2-year prospective study of the electroretinographic response to reversal of checkerboard patterns (P-ERG) obtained in 63 eyes with acute optic nerve lesions. The aim of the study was to document the value of P-ERG regarding diagnosis and prognosis of four types of optic neuropathy: optic neuritis, compressive or hereditary optic atrophy, and traumatic optic neuropathy. We documented visual loss by neuro-ophthalmologic examination and recorded pattern-reversal visual evoked potentials (P-VEP). The initial P-ERG was normal to large- and medium-sized checks in 89% and the P-VEP abnormal in 94% of eyes with acute optic nerve lesions. Forty-six eyes were followed for up to 2 years. Two groups emerged. Group A (n = 17) gradually and permanently had significant reduction of the P-ERG to three separate check sizes. All 17 had no improvement in acuity better than 20/100, retained centrocecal scotomas, and developed optic atrophy. In group B (n = 29) the P-ERG remained within normal limits to one or more check sizes. Twenty-two of these eyes recovered acuity to 20/25 or better and had resolution of the field defect. The data showed that P-VEP was superior to P-ERG in diagnosis of acute and chronic optic nerve lesions. However, significant reduction of the b-wave of the P-ERG to three separate check sizes correlated closely with failure of visual recovery and the eventual development of severe optic atrophy, suggesting a prognostic value for P-ERG in optic neuropathy.