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Purpose: We describe a new surgical treatment for lateropulsion and torticollis arising from an ocular tilt reaction., Methods: Visual sensory parameters and postural abnormalities were retrospectively reviewed in a patient with a partial ocular tilt reaction who was treated with horizontal transposition of the vertical rectus muscles to surgically augment preexisting binocular torsion., Results: Torsional augmentation eliminated the sensation of lateropulsion and produced almost complete resolution of her torticollis and associated body tilt., Conclusions: Binocular torsional augmentation can be used to reduce both lateropulsion and torticollis in the setting of an ocular tilt reaction., (Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To report fusional divergence in children with intermittent exotropia (XT)., Methods: We retrospectively studied 32 children with intermittent XT (aged 4 to 13 years) and 38 visually normal non-strabismic children. Fusional divergence break points were measured using a prism bar, starting from a naturally fused state. Distribution of divergence break points was evaluated. Subnormal fusional divergence was defined as below the 5th percentile in visually normal children. In children with intermittent XT, correlations were calculated between divergence break point and control score, angle of deviation, and convergence break point., Results: The distribution of fusional divergence break points in intermittent XT was normal at near but bimodal at distance. Nine percent had subnormal divergence (<10 prism diopters [pd]) at near, and 48% (<6 pd) at distance. There was a moderate correlation between divergence and convergence break points at near (r = 0.44; P=.01) but only weak inverse correlations between divergence break point and control score (r = -0.29; P = .11) and divergence break point and angle of deviation (r = 0.08; P = . 68) at near., Conclusions: Most children with intermittent XT have normal near fusional divergence but nearly half have reduced distance fusional divergence. We found the magnitude of near divergence break point correlates with the magnitude of near convergence break point. Measuring divergence may provide useful information about fragility of fusion in patients with intermittent XT. Future studies of fusional divergence are needed to establish whether common measurement methods represent true divergence amplitudes and whether such measures have prognostic significance.

Background: Halpern syndrome is a vestibular disorder characterized by vertigo, a tendency to tilt to one side, and spontaneous closure of the ipsilateral eye. Symptoms are exacerbated during monocular viewing with the ipsilateral eye and alleviated during monocular viewing with the contralateral eye. It has long been attributed to a mismatch between visual and vestibular input., Methods: Reexamination of Halpern syndrome in light of new concepts of visuo-vestibular disease., Results: The visually dependent postural disequilibrium in Halpern syndrome recapitulates the dorsal light reflex in fish, in which unequal luminance input to the two eyes evokes a tilt of the body toward the brighter side., Conclusion: Patients with Halpern syndrome close one eye to restore vertical postural orientation. Halpern syndrome may result from reactivation the dorsal light reflex in the setting of central vestibular disease.

A 5-month-old boy developed a large-amplitude, horizontal pendular flutterlike oscillation of the eyes, when placed in the supine position. Magnetic resonance imaging (MRI) 1 month earlier had shown isolated thickening of the optic nerves and chiasm without other central nervous system signal abnormalities. Repeat MRI at 5½ months of age showed a constellation of central nervous system signal abnormalities suggestive of Krabbe disease. The diagnosis was confirmed with serum enzymatic and genetic testing. This case demonstrates that positional ocular flutter can be a presenting sign of Krabbe disease and reinforces the observation that isolated thickening of the anterior visual pathways can antedate other central nervous system abnormalities on MRI., (Copyright © 2011. Published by Mosby, Inc.)

A 21-year-old Kuwaiti man had cerebral palsy, retinitis pigmentosa, hypertension, and renal failure. His younger brother and sister displayed similar findings. Ocular motility examination disclosed aperiodic alternating skew deviation in the patient and his younger brother. Magnetic resonance imaging showed hypoplasia of the superior cerebellar vermis with a "molar tooth" sign. Genetic testing confirmed an AHPI gene mutation on chromosome 6p23.3 in the patient and his siblings, confirming the diagnosis of Joubert syndrome. This case demonstrates the need to consider the diagnosis of Joubert syndrome in adults with retinitis pigmentosa or familial alternating skew deviation., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To investigate the course of near stereoacuity in a cohort of children with untreated intermittent exotropia., Methods: A total of 95 children ages 2-16 years with untreated intermittent exotropia were identified who had measurements of near stereoacuity using the Preschool Randot (PSR) test on at least 2 successive examinations, with no intervening treatment. Deterioration was defined as a drop of at least 3 octaves, as determined by test-retest data. Alternative secondary analyses were performed defining deterioration as a drop of at least 2 octaves or as a transition from normal to subnormal. The main outcome measure was the deterioration rate calculated at 1 and 2 years using a Kaplan-Meier survival analysis., Results: For near stereoacuity, the rate of decreasing 3 or more octaves was 2% (95% CI, 0%-6%) at 1 year and 7% (95% CI, 0%-16%) at 2 years. The alternative secondary analyses revealed similar low deterioration rates. In most cases of apparent deterioration, near stereoacuity returned to baseline levels without treatment., Conclusions: For children with untreated intermittent exotropia, deterioration in near stereoacuity at 1 and 2 years is infrequent. Therefore, performing surgery for intermittent exotropia to proactively prevent deterioration of near stereoacuity cannot be justified. Retesting stereoacuity is critical to determine whether any measured decrease in stereoacuity is real., (Copyright © 2011 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To evaluate fusional convergence and associations with control in children with intermittent exotropia (XT)., Design: Retrospective case series., Methods: Sixty-four children (median age 7 years) with intermittent XT were identified with measures of angle of deviation, control (using a previously reported 0-to-5-point scale), and fusional convergence at a single examination. Total convergence was defined as the sum of the angle of deviation plus additional convergence in reserve. Mean values were compared with published normals. A fusion reserve ratio was calculated as "convergence reserve divided by angle of deviation." Relationships of control score with total amplitude, reserve, recovery, and fusion reserve ratio were assessed using Spearman rank correlations., Results: Convergence differed from normals in children with intermittent XT: total convergence was higher at distance (33 prism diopters [pd] vs 17 pd, P<.0001) and near (38 pd vs 18 pd, P<.0001) whereas convergence reserve was lower at distance (7 pd vs 17 pd; P<.0001). There was a strong correlation between fusion reserve ratio and control score at distance (R=-0.75, P<.0001) and near (R=-0.66, P<.0001)., Conclusions: Children with intermittent XT have subnormal convergence reserves at distance. The fusion reserve ratio correlates well with control and may be useful in grading the severity of intermittent XT., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Monocular elevation deficiency (or "double elevator" palsy) is a descriptive term denoting a congenital deficiency of monocular elevation that is equal in abduction and adduction. We describe a child with monocular elevation deficiency who displayed tethering and buckling of the central lower eyelid in downgaze. We caution that this manifestation of inferior rectus contracture can simulate impaired infraduction in the involved eye.

Vertical ocular motility disorders of supranuclear origin can be congenital or acquired later in life. They produce complex forms of strabismus that require specialized surgical management to restore ocular alignment, eliminate torticollis, treat diplopia, and restore normal lid position. For each condition, surgical management must be individualized to address multiple impediments to fusion. This article outlines the surgical management of three common types of supranuclear vertical strabismus.

Purpose: To identify the inherent complexities in assigning causation in the evolving systems era of pediatric ophthalmology., Design: Philosophical analysis of causality as it pertains to understanding the pathogenesis of pediatric strabismus and nystagmus., Methods: Review of general philosophical problems and profession-related differences in assigning causality as they apply to current controversies in pediatric ophthalmology., Results: Medicine is evolving into a systems era based on a complex network of interacting components including internal systems, gene expression, behavioral factors, and environmental factors. In this context, any definition of cause (epidemiologic, mechanical, physiologic, environmental, genetic, epigenetic, etc.) becomes a function of one's reference point. Thus, as medical science becomes more precise, causality seems to recede. Most current controversies in pediatric ophthalmology are reducible to simple questions of cause and effect. Strabismus and nystagmus often develop without apparent cause, providing a formidable challenge to deciphering disease pathogenesis and assigning causality. In these conditions, assigning causation is impeded by a tendency to confuse links and correlations with cause and effect, an inherent tendency to conflate form and function, the use of terminology that transposes cause and effect, and by our tendency to think linearly about problems that are fundamentally circular., Conclusions: Our tendency to think linearly creates a self-referential system that influences our understanding of cause and effect. Neural activity is characterized by feedback loops and bidirectionality. Understanding neurodevelopmental strabismus and nystagmus requires a dynamic, circular view of causality., (Copyright (c) 2010 Elsevier Inc. All rights reserved.)

Purpose: The course of intermittent exotropia and response to surgery may depend on whether there is underlying monofixation. The purpose of this study was to report the prevalence of sensory monofixation in intermittent exotropia using different stereotests and to determine the risk of misclassifying monofixation based on a single administration of each test., Design: Retrospective case review of children with intermittent exotropia., Methods: Forty-four children were identified for whom Preschool Randot, Frisby, and Titmus stereoacuity were measured at a single examination. Ninety-two children were identified with near stereoacuity measured on 2 sequential visits (Preschool Randot, n = 73; Frisby, n = 66; and Titmus, n = 40). Monofixation was defined as stereoacuity worse than previously published age-referenced normal thresholds, bifixation was defined as at least 40 arc seconds, and uncertain was defined as within normal range for age but worse than 40 arc seconds., Results: In children measured by all 3 tests on the same visit, sensory monofixation occurred in 36% using Preschool Randot, in 48% using Titmus, and in 55% using Frisby (P > .1 for each comparison). There was poor agreement between Frisby and Preschool Randot when classifying monofixation in individual patients (P = .05). In children measured on sequential visits, misclassification occurred in 5% with Preschool Randot, in 13% with Titmus, and in 23% with Frisby (Preschool Randot vs Frisby, P = .005)., Conclusions: Classification of monofixation depends on the stereotest used. Regardless of the stereotest, there is a risk of misclassifying monofixation on a single assessment. Potential misclassification needs to be considered in clinical practice and in study design., (2010 Elsevier Inc. All rights reserved.)

Purpose: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas., Design: Nonrandomized retrospective case series., Participants: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers., Methods: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI., Main Outcome Measures: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia., Results: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia., Conclusions: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful.

Purpose: To evaluate a group of patients with isolated, early-onset, bilateral optic neuropathy for genetic and biochemical evidence of mitochondrial diseases., Methods: This case-control study involved 21 patients, 159 control subjects for mitochondrial (mt)DNA sequencing, and 40 control subjects for relative mtDNA content. Patients were identified who had had decreased vision since childhood due to bilateral optic neuropathy characterized by central visual loss with no other major neurologic or ocular abnormality and no clinical evidence of a mitochondrial syndrome. Clinical examination, electroretinograms, and neuroimaging were performed; the entire mtDNA coding region was sequenced in leukocytes of all patients; relative mtDNA content was assessed; and OPA1 and OPA3 nuclear genes associated with dominant and recessive optic atrophy, respectively, were sequenced. Main outcome measures were clinical description, nonsynonymous (NS) mtDNA nucleotide changes, relative mtDNA content, and OPA1 and OPA3 nucleotide changes., Results: Twenty-one unrelated patients (16 male and 5 female; mean age at first examination 13.6 years) had bilateral moderate, relatively symmetric optic neuropathies and normal neurologic examinations other than strabismus in 11 and congenital nystagmus in 9. Four patients had optic nerve hypoplasia. One patient had the nt 11778 primary Leber hereditary optic neuropathy (LHON) mutation, and three others had mtDNA nucleotide changes predicted to be pathologic. The entire group had a small increase (6.7%) in relative mtDNA content of indeterminate statistical significance. No patient had a polymorphism or mutation of OPA1 or OPA3., Conclusions: A minority of these young patients with sporadic bilateral optic neuropathy had abnormalities of the mitochondrial parameters evaluated. This bilateral optic neuropathy may be due to other genetic, epigenetic, or environmental injury to the optic nerve or to mitochondrial defects not studied.

Rhombencephalosynapsis is a rare, sporadic, posterior fossa malformation characterized by agenesis of the cerebellar vermis, dorsal fusion of the cerebellar hemispheres, and fusion of the dentate nuclei and superior cerebellar peduncles. Ophthalmologic findings in patients with rhombencephalosynapsis have not been described in detail. Optic nerve and chiasmal hypoplasia were reported in two patients. Strabismus has been noted in several patients; however, specific details were not provided. We describe the association of A-pattern strabismus and superior oblique overaction with rhombencephalosynapsis.

Tonus refers to the effects of baseline innervation on musculature in the awake, alert state.(1) Since the normal anatomical resting position of the eyes is one of exodeviation, extraocular muscle tonus plays a vital physiologic role in establishing ocular alignment. Under normal conditions, binocular esotonus is superimposed on the baseline anatomical position of rest to maintain approximate ocular alignment, save for a minimal exophoria that is easily overcome by active convergence. When binocular visual input is preempted early in life, monocular fixation may give rise to a larger dissociated esotonus that gradually drives the 2 eyes into a "convergent" position, resulting in infantile esotropia.(2).

Objective: To examine the results of reversed fixation testing in patients who develop consecutive exotropia after surgery for infantile esotropia., Methods: The reversed fixation test was performed prospectively in 28 patients who developed consecutive exotropia after surgery for infantile esotropia. All patients were assessed for adduction weakness, latent nystagmus, dissociated vertical divergence, and neurologic disease., Results: A positive reversed fixation test, indicating the presence of dissociated horizontal deviation, was found in 14 of 28 patients (50%) with consecutive exotropia. In patients with dissociated horizontal deviation, the exodeviation was usually smaller with the nonpreferred eye fixating than with the preferred eye fixating, and smaller with the preferred eye fixating than during periods of visual inattention or under general anesthesia. Dissociated horizontal deviation correlated with the findings of dissociated vertical divergence, but not with asymmetric adduction weakness, latent nystagmus, or neurologic disease., Conclusions: Dissociated horizontal deviation is a clinical expression of dissociated esotonus. The common clinical presentation of dissociated horizontal deviation as an intermittent exodeviation of 1 eye results from the superimposition of a dissociated esotonus on a baseline exodeviation.

Objective: To document the resolution of oscillatory head movements following surgical realignment of the eyes in children with infantile esotropia and nystagmus., Method: Retrospective review of 3 children who had infantile esotropia, nystagmus, and unexplained head shaking or head nodding., Results: Strabismus surgery restored ocular alignment and produced resolution of the head shaking in all patients. In 1 patient, head shaking accompanied recurrence of the esotropia and again resolved following surgical realignment of the eyes., Conclusions: Head shaking or head nodding can rarely be associated with infantile esotropia and nystagmus. In this syndrome, surgical realignment of the eyes may produce simultaneous resolution of the head oscillations.

The reversed fixation test is a simple clinical test that was developed to visualize subtle dissociated deviations. In recent years, we have used this test to diagnose dissociated vertical divergence and dissociated horizontal deviation in the setting of complex strabismus. In this article, we describe our technique for performing and interpreting the reversed fixation test.

Purpose: To elucidate the pathophysiology of dissociated horizontal deviation., Methods: The reversed fixation test was performed prospectively in 28 patients who developed consecutive exotropia following horizontal extraocular muscle surgery for infantile esotropia. All patients were assessed for the presence of adduction weakness, latent nystagmus, dissociated vertical divergence, and neurologic disease., Results: A positive reversed fixation test, indicating the presence of dissociated horizontal deviation, was found in 14 of 28 patients (50%) with consecutive exotropia. In patients with dissociated horizontal deviation, the exodeviation was usually smaller with the nonpreferred eye fixating than with the preferred eye fixating, and smaller with the preferred eye fixating than during periods of visual inattention or under general anesthesia. Dissociated horizontal deviation correlated with the findings of dissociated vertical divergence, but not with asymmetric adduction weakness, latent nystagmus, or neurologic disease., Conclusions: Using reversed fixation testing, dissociated horizontal deviation can be detected in 50% of patients who develop consecutive exotropia following surgery for infantile esotropia. In this setting, monocular fixation with either eye superimposes a dissociated esotonus upon a baseline exodeviation. Fixation with the nonpreferred eye usually exerts greater esotonus than fixation with the preferred eye, producing an asymmetrical exodeviation during prism and alternate cover testing. Depending on the baseline anatomical position of the eyes, this dissociated esotonus can manifest as an intermittent exodeviation or an intermittent esodeviation. This unrecognized form of ocular motor dissociation may contribute to the pathogenesis of infantile esotropia.

Idiopathic congenital nystagmus is characterized by involuntary, periodic, predominantly horizontal oscillations of both eyes. We identified 22 mutations in FRMD7 in 26 families with X-linked idiopathic congenital nystagmus. Screening of 42 singleton cases of idiopathic congenital nystagmus (28 male, 14 females) yielded three mutations (7%). We found restricted expression of FRMD7 in human embryonic brain and developing neural retina, suggesting a specific role in the control of eye movement and gaze stability.

Skew deviation is a vertical misalignment of the eyes caused by damage to prenuclear vestibular input to ocular motor nuclei. The resultant vertical ocular deviation is relatively comitant in nature, and is usually seen in the context of brainstem or cerebellar injury from stroke, multiple sclerosis, or trauma. Skew deviation is usually accompanied by binocular torsion, torticollis, and a tilt in the subjective visual vertical. This constellation of findings has been termed the ocular tilt reaction. In the past two decades, a clinical localizing value for skew deviation has been assigned, and a cogent vestibular mechanism for comitant and incomitant variants of skew deviation has been proposed. Our understanding of skew deviation as a manifestation of central otolithic dysfunction in different planes of three-dimensional space is evolving. The similar spectrum of vertical ocular deviations arising in patients with congenital strabismus may further expand the nosology of skew deviation to include vergence abnormalities caused by the effects of early binocular visual imbalance on the developing visual system.

Purpose: To evaluate the efficacy of isolated inferior oblique muscle weakening in the treatment of superior oblique palsy., Methods: Forty-seven patients with superior oblique palsy underwent either single-muscle surgery (anteriorization or recession of the inferior oblique muscle) or two-muscle surgery (anteriorization of the inferior oblique muscle combined with recession of the contralateral inferior rectus muscle according to the amount of vertical deviation). In a retrospective noncomparative study the objective surgical effect was calculated as the difference between the deviation at the day before surgery and the deviations 6 weeks and at least 1 year after surgery. Pre- and postoperative sensorimotor status and subjective outcome were evaluated., Results: In patients who underwent isolated inferior oblique muscle surgery the mean preoperative vertical deviation decreased from 15+/-9 (distance)/16+/-10 (near) prism diopters (PD) (anteriorization) and 7+/-5 (distance)/9+/-8 (near) PD (recession) to 4+/-4 (distance)/4+/-6 (near) PD (anteriorization) and 2+/-2 (distance)/2+/-3 (near) PD (recession) at the 1-year follow-up. In patients who underwent two-muscle surgery the mean vertical deviation decreased from 20+/-11 (distance)/21+/-10 (near) PD preoperatively and 6+/-7 (distance)/6+/-6 (near) PD at 1-year follow-up. Subjective assessment showed excellent scores among the patients treated with single-muscle surgery and slightly lower but also favorable scores among the patients treated with combined techniques. A direct comparison of the different outcome scores was not possible because of the more difficult initial situation in patients who underwent combined surgery., Conclusions: Isolated inferior oblique muscle weakening is an effective treatment option for superior oblique palsy up to 15 PD of vertical deviation in primary position. Two-muscle surgery should be reserved for patients with larger vertical deviations.

Aim: To examine the association of distance-near disparity with neurological disease in children with intermittent exotropia., Methods: A retrospective analysis was performed of the medical records of all children with intermittent exotropia examined at the Arkansas Children's Hospital between 1989 and 2002. The study group consisted of children with intermittent exotropia who had a near deviation that exceeded the deviation at distance by at least 10 prism dioptres. The control group consisted of children with intermittent exotropia who had a distance deviation greater than or equal to the deviation at near. The main outcome measures were the prevalence of neurological abnormalities in the study and control groups., Results: Among the 29 patients in the study group, 19 (66%) had a history of concurrent neurological abnormalities. Associated neurological conditions included developmental delay (10 patients), attention deficit disorder (four patients), cerebral palsy (four patients), history of intracranial haemorrhage (four patients), periventricular leucomalacia (three patients), seizures (two patients), cortical visual impairment (two patients), hydrocephalus (one patient), history of anoxic brain damage (one patient), history of encephalitis (one patient), and autism (one patient). Among the 37 patients in the control group, seven (19%) had a history of concurrent neurological abnormalities. The difference in the prevalence of neurological disease between the study group and the control group was significant (p = 0.0002)., Conclusion: Intermittent exotropia increasing with near fixation is associated with neurological disease in children.

Background: Dissociated horizontal deviation is one of several conditions that manifest unequal horizontal deviations depending on which of the 2 eyes is fixating., Purpose: To describe the reversed fixation test as an essential tool to establish the diagnosis of dissociated horizontal deviation., Methods: Analysis of 4 case scenarios depicting the utility of the reversed fixation test., Results: The reversed fixation test distinguishes dissociated horizontal deviation from an unequal exodeviation of the 2 eyes resulting from postoperative slippage or weakness of a horizontal rectus muscle., Conclusion: The reversed fixation test is necessary to establish the diagnosis of dissociated horizontal deviation.

Infantile strabismus is accompanied by latent nystagmus, primary inferior oblique muscle overaction, and dissociated vertical divergence. If we examine the evolutionary underpinnings of these ocular rotations, we can construct a unifying mechanism for the sensorimotor abnormalities that arise in humans with infantile strabismus. Latent nystagmus, primary inferior oblique muscle overaction, and dissociated vertical divergence correspond to visual balancing reflexes that are operative in lateral-eyed animals in yaw, pitch, and roll, respectively. In humans with infantile strabismus, these subcortical visual reflexes are reactivated by a physiologic imbalance in binocular visual input, which resets central vestibular tone in 3-dimensional space. These visual reflexes reveal the evolutionary role of the eyes as sensory balance organs that can directly modulate central vestibular tone. Latent nystagmus, primary oblique muscle overaction, and dissociated vertical divergence should be reclassified as visuo-vestibular eye movements.

Purpose: To describe the clinical presentation and results of three-dimensional video-oculography of a patient with idiopathic microsaccadic opsoclonus., Design: Observational case report., Methods: Neuro-ophthalmologic examination and video-oculographic recording of horizontal, vertical, and torsional eye movements., Results: A 67-year-old man with intermittent blurred vision and oscillopsia, which persisted with monocular occlusion, over a five year period was found to have high frequency, small amplitude back-to-back multivectorial saccadic movements which were visible with slit lamp biomicroscopy and direct ophthalmoscopy. Video-oculography showed a 20 Hz, 0.2 to 1 degree pattern of horizontal, vertical, and torsional microsaccades. This microsaccadic disorder has persisted for five years with no etiology., Conclusions: Microsaccadic opsoclonus is an idiopathic disorder that presents with oscillopsia and intermittent blurred vision. The diagnosis can be confirmed with three-dimensional eye movement recordings.

Purpose: To document reversible corneal edema caused by amantadine in a pediatric patient., Methods: A 14-year-old boy with a neurologic tremor was referred for bilateral visual loss. Our examination disclosed bilateral corneal edema without ocular inflammation. Pachymetry confirmed significantly increased corneal thickness above 900 microm., Results: Review of the patient's medical information revealed recent institution of amantadine as a means to control the patient's tremor. On cessation of this agent, rapid resolution of corneal edema and recovery of visual acuity occurred. Repeat pachymetry measurement revealed normal corneal thickness., Conclusion: In cases of corneal edema and in the absence of any identifiable ocular causes, a review of toxic effects of systemic medication should be undertaken. Amantadine can cause corneal decompensation and needs to be considered as part of the differential diagnosis of corneal edema.

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.