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Purpose: The purpose of this study was to describe the clinical features, management, outcomes, and diagnostic pitfalls in a large series of patients with ocular neuromyotonia., Design: Retrospective cohort., Methods: Patients diagnosed with ocular neuromyotonia from January 1, 2004, through January 1, 2023, seen at one of the 3 Mayo Clinic sites in Rochester, MN, Scottsdale, AZ, and Jacksonville, FL, comprised the study population. We ascertained patients with ocular neuromyotonia through a search using the medical records database. Only patients with an observed episode of ocular neuromyotonia were included and the medical records were reviewed. The main outcome measures were clinical features and outcomes of patients with ocular neuromyotonia., Results: Forty-two patients who were diagnosed with ocular neuromyotonia were included. The median age was 58 years (range, 16-80 years). A history of cranial radiation therapy was present in 39 patients (93%). The sixth cranial nerve was involved in 31 patients (74%). Bilateral disease was found in 2 patients (5%). The median time from onset of diplopia to diagnosis was 8 months (range, 1 month-25 years), with a high rate of initial misdiagnosis in 52%. Twenty of 42 patients (48%) were treated with oral medication, of whom 95% had significant improvement or resolution of symptoms., Conclusion: Prior cranial irradiation is the most common cause for ocular neuromyotonia, affecting the sixth cranial nerve most often. Although delayed and initial misdiagnosis is common, most patients show improved symptoms on medical treatment., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: While large language models (LLMs) are increasingly used in medicine, their effectiveness compared with human experts remains unclear. This study evaluates the quality and empathy of Expert + AI, human experts, and LLM responses in neuro-ophthalmology., Methods: This randomized, masked, multicenter cross-sectional study was conducted from June to July 2023. We randomly assigned 21 neuro-ophthalmology questions to 13 experts. Each expert provided an answer and then edited a ChatGPT-4-generated response, timing both tasks. In addition, 5 LLMs (ChatGPT-3.5, ChatGPT-4, Claude 2, Bing, Bard) generated responses. Anonymized and randomized responses from Expert + AI, human experts, and LLMs were evaluated by the remaining 12 experts. The main outcome was the mean score for quality and empathy, rated on a 1-5 scale., Results: Significant differences existed between response types for both quality and empathy (P < 0.0001, P < 0.0001). For quality, Expert + AI (4.16 ± 0.81) performed the best, followed by GPT-4 (4.04 ± 0.92), GPT-3.5 (3.99 ± 0.87), Claude (3.6 ± 1.09), Expert (3.56 ± 1.01), Bard (3.5 ± 1.15), and Bing (3.04 ± 1.12). For empathy, Expert + AI (3.63 ± 0.87) had the highest score, followed by GPT-4 (3.6 ± 0.88), Bard (3.54 ± 0.89), GPT-3.5 (3.5 ± 0.83), Bing (3.27 ± 1.03), Expert (3.26 ± 1.08), and Claude (3.11 ± 0.78). For quality (P < 0.0001) and empathy (P = 0.002), Expert + AI performed better than Expert. Time taken for expert-created and expert-edited LLM responses was similar (P = 0.75)., Conclusions: Expert-edited LLM responses had the highest expert-determined ratings of quality and empathy warranting further exploration of their potential benefits in clinical settings., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 North American Neuro-Ophthalmology Society.)

Purpose: To elucidate the induced effects of horizontal strabismus on the Bielschowsky Head Tilt Test (BHTT)., Design: Prospective clinical study., Methods: Prospective analysis of BHTT testing in 85 patients with exotropia and 71 patients with esotropia who were examined in a strabismus clinic., Results: Eighty-four of 85 patients with exotropia (98.82%) showed a positive BHTT with an induced hyperdeviation on the side of the tilt (to both sides in 67% and to one side in 32%). Fifty-seven of 71 patients with esotropia (80.2%) showed a positive BHTT with an induced hypodeviation on the side of the tilt (to both sides in 57.7% and to one side in 22.5%). These induced vertical deviations were greater in patients with larger horizontal deviations and in those with constant rather than intermittent deviations; however, they were not influenced by the presence or absence of associated primary oblique muscle overaction., Conclusions: Exotropia and esotropia produce hyperdeviations during BHTT testing, with a hyperdeviation on the side of the tilt observed in patients with exotropia, and hypotropia on the side of the tilt observed in patients with esotropia. These diametrical results are not attributable to any preexistent alteration of neurologic output inherent to these two forms of horizontal strabismus or to associated torsion. Rather, they arise directly from the altered anatomical positions of the two eyes, which cause the eyes to approximate their visual axes more closely to the vertical rectus muscles (in exotropia) and the oblique muscles (in esotropia), enabling the vertical actions of specific cyclovertical muscles to predominate in response to altered utricular output generated by the BHTT.

Competing Interests: The authors report no conflicts of interest.

Objective: To determine the appropriateness of ophthalmology recommendations from an online chat-based artificial intelligence model to ophthalmology questions., Patients and Methods: Cross-sectional qualitative study from April 1, 2023, to April 30, 2023. A total of 192 questions were generated spanning all ophthalmic subspecialties. Each question was posed to a large language model (LLM) 3 times. The responses were graded by appropriate subspecialists as appropriate, inappropriate, or unreliable in 2 grading contexts. The first grading context was if the information was presented on a patient information site. The second was an LLM-generated draft response to patient queries sent by the electronic medical record (EMR). Appropriate was defined as accurate and specific enough to serve as a surrogate for physician-approved information. Main outcome measure was percentage of appropriate responses per subspecialty., Results: For patient information site-related questions, the LLM provided an overall average of 79% appropriate responses. Variable rates of average appropriateness were observed across ophthalmic subspecialties for patient information site information ranging from 56% to 100%: cataract or refractive (92%), cornea (56%), glaucoma (72%), neuro-ophthalmology (67%), oculoplastic or orbital surgery (80%), ocular oncology (100%), pediatrics (89%), vitreoretinal diseases (86%), and uveitis (65%). For draft responses to patient questions via EMR, the LLM provided an overall average of 74% appropriate responses and varied by subspecialty: cataract or refractive (85%), cornea (54%), glaucoma (77%), neuro-ophthalmology (63%), oculoplastic or orbital surgery (62%), ocular oncology (90%), pediatrics (94%), vitreoretinal diseases (88%), and uveitis (55%). Stratifying grades across health information categories (disease and condition, risk and prevention, surgery-related, and treatment and management) showed notable but insignificant variations, with disease and condition often rated highest (72% and 69%) for appropriateness and surgery-related (55% and 51%) lowest, in both contexts., Conclusion: This LLM reported mostly appropriate responses across multiple ophthalmology subspecialties in the context of both patient information sites and EMR-related responses to patient questions. Current LLM offerings require optimization and improvement before widespread clinical use., Competing Interests: POTENTIAL COMPETING INTERESTS The authors report no competing interests.

Background: Accurate diagnosis of efferent visual system disease in neuro-ophthalmology involves the classification of clinical signs as prenuclear, nuclear, or infranuclear in origin. Over many years, I have come to recognize ocular torsional instability as a clinical sign of prenuclear disease., Methods: Retrospective chart review of patients in whom ocular torsional instability was diagnosed using indirect ophthalmoscopy., Results: Twenty patients were diagnosed as having ocular torsional instability (OTI). Eight had neuro-ophthalmologic disease caused by structural injury to prenuclear ocular motor areas. Six of these had structural lesions primarily involving the cerebellum, one had a midbrain glioma, and one had traumatic encephalomalacia. Eight additional patients had infantile strabismus (esotropia in 7, exotropia in 1) associated with various combinations of monocular nasotemporal optokinetic asymmetry, latent nystagmus, and dissociated vertical divergence (DVD), indicating prenuclear involvement of subcortical visuovestibular pathways within the brain. Three additional patients presented with intermittent exotropia with DVD signifying early onset, while 2 had acquired esotropia that was noninfantile in origin. One had partially accommodative esotropia with bilateral inferior oblique overaction, and one presented with acquired esotropia followed by spontaneous secondary exotropia., Conclusions: OTI provides a useful clinical sign of prenuclear ocular motor dysfunction. When detected in patients without any signs of infantile or early-onset strabismus, OTI signifies the need for neuroimaging to rule out neurovestibular or cerebellar causes of prenuclear disease., Competing Interests: The author reports no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Most primary orbital pathology in children is due to bacterial infection. Radiologists typically encounter these cases to evaluate for clinically suspected postseptal orbital involvement. Contrast-enhanced cross-sectional imaging is important for the detection and early management of orbital infection and associated subperiosteal/orbital abscess, venous thrombosis, and intracranial spread of infection. Benign mass-like inflammatory processes involving the pediatric orbit are rare, have overlapping imaging features, and must be distinguished from orbital malignancies., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: To evaluate the therapeutic effect of temporal slant recession of the inferior rectus muscle (TSRIRM) for the treatment of small vertical deviations in patients with vertical diplopia, with consideration of the theoretical additive effects of this procedure on ocular torsion and horizontal incomitance., Methods: Retrospective review of 11 patients who were treated with TSRIRM. Eight patients with vertical diplopia and small hyperdeviations (up to 6 prism diopters (PDs)) were treated with isolated TSRIRMs. Three patients with vertical diplopia from unilateral superior oblique palsies with large hyperdeviations (>15 PDs) were treated with TSRIRMs in conjunction with contralateral inferior oblique recessions., Results: Six of the 8 patients with small vertical deviations had successful vertical realignment with elimination of symptomatic diplopia after an isolated TSRIRM. Two of the eight patients had residual hypertropia with symptomatic diplopia. Three additional patients with unilateral superior oblique palsy had successful vertical realignment with elimination of symptomatic diplopia after ipsilateral inferior oblique recession and contralateral TSRIRM. In total, 9 of 11 patients had successful surgical results after TSRIRM., Conclusions: TSRIRM provides an effective and reliable treatment for small angle vertical strabismus. Its ease of surgical access renders it useful for implementation in an outpatient neuro-ophthalmology setting., Competing Interests: The author reports no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

An 8-year-old boy presented with acute visual loss in the right eye and nausea, vomiting, and diplopia. Imaging revealed a right orbital apex mass. Biopsy showed Langerhans cell histiocytosis (LCH), and the patient was diagnosed with isolated orbital LCH causing an orbital apex syndrome. A 12-month cytarabine chemotherapy course was begun, during which the patient developed bilateral optic disc edema. He was diagnosed with cytarabine-induced intracranial hypertension, which was successfully treated with acetazolamide. The cytarabine course was completed with complete resolution of the LCH lesion. The ophthalmologic relevance of this rare disorder is discussed., Competing Interests: Conflicts of Interest None to disclose for any author., (Copyright © 2022 Elsevier Inc. All rights reserved.)

A 14-year-old boy with a history of shunted congenital hydrocephalus began having headaches with nausea and vomiting after transcontinental flights. He gradually developed horizontal diplopia indicative of mild bilateral sixth nerve palsy, without papilledema or ventriculomegaly. Intracranial pressure monitoring showed no signs of elevation. After he subsequently developed papilledema, surgical exploration showed shunt malfunction, and shunt replacement produced rapid resolution of symptoms. This case demonstrates the importance of relying on clinical history and neuro-ophthalmologic examination in patients with hydrocephalus and suspected shunt failure, even when objective confirmatory evidence of intracranial pressure elevation is lacking., Competing Interests: Declaration of Interest None, (Copyright © 2022. Published by Elsevier Inc.)

Background: Interprofessional electronic consultation (eConsult) is a telemedicine modality in which consulting providers review outside records and provide recommendations without in-person consultation. The purpose of this study was to describe the utilization of eConsults in the management of neuro-ophthalmic conditions., Methods: Retrospective cohort study of all patients who received an eConsult for a neuro-ophthalmic condition at a single quaternary referral center from 2018 to 2020. Main outcome measures included proportion of eConsults in which sufficient data were provided to the neuro-ophthalmologist to generate a definitive management decision, proportion of patients for whom an in-person neuro-ophthalmology evaluation was recommended, and the eConsult's impact on patient care., Results: Eighty eConsults were conducted on 78 patients during the 3-year study period. Forty-eight (60.0%) subjects were female, mean age was 54 years, and 65 (81.3%) were White. The median time from eConsult request to completion was 4 days (range: 0-34 days). The most frequent eConsult questions were vision/visual field disturbances in 28 (35.0%) cases, optic neuropathies in 22 (27.5%), and optic disc edema in 17 (21.3%). At the time of eConsult, sufficient prior information was provided in 35 (43.8%) cases for the neuro-ophthalmologist to provide a definitive management decision. In 45 (56.3%) eConsults, further diagnostic testing was recommended. In-person neuro-ophthalmology consultation was recommended in 24 (30.0%) cases. Sixty-one (76.3%) eConsults provided diagnostic and/or treatment direction, and 12 (15.0%) provided reassurance., Conclusion: eConsults increase access to timely neuro-ophthalmic care and provide diagnostic and treatment direction to non-neuro-ophthalmology providers when sufficient information is provided at the time of eConsult., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Clinical examination of ocular torsion has become an integral part of strabismus diagnosis and management. However, the clinical manifestations of subjective and objective torsion are frequently conflated or disregarded, creating diagnostic confusion and therapeutic dilemmas. The purpose of this analysis is to outline important clinical principles to understand and manage ocular torsion. Ten clinical axioms are embedded within a neurophysiologic framework for torsional control to provide a critical neural mechanism to fortify binocular vision and balance.

Background: To determine whether the vestibular-ocular reflexes (VORs) can be affected by central nervous system injury in children with cortical visual impairment (CVI)., Methods: Retrospective case series. Twenty consecutive children with CVI who presented to a pediatric ophthalmology practice over an 18-month period were included in the study. Horizontal and vertical VORs were assessed by a pediatric neuro-ophthalmologist using the standard doll's head maneuver. MRI studies were independently reviewed by a pediatric neuroradiologist in a masked fashion. The main outcome measures were the integrity of the VORs and the presence of brainstem abnormalities on MRI., Results: VORs were found to be absent or severely impaired in 13/20 (65%) children with CVI. More surprisingly, the doll's head maneuver failed to substantially overcome the deviated eye position in 8/13 (62%) children with conjugate gaze deviations. Reduced brainstem size and signal abnormalities were found in 4/7 children with normal VORs and in 9/13 children with abnormal VORs (P = 0.6), showing noncorrelation with the integrity of the VOR., Conclusion: VORs are commonly impaired in children with CVI. This ocular motor deficit reflects the diffuse cortical and subcortical injury that often accompanies perinatal injury to the developing brain. Consequently, these children may lack important visual compensatory mechanisms to stabilize gaze during head movements. This knowledge can help in planning visual rehabilitation., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Purpose : To evaluate the efficacy of augmented bilateral lateral rectus recession in the treatment of consecutive exotropia. Methods : We retrospectively reviewed records of nine patients who underwent augmented bilateral lateral rectus recession for consecutive exotropia with minimal adduction deficits. Our normal surgical dosing tables for bilateral lateral rectus recession were augmented by adding 1.5-2 mm. All patients had been measured preoperatively using prism and alternate cover testing (PACT), except for one patient in whom Krimsky measurements were performed because of amblyopia. Surgery was deemed successful if postoperative alignment fell within a potential monofixation range of ±10 prism diopters (PD) by PACT at the final postoperative examination. Results : Eight out of nine patients (89%) had a successful outcome. One patient was surgically undercorrected. Despite successful realignment to a state of monofixation syndrome in most patients, there was no significant restoration of stereopsis following strabismus surgery. Conclusions : Surgical augmentation of bilateral lateral rectus recession standard dosing by 1.5-2 mm is efficacious for restoring binocular alignment for both distance and near fixation. This surgical approach may be preferable to bimedial advancements in patients with minimal preoperative adduction deficits who show no significant increase in exotropia during near fixation.

This vignette describes a diagnostic telephone consultation with Bill Hoyt regarding a 65-year-old man who was found to have an unusual vascular pattern overlying the left optic disc. Bill recognized and characterized this condition as prepapillary arterial convolutions, a congenital vascular anomaly that has never entered the scientific literature. Close inspection disclosed evidence of mild vascular derangement in the contralateral eye as well. The cause of this condition remains unknown.

Over the past 50 years, our understanding of optic nerve hypoplasia has advanced in a series of waves, with each wave producing a paradigm shift in clinical diagnosis and management. First was the recognition that optic nerve hypoplasia is a condition distinct from optic atrophy and is a frequent cause of blindness in children. Second was the identification of associated brain malformations. Third was the realization that many children with optic nerve hypoplasia have hypopituitarism. Fourth was the identification of segmental forms of optic nerve hypoplasia. Fifth was the recognition that some children are at risk for sudden death, which can often be prevented with preemptive measures. Last was the identification of additional systemic accompaniments. Genetic studies have been largely unfruitful and, for most cases, the cause remains unknown.

In recent years, we have made enormous strides in elucidating the phenomenology of congenital nystagmus. The purpose of this review is to briefly summarize our current understanding of congenital nystagmus in terms of its clinical symptomatology, pathophysiology, differential diagnosis, and ancillary testing, and clinical management. Finally, this discussion provides the reader with an armamentarium of clinical pearls to facilitate diagnosis of the numerous sensory visual disorders that can underlie congenital nystagmus.

Purpose: To document the association of prenatal brain disruption with secondary perinatal distress in children diagnosed as having cortical visual impairment (CVI)., Design: Retrospective case series., Methods: Eight children with severe CVI and clinical history of perinatal events were included. Case histories and neuroimaging studies were reviewed. The main outcome measures were perinatal history, visual and neurologic findings, and magnetic resonance (MR) imaging., Results: In our patient cohort, MR imaging showed signs of cortical dysgenesis leading to congenital brain malformations such as polymicrogyria consistent with a prenatal timing of CNS injury. Although subcortical white matter changes were common, signs of watershed injury to the visual cortex were absent, suggesting that the visual loss was attributable to a prenatal etiology with secondary birth complications., Conclusion: Some children with CVI and a history of perinatal distress have prenatal dysgenesis of the developing brain. Therefore, a clinical history of perinatal hypoxia-ischemia is nonspecific and merits neuroimaging to identify antecedent brain malformations and timing of injury, which can influence patient diagnosis and management., (Copyright © 2019 Elsevier Inc. All rights reserved.)

We report the case of a 9-year-old girl with chiasmal glioma and longstanding monocular nystagmus, in whom video-oculography showed a disconjugate binocular nystagmus indistinguishable from spasmus nutans. The "acquired monocular nystagmus" associated with chiasmal glioma is actually a binocular nystagmus that is indistinguishable from spasmus nutans, except that it lacks the associated head nodding and torticollis. Superimposed visual loss in one eye predisposes to acquired monocular nystagmus and explains the absence of the other components of the spasmus nutans triad., (Copyright © 2019 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

A 4-year-old girl with maxillary hypoplasia, intermittent exotropia, and high myopia displayed congenital oculonasal synkinesis. We examine the implications for pathogenesis of these disparate craniofacial findings.

Essential infantile esotropia (EIE) is often attributed to a primary disturbance within the visual cortex based upon the findings of monocular horizontal optokinetic asymmetry and correlative horizontal motion detection asymmetry. However, these physiologic aberrations conform to what would be observed if the visual cortex secondarily reconfigured itself to the preexisting subcortical optokinetic motion template. This analysis examines the perspective that the measured cortical aberrations can be explained by prolonged subcortical neuroplasticity, leading to a secondary rewiring of cortical motion pathways. Evolutionary evidence indicates that EIE is generated by subcortical ocular motor centers that subserve nasalward optokinesis. These phylogenetically older subcortical visuo-vestibular pathways include the nucleus of the optic tract, accessory optic system, inferior olive, cerebellar flocculus, and vestibular nucleus. In normal humans, the subcortical visual system becomes inactivated after the first few months of infancy. Mutations or other perturbations that prolong subcortical neuroplasticity may create a persistent simultaneous nasalward optokinetic bias in both eyes to generate infantile esotropia., (© 2019 Elsevier B.V. All rights reserved.)

Purpose: To evaluate the effects of isolated inferior oblique (IO) muscle recession in patients with superior oblique palsy (SOP) and persistent hypertropia in abduction., Methods: Retrospective review of patients with unilateral SOP who were treated with isolated IO recession by a single surgeon (MCB) between January 2008 and December 2017. We included patients with congenital and acquired fourth nerve palsies, with a hyperdeviation of less than 20 prism diopters in primary position and at least 4 prism diopters in abduction by prism and alternate cover test (PACT) during distance fixation. A minimum follow-up of 4-6 weeks was required. Age at surgery, etiology, presence of head tilt, motor alignment in primary and secondary gaze positions at distance and near using PACT, versions, ductions, and torsion were recorded from the patients' chart., Results: Seven patients with SOP were included in this study. Four (57.14%) males, with a mean age at presentation of 41.86 years (range: 6-66 years). Mean follow-up was 13.25 months (range: 1.3-52.2 months). A decrease in mean central gaze hypertropia from 11.4 to 1.71 PD was found. A mean contralateral gaze hypertropia that decreased from 22.28 to 5.71 PD and an ipsilateral gaze hypertropia that improved from 5.86 to 1.14 PD were also noted. Torsion had a mean change of 3.4° of incyclodeviation at the final examination., Conclusions: This study confirms the efficacy of isolated maximal IO recession for the treatment of unilateral SOP that is accompanied by a modest hypertropia of the paretic eye in abduction.