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Skew deviation is likely encountered clinically more often than previously thought. Cerebellar causes of skew deviation appear to be the rarest cause and should be considered by clinicians when encountering patients with findings consistent with skew deviation.This case report describes an undiagnosed, asymptomatic skew deviation in an established glaucoma patient returning for routine follow-up. Appropriate diagnosis and subsequent neuroimaging identified a chronic ischemic cerebellar infarction.A 72-year-old man presented for a routine glaucoma follow-up examination. Cover test revealed a 10-prism diopter left hypertropia, which worsened in right gaze and left head tilt. Double Maddox rod testing showed 5° excyclotorsion of the right eye and no torsion in the left eye. Upright-supine testing was positive. Given the patient's noticeable right head tilt, left hypertropia, increased excyclotorsion of his lower eye, and positive-upright test, the patient was diagnosed with a skew deviation. Brain MRI revealed a chronic, undiagnosed cerebellar ischemic infarction, a known cause of skew deviation and the associated ocular tilt reaction.It can be challenging to identify skew deviations and their underlying causes. When clinically encountering a vertical deviation, appropriate tests (cover-uncover test, Parks-Bielschowsky three-step test, ocular torsion testing, and upright-supine test) are imperative to help make the correct diagnosis. Neuroimaging can then be pursued in hopes of identifying the underlying cause.

Neuromyelitis optica spectrum disease (NMOSD) and multiple sclerosis (MS) share clinical presentations including optic neuritis and brainstem syndromes. Internuclear ophthalmoplegia (INO) is characterized by slowed ipsilateral adduction saccades and results from a lesion in the medial longitudinal fasciculus (MLF). Although INO is a common clinical finding in MS, its prevalence in NMOSD is unknown. The objective of this work is to determine the comparative frequencies of INO in patients with NMOSD and MS and compare clinical features of both disease processes.This is a retrospective study of patients 18 years and older who have an established diagnosis of NMOSD or MS and were evaluated by both neuro-ophthalmology and neuro-immunology specialists between 2014 and 2020. Electronic medical records were screened for documentation of an acute INO at any time during follow-up. Incidence rates were calculated from number of cases of new-onset INO and patient years observed. Logistic regression was used to evaluate the likelihood of developing an INO at any time point for NMOSD vs MS patients. Multivariable analysis was performed by adjusting for age, race, gender, and length of follow-up.Two hundred eighty patients (80 NMOSD, 200 MS) were included. Age range was 18-79 years with a mean age of 35.14 (SD ± 12.41 years). Average length of follow-up in MS and NMOSD patients was 4.18 years vs 3.79 years, respectively (P0.05), and disease duration before the start of the study in MS and NMOSD was 8.76 years vs 4.65 years, respectively (P0.01). Mean disease duration and follow-up time of both groups was 7.58 years and 4.07 ± 2.51 years, respectively. NMOSD patients were predominantly seropositive for AQP4 antibody (61.25%, n = 49). Individuals who had MOG antibody but also met NMOSD criteria were also included (18.75%, n = 15). The frequency of INO at any time point was 1.25% (n = 1) in NMOSD compared with 16% (n = 32) in MS. The incidence rate of new-onset INO in NMOSD (excluding MOGAD) was 3.8/1,000 person years and 23.9/1,000 person years in MS. Adjusted analysis showed that NMOSD patients were 13.89 times (odds ratio [OR] 0.07, 95% confidence interval [CI] 0.01-0.598, P = 0.015) less likely to develop an INO compared with those with MS when including MOGAD patients, 12.5 times less likely (OR 0.08, 95% CI: 0.10-0.67, P = 0.02) when excluding MOGAD patients and 9.62 times less likely (OR 0.10, 95% CI: 0.01-0.87, P = 0.036) for AQP4+ patients.Our study shows that the incidence of new INO (3.8 vs 23.9 per 1,000 person years), and the odds of having INO at any time point are significantly lower in NMOSD than MS. This suggests that INO and consequently MLF lesions are less common in NMOSD. The presence of an INO may help in the differentiation of NMOSD from MS and may aid in earlier implementation of disease appropriate therapy.

This study aimed to analyze the Hess area ratio (HAR%) in cases of blowout fracture treated in our department and clarify the outline of eye movement disorders in blowout fractures. Patients who underwent surgery for orbital blowout fractures in our department were included. Fracture locations were classified into 5 types (A, outside floor; B, C, anterior and posterior floor; and D, E, anterior and posterior medial wall). The HAR% was compared before and after surgery in eligible cases. The relationship between the fracture location and preoperative HAR% was investigated using multiple regression analysis. The study involved 85 patients. Hess area ratio was higher postoperatively than preoperatively (70.75 ± 18.26 versus 90.06 ± 13.99, P 0.01). The postoperative HAR% tended to be higher when the iliac bones were compared to other materials; however, this difference was not significant (90.73 ± 12.91 versus 80.30 ± 17.81, P = 0.178). Fracture locations C and E significantly contributed to the prediction of HAR% as negative regression coefficients (P = 0.024 and 0.013, respectively). The posterior fracture area on both the orbital floor and medial wall contributed to the decrease in preoperative HAR%. This observation indicates that the reconstruction of the posterior region is extremely crucial.

A 74-year-old man presented with falls, dysphagia, and personality change. Examination showed axial parkinsonism (without response to levodopa), low pitched dysarthria, supranuclear vertical gaze palsy, decreased blinking, square-wave jerks, primitive reflexes, apathy, and decreased verbal fluency. Brain MRI showed dorsal midbrain and frontal …

A 69-year-old man with a history of pontine hemorrhage 2 years ago noticed binocular vertical diplopia after the stroke. On examination, there was a small-angle incomitant left hyperdeviation that did not fit the 3-step test for fourth nerve palsy and incyclotorsion of the higher eye. On motility testing, there was an obvious pendular nystagmus. Resting tremor of the right hand was noticed on neurological examination. Examination of the oropharynx revealed rhythmic oscillations of the soft palate synchronous with the eye oscillations and hand tremor. These findings established a diagnosis of oculopalatal myoclonus (OPM). Although OPM is a well-described entity, this case is unique because the patient was completely asymptomatic from OPM and did not complain of oscillopsia but was very bothered by vertical diplopia because of skew deviation. It also demonstrates that OPM may coexist with skew deviation because anatomically vestibulo-ocular pathway is close to the triangle of Guillain-Mollaret and patients with lesions in one pathway should be examined for abnormalities in the other. Finally, it reminds us about the importance of monitoring patients with a history of brainstem insults for emergence of synchronous tremors years later and that simple maneuver-like ex/amining oropharynx may provide a clear diagnosis.

Introduction Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare variation of internuclear ophthalmoplegia associated with bilateral lesions of the medial longitudinal fasciculus. Case report In this case, the edematous lesions of the ipsilateral midbrain infarction appeared to compress the contralateral medial longitudinal fasciculus, resulting in WEBINO. Treatment of focal cerebral edema caused by ischemic stroke is still under debate. However, in this case, antiedema treatment was implemented to prevent the sequelae of the edematous lesion caused by a midbrain infarction, and a relatively rapid improvement of ocular symptoms was observed. Conclusion Herein, we report a rare case of WEBINO caused by a unilateral midbrain infarction in which the patient experienced edema-related neurological deficits. This case provides evidence that early antiedema therapy may benefit patients with similar presentations.

Significance Ocular tilt reaction (OTR) is an abnormal eye-head postural reaction that consists of skew deviation, head tilt, and bilateral ocular torsion. Understanding of the pathway of the vestibulo-ocular reflex (VOR) is essential because this will help to localize the pathology. Purpose The aim of this study was to report a case of OTR with contralateral internuclear ophthalmoplegia (INO) and fifth and seventh cranial nerve palsies. Case report A 51-year-old gentleman with underlying diabetes mellitus presented with sudden onset of diplopia for 3 days. On examination, his visual acuity was 20/30 bilaterally without a relative afferent pupillary defect. He had a right OTR consisting of a right head tilt, a skew deviation with a left eye hypertropia, and bilateral ocular torsion (right excyclotorsion and left incyclotorsion) with nystagmus. He also had a left adduction deficit and right abduction nystagmus consistent with a left INO. Ocular examination revealed evidence of proliferative diabetic retinopathy bilaterally. Two days after the initial presentation, the patient developed left seventh and fifth cranial nerve palsies. MRI showed left pontine infarction and multiple chronic lacunar infarctions. There was an incidental finding of a vascular loop compression on cisternal portions of the left trigeminal, facial, and vestibulocochlear nerves. Antiplatelet treatment was started on top of a better diabetic control. The diplopia was gradually resolved with improved clinical signs. In this case, the left pontine infarction had likely affected the terminal decussated part of the vestibulocochlear nerve from the right VOR pathway, medial longitudinal fasciculus, and cranial nerve nuclei in the left pons. Conclusions The OTR can be ipsilateral to the lesion if the lesion is before the decussation of the VOR pathway in the pons, or it can be contralateral to the lesion if the lesion is after the decussation. In case of an OTR that is associated with contralateral INO and other contralateral cranial nerves palsy, a pathology in the pons that is contralateral to the OTR should be considered. Neuroimaging study can hence be targeted to identify the possible cause.

Neurogenic blepharoptosis after orbital wall reconstruction is a rare complication. It can either present as an isolated blepharoptosis, or accompany with other ocular symptoms. The authors introduce 4 cases of post-operative neurogenic blepharoptosis after reconstruction of medial orbital wall via transcaruncular approach.Immediately after the surgery, 2 adolescent patients experienced unilateral islolated complete blepharoptosis with no other ocular symptoms. The other 2 adult patients presented unilateral complete blepharoptosis accompanied by lateral displacement of the globe and evident extraocular movement limitations. The 2 adolescent patients were observed with no particular treatment, whereas the other 2 adult patients were treated with intravenous systemic steroids. The blepharoptosis symptom began to improve at 2 weeks post-operatively and completely recovered within 3 to 4 months in all patients, while the globe displacement and extraocular movement limitation started to improve at 4 weeks after the surgery and fully recovered within 6 months.Although neurogenic blepharoptosis after medial orbital wall reconstruction is a very rare complication, it can be critical once it occurs. According to the author's experience, it fortunately shows a favorable clinical course of spontaneous recovery. Post-operative neurogenic blepharoptosis usually recovers between 2 weeks and 4 months and other ocular symptoms related to oculomotor nerve injury recovers within 6 months after the surgery.

The contributions of William F. Hoyt, MD, to the field of neuro-ophthalmology are immense. His precision in history-taking and examination skills provided the foundation to fully understand a clinical disorder-determining its underlying pathophysiology, prognosis, and management. We describe two unusual eye movement disorders, superior oblique myokymia and ocular neuromyotonia, and how Dr. Hoyt's contributions shaped our understanding of these clinical entities.

Background Dorsal midbrain syndrome (DMS) consists of a constellation of clinical features, including reduced upgaze, pupillary light-near dissociation, lid retraction, convergence retraction, and eye misalignment. This syndrome results mostly from intrinsic or extrinsic mesodiencephalic tumors or strokes, obstructive hydrocephalus, failure of cerebrospinal fluid shunting to correct obstructive hydrocephalus, and head trauma. Published reports that include imaging corroboration are based on relatively small cohorts and have not included comprehensive patient self-reports on the impact of these abnormalities on quality of life. Methods We conducted a retrospective review of cases of DMS identified between 1998 and 2019 at the University of Michigan using the Electronic Medical Record Search Engine. Patients were included only if they had been evaluated by a neuro-ophthalmologist and had a corroborative imaging abnormality. We collected data on symptoms and on neuro-ophthalmic and neurologic signs. We reviewed brain imaging reports on all 75 patients, and the study neuroradiologist analyzed the imaging in 57 patients. Using a uniform list of questions, we conducted telephone interviews of 26 patients to assess lingering symptoms and their impact on quality of life. Results There were 75 patients, only 5 of whom were younger than 10 years. Neoplasms accounted for 47%, strokes (mostly thalamic) for 25%, nonneoplastic masses for 12%, nonneoplastic hydrocephalus for 7%, traumatic brain injury for 5%, and demyelination for 4%. Reduced upgaze occurred in 93% of patients, being completely absent or reduced to less than 50% amplitude in 67%. Convergence retraction on attempted upgaze occurred in 52%, horizontal misalignment in 49%, vertical misalignment in 47%, and pupillary light-near dissociation in 37%. Optic neuropathy attributed to chronic papilledema occurred in only 3%. Three or more neuro-ophthalmic signs were present in 84%, and only 4% had a single sign-reduced upgaze. Imaging features did not correlate with the frequency or severity of clinical signs. There was some improvement in the clinical signs among the patients with stroke but no change among the patients with neoplasms. In the 26 telephone interviews, patients with neoplasms reported that imbalance had a greater impact on quality of life than did diplopia. Patients with strokes reported that imbalance had the greatest impact initially but that its effect dissipated. Neither group reported lingering effects of impaired upgaze. Conclusions This large series expands on the clinical profile of DMS. Neoplasms and strokes were the most common causes. Obstructive hydrocephalus alone, identified as a major cause in the largest previously published series, was uncommon. At least 3 neuro-ophthalmic signs were present in nearly all patients, with upgaze deficit as predominant. Unlike an earlier report, this study found no correlation between brain imaging and clinical signs. Neuro-ophthalmic signs persisted even after neoplasms were successfully treated and improved only slightly after stroke. Telephone interviews with patients revealed that diplopia and upgaze deficit had less lasting impact on quality of life than did ataxia and concurrent nonneurologic problems.

ObjectiveTo characterize subclinical abnormalities in asymptomatic heterozygote NPC1 mutation carriers as markers of neurodegeneration.MethodsMotor function, cognition, mood, sleep, and smell function were assessed in 20 first-degree heterozygous relatives of patients with Niemann-Pick disease type C (NPC) (13 male, age 52.7 ± 9.9 years). Video-oculography and abdominal ultrasound with volumetry were performed to assess oculomotor function and size of liver and spleen. NPC biomarkers in blood were analyzed. 18F-fluorodesoxyglucose PET was performed (n = 16) to detect patterns of brain hypometabolism.ResultsNPC heterozygotes recapitulated characteristic features of symptomatic NPC disease and demonstrated the oculomotor abnormalities typical of NPC. Hepatosplenomegaly (71%) and increased cholestantriol (33%) and plasma chitotriosidase (17%) levels were present. The patients also showed signs seen in other neurodegenerative diseases, including hyposmia (20%) or pathologic screening for REM sleep behavior disorder (24%). Cognitive function was frequently impaired, especially affecting visuoconstructive function, verbal fluency, and executive function. PET imaging revealed significantly decreased glucose metabolic rates in 50% of participants, affecting cerebellar, anterior cingulate, parieto-occipital, and temporal regions, including 1 with bilateral abnormalities.ConclusionNPC heterozygosity, which has a carrier frequency of 1:200 in the general population, is associated with abnormal brain metabolism and functional consequences. Clinically silent heterozygous gene variations in NPC1 may be a risk factor for late-onset neurodegeneration, similar to the concept of heterozygous GBA mutations underlying Parkinson disease.

Heterozygous pathogenic variants in CACNA1A are associated with 3 classic phenotypes, with variable expression and significant overlap: familial hemiplegic migraine 1, spinocerebellar ataxia 6, and episodic ataxia type 2.

Pineal masses commonly present with Parinaud syndrome, a triad consisting of upgaze restriction, convergence-retraction nystagmus, and pupillary light-near dissociation from compression of the quadrigeminal plate.

Pathogenic variations in the SLC9A6 gene are associated with an X-linked disorder Christianson syndrome characterized by developmental delay, microcephaly, intellectual disability, autistic-like behavior and epilepsy. We identified a novel pathogenic variation in the SLC9A6 gene in a boy with developmental delay and microcephaly. Herein we report the clinical findings of the case diagnosed as Christianson syndrome; his mother was found to carry the same variant.

Purpose of review Congenital fibrosis of the extraocular muscles (CFEOM) is caused by abnormal development of the innervation of extraocular muscles. We update the recent literature regarding the clinical, anatomic, genetic, and molecular characteristics of CFEOM. Surgical considerations are addressed. Recent findings CFEOM is broken down into three main subtypes, CFEOM1, CFEOM2, and CFEOM3. Several recent reports of individuals, as well as family pedigrees, highlight the phenotypic heterogeneity of CFEOM. Intracranial and intraorbital radiologic findings have enhanced our understanding of the disease pathophysiology. Molecular genetics research has increased our understanding of the development of extraocular muscles and their innervation as well as pathophysiology of CFEOM. Summary Our understanding of the pathophysiology of CFEOM has increased with the recent contributions from neuroimaging, molecular genetics, and pedigree analysis. Surgical management of patients with CFEOM continues to be challenging.

Inferior rectus avulsion following blunt trauma is rare, with even fewer reported cases of complete transection. The authors report a case of orbital floor fracture and inferior rectus muscle transection without herniation following blunt orbital trauma. This case first highlights the difficulty in diagnosing complete inferior rectus muscle transection clinically and with imaging and second that an acceptable functional outcome can be achieved by a novel surgical repair approach utilizing contiguous orbital anatomical relations of the inferior oblique and inferior rectus muscles.

Our study revealed that children with sensory processing disorder (SPD) exhibit deficient saccades and pursuits compared with typically developing (TD) children. Optometrists have a key role in caring for patients with SPD. Optometrists are paramount in identifying, coordinating care for, and treating these children.Children with SPD exhibit poor neurological processes, which can affect oculomotor performance. The study aims are to investigate differences in oculomotor skills using the Northeastern State University College of Optometry (NSUCO) oculomotor testing in children with SPD as compared with TD children, to explore effects of age and sex of children in each group on oculomotor performance, and to compare our results to test normative values for both TD and SPD groups.One hundred fifty children aged 7 to 11 years were recruited from surrounding community. The Short Sensory Profile (SSP) was used to assign the children into two groups. Of the eligible children recruited, 60 were TD (SSP ≥155) and 68 had SPD (SSP ≤141). Children were excluded if they reported blindness, strabismus, deafness, ocular disease, fractures within 6 months, or physical disabilities. Gait, balance, and visual skills of all children were assessed. Visual skill evaluation included select visual efficiency and visual processing testing. The NSUCO test was included as part of the visual efficiency evaluation.Results showed that children with SPD demonstrate decreased oculomotor skills on all tests compared with TD children. For the SPD group, boys scored significantly poorer than did girls in head and body movement with saccades and pursuits. Overall, TD group scores confirm the established test norms.Because of significant differences in oculomotor function in children with SPD and the increasing number of children with neurosensory disorders, optometrists should consider NSUCO testing on all pediatric patients and particularly in children with SPD.

Significance This first report of the use of objective measures of disparity vergence as outcome measures for symptomatic convergence insufficiency in children provides additional information that is not accessible with clinical tests. The study results also demonstrate that objective measures of vergence could be used in future randomized clinical trials of binocular vision disorders with children. Purpose This study was designed to evaluate changes in objective measures of disparity vergence after office-based vergence/accommodative therapy (OBVAT) for convergence insufficiency in children 12 to 17 years old. Methods In this prospective trial, we recruited 10 participants with normal binocular vision and 12 with convergence insufficiency. All participants with convergence insufficiency were treated with 12 weeks of OBVAT. The primary outcome measure was average peak velocity for 4° symmetrical convergence steps. Other objective outcome measures of disparity vergence included time to peak velocity, latency, and accuracy. Changes in clinical measures (near point of convergence, positive fusional vergence at near) and symptoms were evaluated. Results There was a statistically significant increase in peak velocity and more accurate response amplitude to 4° symmetrical convergence step stimuli after OBVAT compared with baseline measurements. Near point of convergence, positive fusional vergence, and symptoms also statistically significantly improved after OBVAT. Ten of the 12 participants met clinical success criteria. Conclusions In this prospective study on the treatment of symptomatic convergence insufficiency in children in which both clinical and objective eye movement measurements were used to evaluate the results of treatment, significant changes were found in symptoms and both clinical and objective measures of disparity vergence after completion of OBVAT in children with symptomatic convergence insufficiency.

OBJECTIVES Oculogyric crisis is a dystonic movement disorder characterized by continuous contraction of the ocular muscles and spasmodic movements of the pupils in a fixed position, usually upward. METHODS In this case report, an early-stage acute oculogyric crisis due to low-dose olanzapine in a 15-year-old adolescent boy is presented. RESULTS To the best of our knowledge, this is the first report showing that acute oculogyric crisis can develop with low-dose olanzapine administration. CONCLUSIONS Even though second-generation antipsychotics are safer than conventional antipsychotics in terms of extrapyramidal adverse effects, this possibility should always be taken into consideration through this case report.

Background and ObjectivesOpsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare disorder of the nervous system that classically presents with a combination of characteristic eye movement disorder and myoclonus, in addition to ataxia, irritability, and sleep disturbance. There is good evidence that OMAS is an immune-mediated condition that may be paraneoplastic in the context of neuroblastoma. This syndrome may be associated with long-term cognitive impairment, yet it remains unclear how this is influenced by disease course and treatment. Treatment is largely predicated on immune suppression, but there is limited evidence to indicate an optimal regimen.MethodsFollowing an international multiprofessional workshop in 2004, a body of clinicians and scientists comprising the International OMS Study group continued to meet biennially in a joint professionals and family workshop focusing on pediatric OMAS. Seventeen years after publication of the first report, a writing group was convened to provide a clinical update on the definitions and clinical presentation of OMAS, biomarkers and the role of investigations in a child presenting with OMAS, treatment and management strategies including identification and support of long-term sequelae.ResultsThe clinical criteria for diagnosis were reviewed, with a proposed approach to laboratory and radiologic investigation of a child presenting with possible OMAS. The evidence for an upfront vs escalating treatment regimen was reviewed, and a treatment algorithm proposed to recognize both these approaches. Importantly, recommendations on monitoring of immunotherapy response and longer-term follow-up based on an expert consensus are provided.DiscussionOMAS is a rare neurologic condition that can be associated with poor cognitive outcomes. This report proposes an approach to investigation and treatment of children presenting with OMAS, based on expert international opinion recognizing the limited data available.

As the majority of our patients are spending significant time using computers and reading, it is important to understand any disease process that can affect one's near vision. Convergence insufficiency, not an uncommon condition, is still not screened for by most eyecare professionals. This review aims to report the current screening methods and diagnostic criteria, and to summarize the current treatment of convergence insufficiency.The current literature shows that convergence insufficiency has a prevalence of 2-17% in the general population and an even higher rate, up to 49%, in patients who have suffered a traumatic brain injury. Although the measurement is still not standardized, near point of convergence and patient symptomatology appear to be an appropriate screen for convergence insufficiency. Further study is needed to establish standardization of diagnostic criteria. It is now well recognized that orthoptic/vergence therapy provides excellent improvement in the clinical measurements and symptoms associated with convergence insufficiency.Convergence insufficiency is a condition that causes a significant impact on near vision. Treatment with orthoptic/vergence therapy can reduce symptomatology and greatly improve one's quality of life. Further study is needed to provide an evidence-based definition that encompasses all cases of convergence insufficiency, research possible subtypes of the disease and establish the efficacy of home-based computer therapy as compared to office-based orthoptic/vergence therapy.

Antibodies targeting the 65-kilodalton isoform of glutamic acid decarboxylase (GAD65) are implicated in several autoimmune neurologic disorders.1 Anti-GAD65 antibodies (GAD65-Abs) can be seen in healthy individuals, highlighting the importance of interpreting their significance in the appropriate clinical context.1 Classic neurologic presentations include stiff-person syndrome, cerebellar ataxia, limbic encephalitis, epilepsy, myelopathy, other brainstem manifestations, and extrapyramidal symptoms.1–3 A few cases of focal myoclonus have been described.2 There is one previous report of palatal myoclonus with normal brain MRI.4 We report a patient with GAD65-Abs with relapsing diplopia, cerebellar syndrome, and palatal myoclonus with hypertrophy of the inferior olivary nuclei (ION) seen on 1.5T MRI and a dentato-rubro-olivary pathway lesion on 7T MRI.

Significance Prompt neuroimaging is important to identify multiple sclerosis lesions in the appropriate clinical setting. However, despite a normal brain MRI finding, a diagnosis of multiple sclerosis still must be considered in cases of dorsal midbrain syndrome, even if it is transient. Purpose The purpose of this case report is to present a patient with a transient presentation of dorsal midbrain syndrome, resolving within 1 week of initial symptoms, which was ultimately attributed to multiple sclerosis in the setting of a normal enhanced brain MRI study. Case report A 33-year-old man with new-onset visual complaints was found to have upgaze paresis, eyelid retraction, and pupillary light-near dissociation suggestive of dorsal midbrain syndrome. Within days, enhanced brain MRI was completed and showed a normal finding, and the clinical features of dorsal midbrain syndrome had resolved. Subsequent spine imaging and lumbar puncture lead to an ultimate diagnosis of multiple sclerosis. Conclusions There have been a few reported cases of dorsal midbrain syndrome as the presenting feature of multiple sclerosis. This case is unique because it reports a transient presentation of dorsal midbrain syndrome, documented to have resolved only days after initial presentation, which was ultimately attributed to multiple sclerosis.

Objective:To determine the role of the medial longitudinal fasciculus (MLF) in conveying vestibular signals.Methods:In 10 patients with isolated acute unilateral internuclear ophthalmoplegia (INO) due to an acute stroke, we performed comprehensive vestibular evaluation using video-oculography, head impulse tests with a magnetic search coil technique, bithermal caloric tests, tests for the ocular tilt reaction, and measurements of subjective visual vertical and cervical and ocular vestibular evoked myogenic potentials (VEMPs).Results:The head impulse gain of the vestibulo-ocular reflex (VOR) was decreased invariably for the contralesional posterior canal (PC) (n = 9; 90%) and usually for the ipsilesional horizontal canal (n = 5; 50%). At least one component of contraversive ocular tilt reaction (n = 9) or contraversive tilt of the subjective visual vertical (n = 7) were common along with ipsitorsional nystagmus (n = 5). Cervical or ocular VEMPs were abnormal in 5 patients.Conclusions:The MLF serves as the main passage for the high-acceleration VOR from the contralateral PC. The associations and dissociations of the vestibular dysfunction in our patients indicate variable combinations of damage to the vestibular fibers ascending or descending in the MLF even in strokes causing isolated unilateral INO.

Ocular functions can be affected in almost any type of cerebrovascular accident (CVA) creating a burden on the patient and family and limiting functionality. The present review summarizes the different ocular outcomes after stroke, divided into three categories: vision, ocular motility, and visual perception. We also discuss interventions that have been proposed to help restore vision and perception after CVA.Interventions that might help expand or compensate for visual field loss and visuospatial neglect include explorative saccade training, prisms, visual restoration therapy (VRT), and transcranial direct current stimulation (tDCS). VRT makes use of neuroplasticity, which has shown efficacy in animal models but remains controversial in human studies.CVAs can lead to decreased visual acuity, visual field loss, ocular motility abnormalities, and visuospatial perception deficits. Although ocular motility problems can be corrected with surgery, vision, and perception deficits are more difficult to overcome. Interventions to restore or compensate for visual field deficits are controversial despite theoretical underpinnings, animal model evidence, and case reports of their efficacies.

OBJECTIVE The purpose of this study was to evaluate the pupil response to chromatic stimuli in patients with lesions in the dorsal midbrain and possibly gain new insights into the afferent pupillary pathways. METHODS Color pupillography was performed in 5 patients with dorsal midbrain syndrome (DMS), and their results were compared with those of 20 healthy control subjects. We used full-field red stimuli (605 nm) that primarily address the rod/cone system and blue stimuli (420 nm) that preferentially activate intrinsically photosensitive retinal ganglion cells (ipRGCs) directly, with a duration of 4 seconds and a stimulus intensity of 28 lx corneal illumination under mesopic conditions. One eye was stimulated, and the consensual pupil response was recorded and analyzed. RESULTS The pupillary light reflex in patients with DMS was reduced, differed in shape, and showed a prolonged latency time compared to normal subjects. The blue response was less affected than the red response: the mean maximal relative amplitude (M) was 15.8% (SD = 7.8) in patients with DMS compared with 43.0% (SD = 5.5) in normal subjects for red stimulation, and M = 40.8%, SD = 8.4 (DMS) with M = 58.3%, SD = 4.8 (normals) for blue stimulation. The reduction was 63% for red stimulation but only 30% for blue stimulation in patients with DMS. Moreover, there was a preserved postillumination pupil response to blue stimulation in DMS patients. CONCLUSIONS In DMS, the melanopsin-mediated ipRGC pathway appeared relatively preserved.

A 61-year-old man with a history of alcoholic cirrhosis was transferred from an outside hospital for spontaneous bacterial peritonitis, septic shock, and respiratory failure after intubation. The patient was initially on sedation; however, more than 48 hours after the sedative was discontinued, his mental status remained depressed and he also developed new onset of conjugate rightward gaze deviation. On neurologic examination, the patient was unresponsive to verbal stimuli and sternal rub. He could not follow any command, including closing or opening eyes and squeezing hands. He had remarkable conjugate, forced eye deviation that could not be corrected to cross the midline using the vestibulo-ocular reflex. Corneal and gag reflexes were preserved. He blinked to visual threat less on the left side, had no clear facial asymmetry, and withdrew on his right arm and knees but had a flaccid left arm. His reflexes were brisker on the left biceps and brachioradialis, and the other deep tendon reflexes were absent. His plantar reflex was upgoing on the left side and downgoing on the right side. CT of the head had no significant findings.

Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare and autosomal recessive syndrome. We describe 2 cases of HGPPS which are the first documented in patients of African ancestry from an isolated population in Cape Verde. They demonstrated typical findings on neuro-ophthalmic examination and brain magnetic resonance imaging. One patient had novel heterozymous mutations of the ROB0 3 gene.

PURPOSE: To determine the frequency and types of vision disorders associated with concussion, and to determine the success rate of vision therapy for these conditions in two private practice settings. METHODS: All records over an 18-month period of patients referred for post-concussion vision problems were reviewed from two private practices. Diagnoses of vergence, accommodative, or eye movement disorders were based on pre-established, clinical criteria. Vision therapy was recommended based on clinical findings and symptoms. RESULTS: Two hundred eighteen patient records were found with a diagnosis of concussion. Fifty-six percent of the concussions were related to sports, 20% to automobile accidents, and 24% to school, work, or home-related incidents. The mean age was 20.5 years and 58% were female. Eighty-two percent of the patients had a diagnosis of an oculomotor problem [binocular problems (62%), accommodative problems (54%), eye movement problems (21%)]. The most prevalent diagnoses were convergence insufficiency (CI, 47%) and accommodative insufficiency (AI, 42%). Vision therapy was recommended for 80% of the patients. Forty-six per cent (80/175) either did not pursue treatment or did not complete treatment. Of the 54% (95/175) who completed therapy, 85% of patients with CI were successful and 15% were improved, and with AI, 33% were successful and 67% improved. Clinically and statistically significant changes were measured in symptoms, near point of convergence, positive fusional vergence, and accommodative amplitude. CONCLUSIONS: In this case series, post-concussion vision problems were prevalent and CI and AI were the most common diagnoses. Vision therapy had a successful or improved outcome in the vast majority of cases that completed treatment. Evaluation of patients with a history of concussion should include testing of vergence, accommodative, and eye movement function. Prospective clinical trials are necessary to assess the natural history of concussion-related vision disorders and treatment effectiveness. Language: en

A 73-year-old woman presented with acute impaired eye movements with preserved left eye abduction, right peripheral facial weakness (video 1), and left hemiparesis/hemihypesthesia. These deficits localized to the right pontine tegmentum involving the medial longitudinal fasciculus, facial nerve, abducens nucleus, medial lemniscus, and corticospinal tracts, as shown in figure 1. After tissue plasminogen activator, her left hemiparesis and hemihypesthesia resolved. Rosini et al.1 first described these deficits as 9 syndrome (7th nerve + 1.5 syndrome + 0.5 hemiparesis/hypesthesia = 9). Classically, 1-and-a-half syndrome consists of intranuclear ophthalmoplegia and conjugate horizontal gaze palsy; preserved abduction will be present in the contralateral eye. Our patient's right eye adduction was paretic and not plegic. Our patient was left with an “8-and-a-half syndrome” (figure 2). Infarct, hemorrhage, vasculitis, and demyelination are etiologies of 8-and-a-half syndromes reported in the literature.

A 48-year-old man developed lightheadedness and diplopia upon awakening 10 days prior. Since then, his double vision had been continuous and stable. Upon admission, neurologic examination revealed bilateral internuclear ophthalmoplegia, abducting nystagmus, and intact convergence reflex (video). His blood pressure was 155/100 mm Hg and HbA1c was 8.7%, suggesting a new diagnosis of diabetes mellitus type 2. CT angiography showed calcified plaques of bilateral cavernous internal carotid arteries. MRI showed a lesion with hyperintensity on T2 fluid-attenuated inversion recovery, contrast enhancement, and mild diffusion restriction ventral to the cerebral aqueduct in the pontomesencephalic junction (figure), which is supplied by interpeduncular perforating branches of posterior cerebral arteries.1 For this patient with hypertension and diabetes mellitus, the acute onset of symptoms and imaging findings suggest ischemic stroke due to arteriolosclerosis of perforating arteries, which should be differentiated from demyelinating diseases such as multiple sclerosis. Involvement of bilateral medial longitudinal fasciculus rather than oculomotor nucleus is a rare consequence of stroke.2

FK506 (tacrolimus) is an immunosuppressive drug and more potent than cyclosporine. FK506 is widely used for immunosuppression in the prevention and treatment of graft-versus-host disease after allogeneic bone marrow transplantation and solid organ transplantation. Neurotoxicity is a recognized complication of FK506 therapy, but ptosis and weakness of eye abduction unilaterally has not been reported in association with FK506 administration to date. We discuss a 13-year-old male patient who developed ptosis and weakness of eye abduction unilaterally 90 days after transplantation with bone marrow from an unrelated donor, for acute lymphoblastic leukemia in this case report. FK506 therapy was administered for graft-versus-host disease prophylaxis and CMV infection was treated with ganciclovir. The physical examination findings completely resolved 72 to 96 hours after concomitant FK506 and ganciclovir treatment were terminated.

Objective: To describe the ocular motor abnormalities in 9 patients with a lesion involving the nucleus prepositus hypoglossi (NPH), a key constituent of a vestibular-cerebellar-brainstem neural network that ensures that the eyes are held steady in all positions of gaze. Methods: We recorded eye movements, including the vestibulo-ocular reflex during head impulses, in patients with vertigo and a lesion involving the NPH. Results: Our patients showed an ipsilesional-beating spontaneous nystagmus, horizontal gaze-evoked nystagmus more intense on looking toward the ipsilesional side, impaired pursuit more to the ipsilesional side, central patterns of head-shaking nystagmus, contralateral eye deviation, and decreased vestibulo-ocular reflex gain during contralesionally directed head impulses. Conclusions: We attribute these findings to an imbalance in the NPH–inferior olive–flocculus–vestibular nucleus loop, and the ocular motor abnormalities provide a new brainstem localization for patients with acute vertigo.

Ocular neuromyotonia is a rare, but well-recognized, complication of cranial irradiation.Using figures and videos, we report a 52-year-old man with extensive ocular, brainstem, and lower cranial nerve neuromyotonia postradiation therapy for a fourth ventricle glioma who, in the context of an apparently positive edrophonium test, was initially misdiagnosed with myasthenia gravis.This is the first case of postirradiation neuromyotonia to be reported with such extensive cranial nerve and brainstem involvement.

PURPOSE OF REVIEW: Concussions and their related sequelae have received significant attention given the high-profile media coverage from professional sports and recreational leagues. A better understanding of the diagnosis and symptom management may limit the long-term impact these injuries have on the affected individual. The aim of this review is to provide updated information for both diagnosis and ongoing management for visual symptoms of concussions. RECENT FINDINGS: New testing including a brief vestibular/ocular motor screening assessment and the importance of near point of convergence measurements may prove beneficial to the diagnosis and identification of patients at greater risk for developing postconcussion syndrome. Additionally, the development of postconcussion syndrome is more likely when symptom burden is greater upon presentation. SUMMARY: Currently, there is not a single testing method that can universally identify all individuals with concussion. Current management of concussion focuses on targeted treatment based upon symptoms and signs present at onset to decrease disease burden and help restore baseline functioning as soon as possible. Language: en

A 57-year-old immunosuppressed renal transplant recipient experienced 2 months of oscillopsia, vertigo, ataxia, and headaches. The headaches were described as constant, bifrontal, dull, and associated with photophobia and nausea but no aura or positional component. She described rotational vertigo, both at rest and provoked by leftward head turn and gait instability that developed gradually over the last 4 weeks. Six months prior to presentation, she had a similar episode of headaches, dizziness, and ataxia that resolved spontaneously over 3 months. This was preceded by a dermatomal vesicular rash on the arm. She had undergone renal transplantation 12 years earlier. Three years ago, she had left C2-3 distribution zoster. She denied infectious or rheumatologic symptoms. Her only current medication was mycophenolate mofetil 360 mg PO BID.

PURPOSE To describe ocular motility and effectiveness of an extended conjunctival incision with reattachment of the inferior oblique muscle to repair combined orbital medial and inferior wall fractures. METHODS The authors retrospectively studied 20 eyes from 20 patients who underwent combined orbital medial and inferior wall fractures surgery with reattachment of the inferior oblique muscle from January 2007 to December 2011. Single large L-shaped implant was inserted into the fracture site. All patients were evaluated preoperatively and postoperatively at 1, 3, and 6 months, by computed tomography, Hertel exophthalmometry, the Hess test of ocular motility, and the Goldmann diplopia test. RESULTS Sixteen patients (80%) had no significant enophthalmos or diplopia after surgery. Inferior oblique motility was not changed in 12 patients (60%) after reattachment of the inferior oblique muscle. After 1 month, inferior oblique underaction was presented in 8 patients (40%) and in 4 patients (20%) after 6 months. Finally, only 2 patients (10%) had the inferior oblique underaction remained, it was not improved. CONCLUSIONS The extended conjunctival incision with reattachment of the inferior oblique muscle for combined orbital wall fracture repair offers a wide surgical field and space for a single large implant insertion and corrects the enopthalmos. The reattachment of the inferior oblique muscle does not contribute to the development of inferior oblique underactions or diplopia that was resolved spontaneously within 6 months after surgery.

Mitochondrial encephalopathy, lactic acidosis, and stroke-like symptoms (MELAS) and MIDD (maternally-inherited diabetes mellitus and deafness) are caused by A3243G transfer RNA mutations that affect mitochondrial function. Hearing loss and early onset diabetes mellitus constitute the main MIDD phenotype. Regarding the ophthalmologic manifestations of MIDD, we hypothesized that decreased vestibulo-ocular reflex (VOR) gain in patients with MIDD may contribute to impaired dynamic visual acuity.Neuro-ophthalmologic, neuroimaging, and neuro-otologic evaluations were performed in 2 nonrelated patients with MIDD who complained of oscillopsia with head movement. We obtained quantitative recording of the horizontal and the vertical VOR, using the video head impulse test device.In the 2 patients, we detected visual, ocular motor, and vestibular abnormalities. Decreased VOR gain in the planes of all 3 semicircular canals and impaired dynamic visual acuity was demonstrated in both cases.MIDD patients are primarily recognized by their advanced hearing loss or deafness, early onset diabetes mellitus, and lactic acidosis. Decreased vision in these patients relates primarily to peri-macular retinal atrophy. In addition, loss of vestibular function causes poor dynamic visual acuity. Both patients, in their late fifties, had evidence of progressive central and peripheral nervous system dysfunction.

A 46-year-old Chinese man with diabetes mellitus, hypertension, and hyperlipidemia, developed sudden onset of vertiginous dizziness and diplopia that was worse on looking up. His symptoms were not preceded by any antecedent infections, neither was there any headache. Blood pressure on arrival in the Emergency Department was 145/95 mm Hg and his neurologic examination revealed bilateral upward gaze restriction (video on the Neurology ® Web site at [Neurology.org][1]). There was voluntary lid retraction by the patient. The pupils were 3 mm bilaterally and reactive to light and accommodation, there was no ptosis or nystagmus, and no fatigability could be elicited. Apart from esophoria and pseudo-6th nerve palsy, the rest of the eye movements were normal. Other cranial nerves, motor, sensory, and cerebellar examinations were unremarkable. The vertical optokinetic reflex was intact and there was no tremor of the upper limbs. On forced eye closure, there was upward deviation of his eyes consistent with the Bell phenomenon. ECG did not show any atrial fibrillation or ischemic changes. The basic blood investigations were unremarkable. He was initially diagnosed with myasthenia gravis with frontalis overactivity and complex ophthalmoplegia by the admitting emergency physicians. [1]: http://neurology.org/lookup/doi/10.1212/WNL.0000000000002629

BACKGROUND Internet video sharing sites allow the free dissemination of educational material. This study investigated the quality and educational content of videos of eye movement disorders posted on such sites. METHODS Educational neurological eye movement videos were identified by entering the titles of the eye movement abnormality into the search boxes of the video sharing sites. Also, suggested links were followed from each video. The number of views, likes, and dislikes for each video were recorded. The videos were then rated for their picture and sound quality. Their educational value was assessed according to whether the video included a description of the eye movement abnormality, the anatomical location of the lesion (if appropriate), and the underlying diagnosis. RESULTS Three hundred fifty-four of these videos were found on YouTube and Vimeo. There was a mean of 6,443 views per video (range, 1-195,957). One hundred nineteen (33.6%) had no form of commentary about the eye movement disorder shown apart from the title. Forty-seven (13.3%) contained errors in the title or in the text. Eighty (22.6%) had excellent educational value by describing the eye movement abnormality, the anatomical location of the lesion, and the underlying diagnosis. Of these, 30 also had good picture and sound quality. The videos with excellent educational value had a mean of 9.84 "likes" per video compared with 2.37 for those videos without a commentary (P < 0.001). The videos that combined excellent educational value with good picture and sound quality had a mean of 10.23 "likes" per video (P = 0.004 vs videos with no commentary). There was no significant difference in the mean number of "dislikes" between those videos that had no commentary or which contained errors and those with excellent educational value. CONCLUSIONS There are a large number of eye movement videos freely available on these sites; however, due to the lack of peer review, a significant number have poor educational value due to having no commentary or containing errors. The number of "likes" can help to identify videos with excellent educational value but the number of "dislikes" does not help in discerning which videos have poor educational value.

Little is known about the prevalence of refractive error, binocular vision, and other visual conditions in Australian Indigenous children. This is important given the association of these visual conditions with reduced reading performance in the wider population, which may also contribute to the suboptimal reading performance reported in this population. The aim of this study was to develop a visual profile of Queensland Indigenous children.Vision testing was performed on 595 primary schoolchildren in Queensland, Australia. Vision parameters measured included visual acuity, refractive error, color vision, nearpoint of convergence, horizontal heterophoria, fusional vergence range, accommodative facility, AC/A ratio, visual motor integration, and rapid automatized naming. Near heterophoria, nearpoint of convergence, and near fusional vergence range were used to classify convergence insufficiency (CI).Although refractive error (Indigenous, 10%; non-Indigenous, 16%; p = 0.04) and strabismus (Indigenous, 0%; non-Indigenous, 3%; p = 0.03) were significantly less common in Indigenous children, CI was twice as prevalent (Indigenous, 10%; non-Indigenous, 5%; p = 0.04). Reduced visual information processing skills were more common in Indigenous children (reduced visual motor integration [Indigenous, 28%; non-Indigenous, 16%; p0.01] and slower rapid automatized naming [Indigenous, 67%; non-Indigenous, 59%; p = 0.04]). The prevalence of visual impairment (reduced visual acuity) and color vision deficiency was similar between groups.Indigenous children have less refractive error and strabismus than their non-Indigenous peers. However, CI and reduced visual information processing skills were more common in this group. Given that vision screenings primarily target visual acuity assessment and strabismus detection, this is an important finding as many Indigenous children with CI and reduced visual information processing may be missed. Emphasis should be placed on identifying children with CI and reduced visual information processing given the potential effect of these conditions on school performance.

A 63-year-old man experienced transient vertical oscillopsia lasting several seconds for 2 months. Examination disclosed paroxysmal excyclotorsion of the right eye, spontaneously or triggered by adduction. Eye movements using 3D video-oculography showed intermittent, monocular phasic movements which consisted of excyclotorsion of the right eye mixed with a small amount of supraduction and abduction, and a tonic movement with excyclotorsion and slight elevation. Orbital and brain MRI was unremarkable. Administration of oxcarbazepine markedly decreased the severity and frequency of the episodes. The oculographic characteristics in our patient may indicate that inferior oblique myokymia may be attributed to aberrant, spontaneous discharges in the inferior oblique motor unit.

Conjugate gaze deviation is associated with acute ischemic stroke (AIS), although previously only measured on a 2D plane. The current study evaluates 3D imaging efficacy to assess conjugate gaze deviation and correlate direction and strength of deviation to neuro-clinical findings. A retrospective analysis of 519 patients who had CT scans for suspected AIS at our institution. Direction and angle of eye deviation were calculated based on 2D axial images. Volumetric reconstruction of CT scans allowed for calculation of 3D conjugate gaze adjusted length (CGAL). Angle, direction, and vector strength of both 2D and 3D scans were calculated by an artificial intelligence algorithm and tested for agreement with hemispheric ischemia location. CGAL measurements were correlated to NIHSS scores. Follow up MRI data was used to evaluate the sensitivity and specificity of CGAL in the identification of AIS. The final analysis included 122 patients. A strong agreement was found between 3D gaze direction and hemispheric ischemia location. CGAL measurements were highly correlated with NIHSS score (r = .72, P = .01). A CGAL >0.25, >0.28, and >0.35 exhibited a sensitivity of 91%, 86%, and 82% and specificity of 66%, 89%, and 89%, respectively, in AIS identification. A CGAL >0.28 has the best sensitivity-specificity balance in the identification of AIS. A CGAL >0.25 has the highest sensitivity. Given CED's correlation with NIHSS score a 1/4 deviation in the ipsilateral direction is a sensitive ancillary radiographic sign to assist radiologists in making a correct diagnosis even when not presented with full clinical data.

Background Progressive multifocal leukoencephalopathy (PML) is a severe often fatal opportunistic infection of the central nervous system caused by reactivation of a ubiquitous polyoma virus, JC virus. Although typically characterized by multifocal asymmetric subcortical white matter lesions, it may be monofocal and affect the cortical gray matter. Among the broad spectrum of clinical manifestations that occurs with PML, visual complaints are common. Evidence acquisition Combination of representative personally observed cases of PML and comprehensive review of case series of PML from 1958 through 2014. Results Neuro-ophthalmic signs and symptoms were reported in approximately 20%-50% of patients with PML and can be the presenting manifestation in half of these. A majority of these presentations occur from damage to cerebral visual pathways resulting in visual field defects, cortical blindness, and other disorders of visual association. Given the decreased frequency of infratentorial and cerebellar involvement, ocular motility disorders are less common. Conclusions Visual complaints occur in patients with PML and are often the presenting sign. Awareness of this condition is helpful in avoiding unnecessary delays in the diagnosis of PML and management of the underlying condition. Recent guidelines have established criteria for diagnosis of PML in the high-risk patient population and strategies to mitigate the risk in these populations.

Purpose The forced duction test (FDT) is important during the surgical treatment of ocular motility disturbance due to orbital wall fracture, but the lack of objective standards yields various test results based on individual examiners' subjective assessments. Methods Medial limbus abduction values of the affected eye were measured during preoperative and postoperative FDT, and the values were compared with those of the normal eye. Results More accurate results were obtained using the modification of the FDT in the surgical repair of medial orbital wall fracture with entrapments. Conclusions The modified FDT used here in medial orbital wall fracture with limited ocular motility can contribute to more effective and positive surgical outcomes by providing an objective reference point.

Stiff-person syndrome (SPS) is an autoimmune disease characterized by muscle rigidity, episodic muscle spasms, as well as continuous cocontraction of agonist and antagonist muscles.