Bilateral Internuclear Ophthalmoplegia Research Articles

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) as part of the clinical presentation of pediatric multiple sclerosis, successfully treated with therapeutic plasma exchange

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) as part of the clinical presentation of pediatric multiple sclerosis, successfully treated with therapeutic plasma exchange

Multiple Sclerosis: An Emergency Medicine-Focused Narrative Review

BackgroundMultiple sclerosis (MS) is a rare but serious condition associated with significant morbidity. ObjectiveThis review provides a focused assessment of MS for emergency clinicians, including the presentation, evaluation, and emergency department (ED) management based on current evidence. DiscussionMS is an autoimmune disorder targeting the central nervous system (CNS), characterized by clinical relapses and radiological lesions disseminated in time and location. Patients with MS most commonly present with long tract signs (e.g., myelopathy, asymmetric spastic paraplegia, urinary dysfunction, Lhermitte's sign), optic neuritis, or brainstem syndromes (bilateral internuclear ophthalmoplegia). Cortical syndromes or multifocal presentations are less common. Radiologically isolated syndrome and clinically isolated syndrome (CIS) may or may not progress to chronic forms of MS, including relapsing remitting MS, primary progressive MS, and secondary progressive MS. The foundation of outpatient management involves disease-modifying therapy, which is typically initiated with the first signs of disease onset. Management of CIS and acute flares of MS in the ED includes corticosteroid therapy, ideally after diagnostic testing with imaging and lumbar puncture for cerebrospinal fluid analysis. Emergency clinicians should evaluate whether patients with MS are presenting with new-onset debilitating neurological symptoms to avoid unnecessary testing and admissions, but failure to appropriately diagnose CIS or MS flare is associated with increased morbidity. ConclusionsAn understanding of MS can assist emergency clinicians in better diagnosing and managing this neurologically devastating disease.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Bilateral Internuclear Ophthalmoplegia: A Case Report.

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Bilateral Internuclear Ophthalmoplegia: A Case Report.

Convergence Exercises for Convergence Insufficiency Type Exotropia in Bilateral Internuclear Ophthalmoplegia

Purpose; To report a case of successfully treated convergence insufficiency type exotropia (CI-XT) in the context of bilateral internuclear ophthalmoplegia (BINO) using only convergence exercises.
 Observations; A 17-year-old male with a history of a resected posterior fossa pilocytic astrocytoma presented with constant diplopia since the craniotomy. Patient and his parents were adamant in pursuing surgical treatment for his new onset strabismus. With appropriate counseling and management with convergence exercises only, the CI-IXT was successfully treated without surgery over the course of two years.
 Conclusion and Importance; We present a non-invasive approach to the treatment of CI-IXT in the context of a BINO, also known as wall-eyed BINO (WEBINO). This approach should be considered before any surgical intervention, especially if prism is unable to successfully neutralize diplopia for any extended period of time.

From WEMINO To WEBINO With Unilateral LMN Facial Palsy (P5-4.002)

<h3>Objective:</h3> To describe a case of progression from apparent trochlear nerve palsy, to wall-eyed monocular internuclear ophthalmoplegia (WEMINO), to wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) with unilateral lower motor neuron (LMN) facial palsy. <h3>Background:</h3> Various descriptions of INO with cranial nerve palsies are in the literature, but we report a patient with a remarkably instructive stepwise evolution. <h3>Design/Methods:</h3> Review of records of a patient with diplopia at our institution. <h3>Results:</h3> A 42-year-old woman presented acutely with oblique binocular diplopia worsening on right gaze and left head tilt, with left hypertropia. Trochlear nerve palsy was suspected but contrasted MRI brain with orbits, and initial CSF testing were unrevealing. On day 3, the hypertropia partly improved but she developed left eye adduction deficit. Given the fluctuation, myasthenia gravis was considered high in the differential diagnosis while test results were awaited. On day 7, she developed LMN left facial palsy and bilateral adduction deficit with abducting eye nystagmus, leading to the realization that a single progressing lesion in the pontine tegmentum could explain her entire sequence of events. Repeat MRI confirmed a minute enhancing lesion in the floor of the fourth ventricle corresponding to the dorsomedial surface of the left facial colliculus and bilateral medial longitudinal fasciculi (MLF), likely of demyelinating etiology. Her pending CSF tests returned with 7 oligoclonal bands. She received intravenous methylprednisolone and her symptoms nearly resolved by day 14. <h3>Conclusions:</h3> This patient presented with evolving symptoms that appeared to localize to the trochlear nerve initially, but lacked imaging correlate. It is clear in retrospect that her initial presentation represented a skew deviation from early INO, progressing to WEMINO and eventually WEBINO with unilateral fascicular facial palsy. The anatomical correlate was confirmed on repeated imaging. Her clinical course beautifully illustrates the importance of lesion localization for etiological diagnosis. <b>Disclosure:</b> Dr. Senthamarai Siddharthan has nothing to disclose. Dr. Witt has nothing to disclose. Dr. Kini has nothing to disclose. Dr. Vasireddy has nothing to disclose. The institution of Dr. Mathur Kumaraswamy has received research support from Woolsey Pharmaceuticals.

Teaching Video NeuroImages: Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia

A 65-year-old man with a history of hypertension presented with double vision. Examination revealed wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) (figure 1) and nonsymptomatic, pulse-synchronous, pulsatile proptosis of the left eye (video 1). According to the patient, there was a history of blunt head trauma in childhood and the pulsatile eye was present since then. CT revealed a meningocele into the left orbit due to a bony defect in the orbital roof (figure 2A). CT angiography ruled out a carotid-cavernous fistula. MRI showed an acute infarction at the midline of the midbrain tegmentum, which involved the medial longitudinal fasciculus bilaterally and caused the WEBINO (figure 2B).1 The pulsatile proptosis was attributed to the childhood head injury and due to the orbital roof fracture and associated meningocele pulsation of the brain vessels passed onto the CSF.2

Orbital Myositis Mimicking Wall-Eyed Bilateral Internuclear Ophthalmoplegia

A 56-year-old woman reported a 2-week history of diplopia associated with mild pain and periorbital edema. On examination, the patient had a large exotropia (A). While vertical eye movements were intact, there was limited adduction of each eye on right (B) and left (C) gaze. These findings were consistent with wall-eyed bilateral internuclear ophthalmoplegia. Postcontrast axial T1 fat-suppressed magnetic resonance imaging (D) revealed bilateral enlargement and enhancement of the medial rectus muscles indicative of orbital myositis.

Neuro-Optometric Treatment of Wall-eyed Bilateral Internuclear Ophthalmoplegia (WEBINO)

Background Wall-eyed Bilateral Internuclear Ophthalmoplegia (WEBINO) is an ocular motility disorder characterized by bilateral exotropia in primary gaze, bilateral internuclear ophthalmoplegia with defective adduction of both eyes and nystagmus of the contralateral eye on abduction. Convergence is impaired because the lesion is in the midbrain. The etiology of WEBINO includes demyelination, stroke, and tumors, but midbrain infarction is the most common.1 Although extraocular muscle surgery and botulinum toxin injections are typically used for management2 of WEBINO, neuro-optometric rehabilitation can be utilized as an effective option to improve vision. Case Report A 39-year-old Caucasian male with non-traumatic brain injury due to left intraparenchymal hemorrhage from stroke, presented with WEBINO and multiple vision signs and symptoms, including blurred vision, alternating exotropia, diplopia, and strabismus. During the previous 8 months, he participated in physical therapy, occupational therapy, and speech therapy. He had difficulty participating in his therapies due to his vision issues. Single vision distance lenses were prescribed to improve vision clarity and 6 in-office neuro-optometric rehabilitation sessions were administered to improve range of motion of eye movements and to improve vision. Conclusions Neuro-optometric rehabilitation utilizes a systematic approach to treating patients with binocular vision dysfunction, while integrating cognitive, motor, and sensory skills. Prognosis is optimized with an individualized approach. Top-down processing stimulates the brain in a more direct way to elicit plasticity-mediated motor relearning.3 In this case, extraocular muscle surgery and botulinum toxin injections were not optimal choices and neuro- optometric rehabilitation was utilized to manage WEBINO and improve quality of life.

WEBINO (Wall-eyed bilateral internuclear ophthalmoplegia) syndrome due to mesencephalon infarction

WEBINO (Wall-eyed bilateral internuclear ophthalmoplegia) syndrome due to mesencephalon infarction

Multiple sclerosis and occlusive retinal vasculitis: A case series

AbstractPurpose: Retinal vascular changes are a common finding in patients with multiple sclerosis (MS). While simple peripheral retinal periphlebitis is by far the most frequent manifestation, a spectrum exists that ranges up to the presence of proliferative retinal vasculitis.Methods: Case series of four patients with occlusive retinal vasculitis.Results: Three patients presented with complaints of visual loss and ocular discomfort: a 26‐year‐old man presented with vitreous inflammation, vitreous haemorrhage, macular edema and papillitis; a 44‐year‐old woman presented with intermediate uveitis and macular edema; and a 25‐year‐old woman presented with granulomatous panuveitis, papillitis and foveolar serous detachment. A fourth patient was addressed because of abnormal eye movements detected during a work‐up for dizziness. Examination revealed bilateral internuclear ophthalmoplegia with adduction lag. He had no visual complaints other than subtle floaters in the left eye. Anterior segment examination showed no signs of inflammation, whereas vitreous snowballs were present. Fluorescein angiography revealed occlusive retinal vasculitis in all patients, resulting in active neovascularization in two patients with vitreous haemorrhage in one of them. All patients required retinal laser photocoagulation. Extensive work‐up revealed multiple sclerosis as the underlying disease in three patients; the remaining patient had already been diagnosed with MS at the time of presentation. Nevertheless, additional work‐up was performed in this patient to exclude other possible causes.Conclusions: Occlusive retinal vasculitis is a rare manifestation in patients with MS and may result in neovascularization, vitreous haemorrhage, retinal detachment and neovascular glaucoma. MS should therefore be considered in the differential diagnosis of ischemic retinal disease. In addition, one should be alert for signs of retinal vasculitis in patients with MS, as it can present with few or no symptoms.

Conservative and Surgical Management of Unilateral and Bilateral Internuclear Ophthalmoplegia (INO)-A Retrospective Analysis.

To report the outcomes of the natural progression and ophthalmic treatment of patients reviewed in a tertiary hospital trust with unilateral or bilateral internuclear ophthalmoplegia. A retrospective case note analysis was performed and 33 patients diagnosed with unilateral or bilateral internuclear ophthalmoplegia (INO) were identified. The diagnosis, aetiology, presence of diplopia, ophthalmic management options and progression were recorded and analysed. This included both conservative and surgical management. The most common aetiologies of INO within this cohort were stroke/ischaemic (69.7%) and multiple sclerosis (MS) (30.3%). Unilateral INO was more prevalent than bilateral INO, with 20 cases (60.6%) compared to 13 cases (39.4%), respectively. A higher proportion of unilateral INO were attributed to stroke (90%) whilst a higher proportion of bilateral INO were attributed to MS (61.5%). The most prescribed management at primary assessment was occlusion (45.5%) and prisms (24.2%). Some patients required no orthoptic intervention (30.3%). Two patients had surgical management of strabismus secondary to bilateral INO. Occlusion was the most common form of management for symptomatic relief of diplopia. Patients who presented at the first visit with no symptoms were unlikely to need any orthoptic intervention. Of the two patients who went on to require surgical intervention, restoration of binocular single vision (BSV) was achieved post-operatively with the use of a Fresnel prism. However, the differences in both surgical technique and number of surgeries required make this difficult to generalise. Additional research is needed to further explore the surgical management of INO.

Titanium T-Plate as a Stabilizing Platform in the Management of Acquired Nystagmus and Oscillopsia Without a Null Zone.

The primary goal of nystagmus treatment is to improve visual function and quality of life. Current surgical interventions are limited by regression of effect, serious complications, and reliance on a null zone. Tse and colleagues in 2017 reported the use of a T-plate/suture fixation platform for globe stabilization to treat acquired nystagmus and oscillopsia without a null zone in a patient with bilateral internuclear ophthalmoplegia. However, the improvement in nystagmus was not objectively quantified. This case series reports 2 patients who underwent bilateral T-plate placement which resulted in immediate and sustained improvement of nystagmus objectively measured by videonystagmography.

Oblique saccades in internuclear ophthalmoplegia.

Oblique saccades often display component stretching, in which the shorter vector in one cardinal direction is slowed so that its duration matches that of the longer vector in the orthogonal direction, resulting in a straighter trajectory. In internuclear ophthalmoplegia, adducting saccades are typically slowed while vertical saccades are unaffected. It is not known whether these slowed adducting movements are accompanied by adaptive component stretching of the vertical vector during oblique saccades. This was a cross-sectional study. We recorded the saccadic eye movement in 5 patients with right or bilateral internuclear ophthalmoplegia from multiple sclerosis and 17 healthy controls, using an EyeLink 1000 machine. The target stimulus was located at varying angles (0-360) and amplitudes (4, 8, 12 degrees). For each saccade we have calculated the curvature index as the main outcome measure, which is the area between the actual and ideal straight trajectory for oblique saccadic eye movements, divided by the square of the length of the straight trajectory, to give a unit-less metric for curvature. In the 17 control subjects, curvature showed a strong positive correlation between adducting saccades and the yoked abducting saccades of the other eye. In internuclear ophthalmoplegia, adducting saccades showed a strong curvature concave to the horizontal meridian, indicating inadequate component stretching, while abducting saccades did not differ from controls. This new sign of oblique saccadic curvature in internuclear ophthalmoplegia indicates a limitation of the range of central adaptive changes in response to distal lesions affecting transmission of the saccadic command.

Importance of monitoring the ocular surface of patients with Kearns‐Sayre syndrome: a case report

AbstractPurposeKearns‐Sayre Syndrome (KSS) is a congenital mitochondrial myopathy initially described by the triad: Progressive external ophthalmoplegia, pigmentary retinopathy, heart block. Surgical procedures to alleviate ptosis can cause corneal injury from exposure. The lack of blinking can lead to a difficult contraction of the muscles responsible for squeezing the meibomian glands causing their dysfunction (MGD). Therefore, the follow‐up is of special interest in those cases where we suspect KSS due to the probable appearance of complications.MethodsWe present the case of a male of 9 years old who presented a sudden‐onset ptosis and bilateral internuclear ophthalmoplegia. Initially, he was diagnosed with Myasthenia. At the age of 12, resection of the elevator upper eyelid was performed in both eyes (BE). In the successive visits: negative Bell's sign, a progressive MGD and significant corneal thinning in the lower half of the BE were observed, and the right eye (RE) was affected with an infiltrated ulcer.ResultsTreatment with high hydration in BE and RE was treated with antibiotics eye drops and antibiotic ophthalmic ointment. After reviewing the history and given the high suspicion of KSS, genetic tests were requested to confirm it. Maintenance treatment was settled in BE with high hydration, 20% autologous serum and therapeutic contact lens. After a year of exhaustive monthly follow‐up, the patient remains stable and there is no progression of the corneal thinning.ConclusionsIt is important to take care of the ocular surface in these cases of KSS due to the corneal exposure after ptosis surgery. We must achieve stability in terms of symptoms and visual acuity without incurring other complications such as infections or perforation.

Bilateral internuclear ophthalmoplegia developing as a manifestation of dengue fever and treatment by modified Nishida procedure: A case report and review of the literature

<br>Typical causes of internuclear ophthalmoplegia (INO) include demyelinating conditions, with inflammation, infection, and trauma being less common causes. Diplopia associated with INO severely impacts the patient's quality of life. Orthoptic treatment usually fails; therefore, appropriate surgical treatment should be considered. We report the unique case of a 25-year man with bilateral INO developing as a manifestation of dengue fever-related encephalitis and its successful treatment by modified Nishida procedure.<br>

A case of cardioembloic stroke with wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome

Here, we report a case of an 85-year-old man who presented sudden onset of diplopia, dysarthria, and gait disturbance. On admission, he exhibited bilateral adduction palsy, convergence palsy, and binocular exotropia in the forward gaze showing wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome. He had a history of chronic nonvalvular atrial fibrillation. DWI-MRI revealed acute ischemic lesions in the paramedian pontine tegmentum, lower midbrain, both cerebellar hemispheres, and left frontal cortex. He was thus diagnosed with an acute phase of cardioembolic stroke. Subsequently, the right eye adduction palsy in the forward gaze was slightly improved, but other eye movement disorders persisted during discharge from the hospital. The pathology was suspected to involve bilateral damages to both medial longitudinal fasciculus and the paramedian pontine reticular formation. WEBINO syndrome was not only ascribed to lacunar infarction and large artery atherosclerosis but also cardioembolic stroke. The presence of other non-eye symptoms and multiple ischemic lesions could be the characteristics of WEBINO syndrome following cardioembolic stroke.

Cerebrovascular Infarction Presenting as Bilateral Internuclear Ophthalmoplegia in a 75 y/o Patient

Objective To present a case of Bilateral Internuclear Ophthalmoplegia in a 75yo patient Case Description A 75 year old, Filipino, male, known hypertensive for 10 years, but not compliant to medications, presented with a sudden onset binocular horizontal, diplopia and bilateral exotropia associated with dizziness. No other symptoms like slurring of speech, body weakness, numbness were noted. Primary gaze exotropia, abduction nystagmus, gaze evoked vertical nystagmus were also noted. Imaging was done which revealed hypodense focus in the subcortical region of the right centrum semiovale extending to the right corona radiata, with unremarkable orbits, midbrain and pons findings. WEBINO was still considered even without the presence of a pontine lesion in the imaging. The patient was advised patching and was asked to follow up after a month

Wall-eyed bilateral internuclear ophthalmoplegia in posterior circulation stroke-a case report

Internuclear ophthalmoplegia is characterised by restricted ocular motility in lateral gaze in which the affected eye shows impairment of adduction and it results from damage to medial longitudinal fasciculus (MLF). Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an extremely rare neurological manifestation which has typical signs including primary gaze exotropia, vertical gaze palsy, ptosis, abducting nystagmus. The common and serious etiological factor is cerebrovascular accident involving the vessels supplying MLF and many cases have life threatening associated neurological impairment. In this case report we have discussed about a gentleman who presented with bilateral ptosis, primary gaze exotropia and headache. Patient found to have vertical gaze palsy and abducting nystagmus on examination. Computed tomography (CT) imaging shows infarct in pontine region and CT angiography revealed basilar artery occlusion supplying region of pons with involvement of posterior cerebral artery. Patient treated with antiplatelet and diplopia managed. Patient showed improvement on subsequent follow-up visits.

Exotropic bilateral internuclear ophthalmoplegia following a superior cerebellar artery aneurysm clipping

A 62-year-old man developed binocular diplopia during hospitalization for an upper-gastrointestinal bleed 1 month after clipping of a superior cerebellar artery aneurysm. Gradient echo showed punctate microhemorrhage at the level of left inferior colliculus (Fig. 1). Motility examination revealed primary gaze left hypertropia (presumed skew deviation) with bilateral exotropia, adduction deficits, and dissociated horizontal abducting nystagmus and downbeat component with intact convergence, consistent with an exotropic bilateral internuclear ophthalmoplegia (EBINO; see Video).

Wernekink commissure syndrome: clinico-radiological criteria

Introduction: Wernekink commissure syndrome is a rare midbrain syndrome selectively affecting the Wernekink commissure, characterized by bilateral cerebellar ataxia and eye movement disorders, especially internuclear ophthalmoplegia. This article aims at proposing clinico-radiological criteria for Wernekink commissure syndrome with review of the neural circuitary responsible, to aid in recognition and reporting.&#x0D; Methods: This was a prospective study conducted at Department of Neurology, at Pushpagiri institute of medical sciences and research centre, Thiruvalla, Kerala, India, over a period of 5 years among patient with pure midbrain syndromes. All patients with pure midbrain infarction were studied. Subjects presenting with clinical features of Wernekink commissure syndrome were shortlisted and were assessed by investigators independently. Neuroradiology was assessed by investigators 1 and 2, independently. The demographic profile, risk factors, clinical features, neuroimaging findings and outcomes were analysed using SPSSv21.&#x0D; Results: Details of 43 subjects with pure midbrain stroke were included in the study. 8 had clinical features of Wernekink commissure syndrome. The most common findings were bilateral ataxia and unilateral or bilateral internuclear ophthalmoplegia. None of our patients had palatal tremor. Unilateral caudal paramedical infarction was seen on MRI in 5 patients, whereas it was bilateral in 3 patients.&#x0D; Conclusions: The proposed clinico-radiological criteria consisting of all of the essential criteria (Ipsilateral internuclear ophthalmoplegia, Unilateral or bilateral ataxia and Neuroradiological evidence of Caudal midbrain involvement) with or without one among the supportive criteria (Rubral Tremor, Palatal myoclonus) can safely point at a diagnosis of Wernekink commissure syndrome.