Wall-eyed 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

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.

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

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

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.

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.

WEBINO syndrome (wall-eyed bilateral internuclear ophthalmoplegia) secondary to ischemic stroke, about a case

WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) syndrome is characterized by bilateral adduction impairment, nystagmus of the abducting eye, and primary gaze exotropia. We present the case of a 68 year-old man who was initially attended in emergency department with sudden onset diplopia. Neurological exploration revealed WEBINO and gait ataxia. Relevant medical history included liver transplantation and subsequent tacrolimus prescription. Complementary exams revealed ischemic lesion in mesencephalic tegmentum, involving medial longitudinal fasciculus and pretectal area. WEBINO syndrome is unfrequent. Among its etiologies, ischemic and demyelinating are the most frequent. In our case, iatrogenic etiology was also considered. Clinical recognition of this syndrome is required to perform adequate exams in order to reach diagnosis.

Síndrome de WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) secundario a ictus isquémico, a propósito de un caso

WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) syndrome is characterized by bilateral adduction impairment, nystagmus of the abducting eye, and primary gaze exotropia. We present the case of a 68 year-old man who was initially attended in emergency department with sudden onset diplopia. Neurological exploration revealed WEBINO and gait ataxia. Relevant medical history included liver transplantation and subsequent tacrolimus prescription. Complementary exams revealed ischemic lesion in mesencephalic tegmentum, involving medial longitudinal fasciculus and pretectal area. WEBINO syndrome is unfrequent. Among its etiologies, ischemic and demyelinating are the most frequent. In our case, iatrogenic etiology was also considered. Clinical recognition of this syndrome is required to perform adequate exams in order to reach diagnosis.

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

Commenting on “Teaching Video NeuroImages: Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia,” Lee et al. offer an alternative interpretation of the MRI findings. They explain that the lesion suggested by the authors is chronic in nature and too anterior in the pons. They propose that the more likely explanation for the patient's wall-eyed bilateral internuclear ophthalmoplegia is the symmetric high-signal intensity lesions just beneath the facial colliculi. Authors Papageorgiou et al. reviewed the diffusion-weighted MRI of their patient and found that it showed a focal area of restricted diffusion at the midline of the pontine tegmentum beneath the floor of the fourth ventricle, which involves the medial longitudinal fasciculus bilaterally as correctly suggested by Lee et al. A correction appears on page 900. Commenting on “Teaching Video NeuroImages: Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia,” Lee et al. offer an alternative interpretation of the MRI findings. They explain that the lesion suggested by the authors is chronic in nature and too anterior in the pons.

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

We read with interest the Papageorgiou et al.1 Teaching Video NeuroImage on pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia. While this is a well-written case report with a good quality video, we offer an alternative interpretation of the MRI findings. At the pontine level, the medial longitudinal fasciculus (MLF) is situated beneath the floor of the fourth ventricle on either side of the midline,2 and lesions involving bilateral MLFs can cause wall-eyed bilateral internuclear ophthalmoplegia (WEBINO).3 However, the lesion that the authors indicated with the arrow on the T2-weighted MRI (figure, white arrow)1 is located more anteriorly compared to the area of the MLF on MRIs in previous reports3 and on anatomic templates.2 In addition, the lesion indicated appears chronic given the strong high signal intensity, similar to that of the CSF, even though the interval from the symptom onset to MRI was not provided. Instead, we propose that the symmetric high signal intensity lesions just beneath the facial colliculi were responsible for WEBINO in this patient (figure, black arrow added).

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

We thank Lee et al. for the insightful comments on our Teaching Video NeuroImage on pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia (WEBINO).1 The causative lesions for bilateral WEBINO can be located in the tegmentum of pons, pons–midbrain, or midbrain, with some injuries almost bisecting the brainstem.2,3 As the MRI presented by Lee et al. discloses multiple hyperintense foci at the pontomesencephalic level, we reviewed the diffusion-weighted MRI, which shows a focal area of restricted diffusion at the midline of the pontine tegmentum beneath the floor of the fourth ventricle, which involves the medial longitudinal fasciculus (MLF) bilaterally (figure). The patient had presented 2 days after onset of diplopia and this lesion was consistent with an acute ischemic stroke. Although we indicated in the text and figure legend of the T2-weighted MRI that the infarction was located at the midline of the midbrain tegmentum,1 the arrow on the MRI had been placed more anteriorly by mistake. Hence, we agree with Lee et al. that, at the pontine level, the MLF is located just ventral to the fourth ventricle, and lesions of this area can lead to bilateral WEBINO.4

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

In the Teaching Video NeuroImage “Pulsatile proptosis and wall-eyed bilateral internuclear ophthalmoplegia” by Papageorgiou et al.,1 there is an error in the arrow placement in figure 2. Please see the correct version below. The authors regret the error.

Anti-aquaporin-4 antibody-positive dorsal midbrain syndrome

Neuromyelitis optica spectrum disorders (NMOSD) can cause various ocular motor disorders in addition to optic neuritis. Ocular motor findings associated with NMOSD include spontaneous vertical and gaze-evoked nystagmus, wall-eyed bilateral internuclear ophthalmoplegia, and trochlear nerve palsy. The association between dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity has not been reported. Here, we report a patient displaying typical dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity.

A Case Report of WEBINO Syndrome with Convergence Impairment

A 73-year-old man suddenly presented with alternating exotropia, bilateral medial longitudinal fasciculus (MLF) syndrome, and impaired convergence. This symptom has been known as wall-eyed bilateral internuclear ophthalmoplegia (WEBINO syndrome). Diffusion-weighted images on magnetic resonance imaging showed a smalllocalized lesion in his pontine tegmentum. Based on he had some ischemic risk factors; he was diagnosed as having an ischemic stroke and started an antiplatelet therapy. His convergence impairment was improved, but abduction impairment of the right eye remained even 3 months later. The causing lesion of WEBINO syndrome has been reported to be either midbrain or pons mainly due to ischemic strokes or demyelinating diseases. In some cases, this syndrome was accompanied by convergence impairment; however, the pathophysiologic mechanism has not yet been elucidated. The neural circuit of vergence is discharges to the medial rectus subnucleus located in the abducens nerve nucleus. The mesencephalic reticular formation and nucleus reticularis tegmenti pontis (NRTP) are important regions in making signals of the supranuclear pathway, and NRTP impairment can induce the slow and fast vergence impairment. Thus, an involvement of near region of NRTP might be associated with WEBINO syndrome with convergence impairment.

Unusual Clinical Presentations and Mechanism of Bilateral Thalamic Infarcts: A Case Series (P06.250)

Objective: To highlight the breadth of clinical syndromes and mechanisms of artery of Percheron territory strokes. Background Simultaneous bilateral thalamic infarction represents a relatively rare stroke syndrome. The underlying vascular anatomy often suspected in these cases is a variant of the posterior circulation first described by Percheron in 1975, wherein the paramedian thalami as well as rostral midbrain structures are supplied by a single arterial branch of the proximal posterior cerebral artery. The artery of Percheron, though difficult to visualize even with conventional angiography, may be present in as many as one third of patients. Pathophysiologically, these strokes can be attributed to athero- or thromboembolic phenomena, or result from hypoperfusion due to poor cardiac output. The classical clinical presentation is described as a triad of hypersomnolence, amnesia, and vertical gaze paresis. However, in practice, presentations can be far more variable, with a myriad of cognitive and behavioral changes in addition to eye findings, representing a challenge to the clinician in terms of both lesion localization and determination of etiology. Design/Methods: Case series. Data collected from chart review, serial neurologic examinations. Results: We present a series of three cases of bilateral paramedian thalamic infarcts. In one, we report for the first time an association with WEBINO (wall-eyed bilateral internuclear ophthalmoplegia) syndrome, itself a rare phenomenon with fewer than 20 cases reported in the English literature.We describe another patient with unilateral oculomotor nerve palsy, an unusual stroke presentation. Finally, we present a patient with amnesia and behavioral changes whose stroke occurred in the setting of atrial myxoma. Conclusions: Patients with artery of Percheron territory strokes may present with unusual clinical findings that can confound the diagnosis and potentially delay treatment. The protean clinical presentation represents a challenge to the clinician with respect to lesion localization and etiology. Disclosure: Dr. Manchak has nothing to disclose. Dr. Odrzywolski has nothing to disclose. Dr. Kelly has nothing to disclose. Dr. Benesch has nothing to disclose.

WEBINO and the Return of the King's Speech

We present a 69year-old man with hypertension who developed the sudden onset of horizontal binocular diplopia and stuttering of speech. On examination, bilateral exotropia (i.e. ‘wall-eyed’) was observed in the primary position. Attempted horizontal saccades revealed bilateral internuclear ophthalmoplegia; all consistent with the wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome. Convergence, vertical saccades and vestibular ocular reflexes were likewise impaired. Pupillary and levator palpebrae superioris functions were intact. Mild left-sided dysmetria, intention tremor and dysdiadochokinesia were elicited. Conspicuously, further characterization of the patient's history revealed that he had stuttered as a child, but it had resolved in adolescence. Brain MRI revealed an acute infarction of the mesencephalic and upper pontine tegmentum involving the periaqueductal gray region and the medial longitudinal fasciculus bilaterally with greater involvement of the left. Like the WEBINO syndrome, re-emergent developmental stuttering is a rare neurologic phenomenon. To our knowledge, this is the first case report of a mesencephalic and upper pontine infarction causing both syndromes. We discuss the pathobiological underpinnings of the WEBINO syndrome and neurogenic stuttering and in relationship to this unusual case.

Teaching Neuro Images : Isolated pineal hemorrhage

An 80-year-old woman with a history of hypertension and deep vein thrombosis, who was taking warfarin, presented with sudden-onset coma. Examination was remarkable for wall-eyed bilateral internuclear ophthalmoplegia, small minimally reactive pupils, and spontaneous flexor posturing of the arms. These findings localized to the midbrain and suggested the presence of acute hydrocephalus with compression of the periaqueductal …

A small pontine infarct on DWI as a lesion responsible for wall-eyed bilateral internuclear ophthalmoplegia syndrome

A 64-year-old man presented with alternating exotropia and bilateral medial longitudinal fasciculus (MLF) syndrome known as wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome. Diffusion-weighted imaging showed a small localized lesion in the median dorsal pons, and high-resolution T2-weighted imaging revealed slight left deviation of the lesion. A small penetrating artery was assumed to be occluded at the level of the MLF decussation. The median dorsal pons appears to be a location for the lesions causing WEBINO syndrome.