Article

Wall-eyed bilateral inter nuclear ophthalmoplegia with vertical gaze palsy

Abstract and Figures

A 64-year-old woman, diabetic and hypertensive, presented with sudden onset painless diplopia and alteration of consciousness. She was drowsy with neurological examination revealing exotropia of both eyes in primary position with bilateral internuclear ophthalmoplegia on attempted horizontal gaze (Figure 1a). Pupils were normal bilaterally. Convergence, vertical saccades, pursuits and vestibular ocular reflexes were impaired. Cranial magnetic resonance imaging (MRI) showed an acute infarct in the midline of mesencephalic …
Content may be subject to copyright.
Clinical picture
Wall-eyed bilateral inter nuclear ophthalmoplegia with
vertical gaze palsy
A 64-year-old woman, diabetic and hypertensive,
presented with sudden onset painless diplopia and
alteration of consciousness. She was drowsy with
neurological examination revealing exotropia of
both eyes in primary position with bilateral internu-
clear ophthalmoplegia on attempted horizontal
gaze (Figure 1a). Pupils were normal bilaterally.
Convergence, vertical saccades, pursuits and ves-
tibular ocular reFexes were impaired. Cranial mag-
netic resonance imaging (MRI) showed an acute
infarct in the midline of mesencephalic tegmentum
involving the bilateral medial longitudinal fasciculus
(Figure 1b).
Wall-eyed bilateral inter nuclear ophthalmoplegia
(WEBINO) is a rare disorder characterized by
bilateral exotropia on primary gaze, bilateral inter
nuclear ophthalmoplegia (INO) and impaired con-
vergence. It may also be associated with vertical
gaze palsy, up-beat nystagmus and skew deviation.
Common etiology includes inflammatory, toxic, in-
fectious, degenerative, traumatic, postsurgical,
demyelinating and neoplastic conditions, but mid-
brain infarction is the most common reported
etiology.
1
While the pathophysiology of the WEBINO
syndrome remains controversial, the clinical
constellation of Endings are thought to be due to bi-
lateral medial longitudinal fasciculus (MLF) damage
producing bilateral INO, likely in conjunction with
abnormalities of the medial rectus sub nuclei (MRSN)
of the ventral oculomotor nuclear complex culminat-
ing in bilateral exotropia and signifying bilateral con-
vergence failure.
2
As MRSN neurons are scattered
within the MLF at the level of the
ponto-mesencephalic junction, a lesion at this level
could affect both structures, resulting in the WEBINO
syndrome.
1,2
Variable degree of vertical gaze
abnormalities in WEBINO is explained by the fre-
quent concomitant involvement of rostral interstitial
nucleus of MLF or the interstitial nucleus of Cajal in
the midbrain-thalamic region.
2
Photographs and text from: S. Chakravarthi, P. Kesav
and D. Khurana, Department of Neurology, Post
Graduate Institute of Medical Education and
Research, Chandigarh, India.
email: dherajk@yahoo.com
Conflict of interest: None declared.
References
1. Chen CM, Lin SH. Wall-eyed bilateral internuclear ophthal-
moplegia from lesions at different levels in the brainstem.
J Neuroophthalmol 2007; 27:9–15.
2. Beh SC, Frohman EM. WEBINO and the return of the King’s
speech. J Neurol Sci 2012; 315:153–55.
Figure 1. (a) Clinical photograph showing (in a clock-
wise pattern) impaired adduction of left eye with restricted
abduction of right eye on attempted right horizontal gaze,
impaired adduction of right eye with restricted abduction
of left eye on attempted left horizontal gaze. Both upward
and downward gaze paresis is evident on attempted ver-
tical gaze as is bilateral exotropia. (b) Cranial MRI show-
ing acute infarct in midline mesencephalic tegmentum
with hyperintensity on diffusion weighted sequence and
hypointensity on apparent diffusion coefficient sequence.
!The Author 2013. Published by Oxford University Press on behalf of the Association of Physicians.
All rights reserved. For Permissions, please email: journals.permissions@oup.com Page 1 of 1
Q J Med
doi:10.1093/qjmed/hct021
QJM Advance Access published January 25, 2013
by guest on February 1, 2013http://qjmed.oxfordjournals.org/Downloaded from
... Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare disorder associated with lesions in the midbrain tegmentum affecting the MLF bilaterally. 54,55 The distinguishing clinical features of WEBINO are bilateral exotropia on primary gaze and bilateral internuclear. 54,55 Pupillary and levator function, as well as vestibulo-ocular reflex, are often normal. ...
... 54,55 The distinguishing clinical features of WEBINO are bilateral exotropia on primary gaze and bilateral internuclear. 54,55 Pupillary and levator function, as well as vestibulo-ocular reflex, are often normal. 55 Despite inability to adduct past midline in saccade or smooth pursuit and severe limitations in vertical saccade and smooth pursuit, the full range of horizontal and vertical eye movements can be elicited in patients during oculocephalic maneuvering. ...
... 54,55 Pupillary and levator function, as well as vestibulo-ocular reflex, are often normal. 55 Despite inability to adduct past midline in saccade or smooth pursuit and severe limitations in vertical saccade and smooth pursuit, the full range of horizontal and vertical eye movements can be elicited in patients during oculocephalic maneuvering. 54 It is important to distinguish WEBINO from one-and-a-half syndrome, which presents with unilateral horizontal gaze palsy and intranuclear ophthalmoplegia, on the basis of dissociated abducting nystagmus, impaired convergence, and supranuclear vertical gaze palsy. ...
Article
False localizing signs (FLS) and other misleading neurological signs have long been an intractable aspect of neurocritical care. Because they suggest an incorrect location or etiology of the pathological lesion, they have often led to misdiagnosis and mismanagement of the patient. Here, we reviewed the existing literature to provide an updated, comprehensive descriptive review of these difficult to diagnose signs in neurocritical care. For each sign presented, we discuss the non-false localizing presentation of symptoms, the common FLS or misleading presentation, etiology/pathogenesis of the sign, and diagnosis, as well as any other clinically relevant considerations. Within cranial neuropathies, we cover cranial nerves III, IV, V, VI, VII, VIII, as well as multiple cranial nerve involvement of IX, X, and XII. FLS ophthalmologic symptoms indicate diagnostically challenging neurological deficits, and here we discuss downbeat nystagmus, ping-pong-gaze, one-and-a-half syndrome, and wall-eyed bilateral nuclear ophthalmoplegia (WEBINO). Cranial herniation syndromes are integral to any discussion of FLS and here we cover Kernohan's notch phenomenon, pseudo-Dandy Walker malformation, and uncal herniation. FLS in the spinal cord have also been relatively well documented, but in addition to compressive lesions, we also discuss newer findings in radiculopathy and disc herniation. Finally, pulmonary syndromes may sometimes be overlooked in discussions of neurological signs but are critically important to recognize and manage in neurocritical care, and here we discuss Cheyne-Stokes respiration, cluster breathing, central neurogenic hyperventilation, ataxic breathing, Ondine's curse, and hypercapnia. Though some of these signs may be rare, the framework for diagnosing and treating them must continue to evolve with our growing understanding of their etiology and varied presentations.
... Studies have shown that the disease is often associated with brainstem ischemia and hydrocephalus, immunotherapy of tumors, and diseases such as multiple sclerosis. This study summarized 39 case reports, including 42 WEBINO patients (Inocencio and Ballecer, 1985;Lana et al., 1990;Fay and Strominger, 1999;Korkmaz et al., 2002;Ozer et al., 2005;Chen and Lin, 2007;Kim et al., 2008;Matsumoto et al., 2008Matsumoto et al., , 2019Ushio et al., 2008;Jacob et al., 2010;Sierra-Hidalgo et al., 2010;Shinoda et al., 2011;Beh and Frohman, 2012;Jadhav and Prasad, 2012;Sakamoto et al., 2012;Bar et al., 2014;Chakravarthi et al., 2014;de Mora et al., 2014;Mathis et al., 2014;Muralidhar et al., 2014;Nakajima et al., 2014;Toufeeq and Dave, 2014;Agarwal et al., 2015;Ljevak et al., 2015;Man et al., 2015;de Souza et al., 2017;Sajjadi et al., 2017;Zou and Chen, 2017;Keereman et al., 2018;Papageorgiou et al., 2018;Im et al., 2020;Vázquez-Justes et al., 2020;Yazdi et al., 2020;Ansari et al., 2021;Jo et al., 2021;Petrik et al., 2021;Sinha et al., 2021;Wako et al., 2022). The age range of the patients was 12-85 years; 26 were male (61.9%), 15 were female (35.7%), and the gender of one was unknown. ...
Article
Full-text available
Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an uncommon ocular motility disorder that encompasses the following clinical signs: bilateral adduction deficits, bilateral abducting nystagmus, convergence lost, and a large angle exotropia in primary gaze. Here we report a case of a 55-year-old man presenting with atypical WEBINO syndrome with unilateral exotropia. The coverage test was used to record the patient's alternating exotropia. The patient experienced diplopia and ophthalmoplegia and was admitted to our hospital 3 days after the onset of the double vision. Neurologic examination showed left eye exotropia and bilateral internuclear ophthalmoplegia with impaired convergence. Vertical saccades of the left eye were also limited. Consequently, an MRI scan suggested an acute infarction in the left of the pontine tegmentum. The patient was finally diagnosed with pons infarction and was treated with anticoagulation and anti-platelet aggregation therapy.
Article
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.
Preprint
Full-text available
Background: Wall-eyed bilateral internuclear ophthalmoplegia syndrome (WEBINO) is a rare disorder comprising bilateral exotropia, bilateral internuclear ophthalmoplegia, and impaired convergence. It is usually caused by a midbrain lesion. Case presentation :We describe a rare case of WEBINO, with the lesion located in the lower pons, in a 69-year-old man with acute ischemic stroke. Conclusions :Our report alerts physicians to the possibility of bilateral WEBINO due to unilateral infarction.
Article
WEBINO 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 prectal 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.
Article
Full-text available
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. Copyright © 2020 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.
Article
Full-text available
A 45 years old male presented to us with a history of sudden onset painless diplopia for the past two weeks. There was a previous history of cerebrovascular accident (CVA) three weeks back. Examination revealed bilateral internuclear ophthalmoplegia with vertical gaze anomaly and postural instability. A diagnosis of wall eyed bilateral internuclear ophthalmoplegia (WEBINO) with probable progressive supranuclear palsy (PSP) was made. This is a very rare disorder; the ocular symptoms developing one week after a CVA plus PSP like signs and symptoms makes it a rarest of rare case.
Article
Case presentationA 53-year-old patient presented in the neurological emergency department complaining of sudden onset of blurry vision, as well as difficulty opening his eyes. He had no previous medical history, and of significance was his body mass index of 48. Initial neurological examination showed binocular vertical double vision, bilateral ptosis with exotropia of the left eye, bilateral adduction deficit combined with abducting nystagmus (wall-eyed bilateral internuclear ophthalmoplegia, WEBINO) and vertical gaze paralysis. Bedside examination showed normal acuity and no visual field deficit. During the examination, patient quickly deteriorated, becoming comatose, tetraplegic, with unreactive pupils, anisocoria (right pupil wider) and ataxic breathing. After lowering his blood pressure from 260/160 to 160/90 mmHg, his condition improved to somnolence, dysarthria, bilateral ptosis, anisocoria with medium sized fixed pupils (R > L), bilateral adduction deficit with abducting nystag ...
Article
Full-text available
We present a 69 year-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.
Article
Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rarely reported syndrome. There is dispute about whether WEBINO is caused by a pontine or a midbrain lesion and whether the medial rectus subnuclei are implicated. In a study of the clinical and imaging features of four patients with WEBINO, we found that that three of four lesions involved the midbrain but not necessarily the medial rectus subnuclei.