Internuclear Ophthalmoplegia Research Articles

Wall – Eyed monocular Internuclear Ophthalmoplegia (WEMINO): Case report

Internuclear Ophthalmoplegia (INO) is a disorder of ocular motility caused by the damage to the Medial Longitudinal Fasciculus (MLF), which allows coordinated eye movements towards a horizontal direction. Wall-Eyed Monocular Internuclear Ophthalmoplegia (WEMINO) is an uncommon clinical presentation of INO that associates ipsilateral exotropia; the underlying pathophysiology of the syndrome is not yet fully understood.

A Case of Chronic Relapsing Inflammatory Optic Neuropathy

Chronic relapsing inflammatory optic neuropathy (CRION) is a recently described form of recurrent isolated subacute optic neuropathy, with accumulating evidence that it is a nosological distinct entity. The condition is highly responsive to systemic steroid treatment and prone to relapse on steroid withdrawal. Diagnosis and management of this condition is often challenging. This 33-year-old lady with family history of multiple sclerosis (MS), with uniocular visual loss of her right eye since 2 years old without apparent cause, presented with reduction of vision and loss of colour vision in the left eye, associated with painful eye movement. There was internuclear ophthalmoplegia but slit lamp examination were unremarkable. She had no other related sensory or motor symptoms. Magnetic resonance imaging (MRI) did not reveal any features of MS. Aquaporin-4 antibody, anti-MOG and gene testing for Leber’s hereditary optic neuropathy were all negative. Metabolic, infective, and other autoimmune causes were also excluded. Visual evoked potential studies of left eye showed a mild reduction in amplitude with no prolongation of latency. Her multiple optic neuritis recurrences were treated with intravenous steroids followed by tapering regime of oral prednisolone with good effect. Knowledge of this rare condition as part of the differential diagnoses of possible aetiologies of optic neuropathy is important among Ophthalmologists, as prompt diagnosis and steroid treatment helped reduce the associated risk of blindness. Multiple relapses after initial successful treatment of inflammatory optic neuropathy should raise the suspicion of CRION.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S19

Surgical outcome and technical issues in symptomatic brainstem cavernous malformations

IntroductionSafe entry zone for brain stem surgery has already been established. Selective approaches or routes can be used according to anatomical locations. Appropriate surgical corridors for Brainstem Cavernous Malformations (CM) are critical where neuronavigation is not available. Preoperative GCS, comorbid conditions, and patient's age are strong predictors of the outcome of the surgery. ObjectiveThe study aims to assess the surgical outcome of the brainstem CM. MethodA total of eight patients were observed retrospectively from 2013 to 2017 in a private hospital in Dhaka, Bangladesh, of which the male: female ratio was 3:2. Four patients underwent surgeries, and four patients were treated without surgery. Multiple variables, including preoperative GCS, age of the patient, and comorbid conditions, were analyzed. Operative procedures were analyzed in detail. The patient's symptoms and signs, the timing of surgery, and recurrent hemorrhage were observed. ResultsOut of four (4) surgical patients-1 died due to pneumonia after two months of surgery. One patient developed internuclear ophthalmoplegia immediately after surgery and recovered after two months. 2 patients recovered entirely, of which one developed obstructive hydrocephalus and was managed by endoscopic third ventriculostomy. ConclusionSafety corridors for brainstem CM are to be selected according to the location of the lesions. Preoperative patient status, appropriate patient selection, and perioperative management of the patients are few predictors of surgical outcomes. Making a pseudo plane in removing CM with sharp microdissection is technically helpful even in adhering lesions.

Immunotherapy‐responsive brainstem lesion accompanied with anti‐galactocerebroside antibody

AbstractBackgroundBrainstem lesions are a diagnostic challenge and clinicians have to differentiate between many diseases including acute disseminated encephalomyelitis, multiple sclerosis (MS), Bickerstaff brainstem encephalitis (BBE) and brain tumors. The anti‐galactocerebroside (Gal‐C) antibody is an anti‐glycolipid antibody that is often detected in autoimmune peripheral neuropathy. However, anti‐Gal‐C antibody is rarely detected in autoimmune central nervous system diseases. Here, we report the case of an anti‐Gal‐C antibody‐positive patient who had a brainstem lesion that mimicked MS and responded to immunotherapy.Case presentationWe report a 32‐year‐old woman who presented with subacute numbness of the right lower extremities and hemiataxia. Her cerebrospinal fluid examination showed an elevated myelin basic protein level and an oligoclonal band. Brain magnetic resonance imaging showed a T2 high‐intensity lesion from the left brainstem to the cerebellum. She was treated with steroid pulse therapy, but internuclear ophthalmoplegia and left peripheral facial palsy developed. Her brain magnetic resonance imaging showed an enlargement of the brainstem lesion. Therefore, plasma exchange therapy and high‐dose immunoglobulin therapy were administered, and her symptoms eventually ameliorated. First, we diagnosed her with MS and started dimethyl fumarate. After anti‐GM2 and anti‐Gal‐C antibody positivity was revealed, we diagnosed an anti‐Gal‐C antibody‐related brainstem lesion. There have been few reports of anti‐Gal‐C antibody‐positive central nervous system diseases.ConclusionsThe present case highlights that an anti‐Gal‐C positivity can cause brainstem inflammatory lesions, which should be differentiated from acute disseminated encephalomyelitis, Bickerstaff brainstem encephalitis and MS. The patient’s symptoms improved with intense immunotherapy, and antibody measurement led to the discontinuation of unnecessary disease‐modifying drugs.

The phenomenon of Lhermitte

Life in pedants’ corner is (and was BC—before the COVID-19 pandemic) always more rewarding when clinical and scientific morsels from our recent reading can be shared over coffee (black and...

A Case Report of Primary Position Downbeating Nystagmus in Association with a Unilateral Internuclear Ophthalmoplegia (INO) Secondary to a Dorsal Midbrain Infarction. (4937)

A Case Report of Primary Position Downbeating Nystagmus in Association with a Unilateral Internuclear Ophthalmoplegia (INO) Secondary to a Dorsal Midbrain Infarction. (4937)

MRI topography of lesions related to internuclear ophthalmoplegia in patients with multiple sclerosis or ischemic stroke.

Internuclear ophthalmoplegia is a dysfunction of conjugate eye movements, caused by lesions affecting the medial longitudinal fasciculus (MLF). Multiple sclerosis (MS) and ischemic stroke represent the most common pathophysiologies. While magnetic resonance imaging (MRI) allows for localizing lesions affecting the MLF, comprehensive comparative studies exploring potential different spatial characteristics of lesions affecting the MLF are missing until now. We retrospectively investigated MRI examinations of 82 patients (40 patients with MS and 42 patients with ischemic stroke). For lesion localization, the brainstem was segmented into (1) ponto-medullary junction, (2) mid pons, (3) upper pons, and (4) mesencephalon. Corresponding lesions affecting the MLF were observed in 29/40 (72.5%) MS and 38/42 (90.5%) stroke patients. Compared to stroke patients, MS patients had significantly more lesions in multiple locations (P < .001). Stroke patients showed more lesions at the level of the mesencephalon (P < .001), while lesions at the level of the ponto-medullary junction, mid, and upper pons did not statistically differ between the groups. Our results demonstrate that multiple lesions affecting the MLF make inflammatory-demyelination due to MS more likely, while lesion localization at the level of the mesencephalon favors ischemia.

Double vision and facial palsy

A 26-year-old man noticed a facial asymmetry and double vision on awaking and went to the emergency room. There was left eye exotropia at rest and a total horizontal gaze...

A rare case of unilateral Cogan\u2019s anterior internuclear ophthalmoplegia, upgaze palsy and ataxia caused by dorsal tegmentum lesion at pontomesencephalic junction

BackgroundCogan’s anterior internuclear ophthalmoplegia (INO) is characterized by INO with inability to converge and commonly thought to be due to rostral midbrain lesion. A lesion outside midbrain that causes unilateral Cogan’s anterior INO combined with upgaze palsy and ataxia are rarely described.Case presentationA 67-year old male presented with left Cogan’s anterior internuclear ophthalmoplegia (INO), left appendicular ataxia and bilateral upgaze palsy. A Magnetic Resonance Imaging (MRI) and Magnetic Resonance Angiography (MRA) brain showed a left dorsal tegmental infarct at the level of pontomesencephalic junction.ConclusionsThis case highlights the clinical importance of Cogan’s anterior INO in combination with upgaze palsy and ataxia, and report possible site of lesion in patients with such constellation. Clinicians should consider looking for cerebellar signs in cases of Cogan’s anterior INO, apart from just considering localizing the lesion at the midbrain.

A model for interrogating the clinico-radiological paradox in multiple sclerosis: Internuclear ophthalmoplegia.

Background and purposeThe clinico‐radiological paradox in multiple sclerosis (MS) is well recognized, relevant and yet poorly understood. The suitability of an in vivo model for the clinico‐radiological paradox was tested, using internuclear ophthalmoplegia (INO) and the medial longitudinal fasciculus (MLF).MethodsIn this cross‐sectional study lesions of the MLF were rated by an experienced MS neuroradiologist blinded to all other information. The presence of an INO was objectively determined by a validated infrared oculography protocol (DEMoNS). Clinical information, including the National Eye Institute Visual Function Questionnaire, was obtained.ResultsThis study included 202 patients with MS. The clinico‐radiological paradox occurred in 50 patients (25%). This consisted of 45 patients having an INO without an MLF lesion and five patients with an MLF lesion but without an INO. The visual function overall score was related to the presence of an INO (p = 0.016), but not to MLF lesions seen on magnetic resonance imaging (MRI) (p = 0.207). A consensus list of potential causes for the clinico‐radiological paradox was compiled and the MRI images were deposited in a repository.ConclusionThis study provides an objective and quantitative model to investigate the clinico‐radiological paradox. Our data suggest that pathology of the MLF is more frequently detected and more clinically relevant by infrared oculography than by MLF lesion rating on MRI.

Central nystagmus in progressive supranuclear palsy: A neglected clinical feature?

BackgroundProgressive supranuclear palsy (PSP) features parkinsonism characterized by early postural instability, falls and prominent eye movement abnormalities that consist of saccadic slowing, followed by gaze limitation. Nystagmus is not considered typical for PSP, being more commonly associated with multiple system atrophy. ObjectivesTo describe the prevalence and phenomenology of nystagmus in patients with PSP. Methods42 patients with probable PSP underwent detailed clinical eye movement examination. Patients with nystagmus performed video-nystagmography. T-test, Chi-Square test and Wilcoxon signed-rank test were used to test differences in demographic data, disease duration and PSP subtype between patients with and without nystagmus, and for analysis of video-nystagmographic data. ResultsAmong 42 patients with PSP, we identified 15 patients (35,7%) with gaze-evoked nystagmus, predominantly horizontal. Clinically, 10/15 patients had symmetrical or asymmetrical gaze - evoked nystagmus (Type 1), while 5/15 patients had dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia (Type 2). Nystagmus and eye movement abnormalities were further characterized by video-nystagmography. There was no significant difference in age, disease duration or PSP subtypes between patients with and without nystagmus. ConclusionCentral nystagmus is present in more than a third of patients with progressive supranuclear palsy. It may present as symmetrical or asymmetrical gaze-evoked nystagmus or as dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia and probably arises from neurodegeneration of the neural integrator. Nystagmus in PSP has been a hitherto under-described feature and its presence should not deter clinicians from a diagnosis of PSP.

Bickerstaff's brainstem encephalitis with monospecific anti-GT1a antibody: A subtype of Guillain–Barre syndrome

Bickerstaff's brainstem encephalitis is an uncommon post-infectious causing autoimmune neurological disease, which is usually associated with the GQ1b antibody. We described a young man with recent upper respiratory tract infection, who presented with sudden onset of left eye ptosis with internuclear ophthalmoplegia, staccato speech with left-sided hemiplegia but with normal deep tendon reflexes. The cerebral imaging was normal and cerebrospinal fluid analysis produced no cyto-albuminologic dissociation, negative result of tuberculosis culture and virus PCR. He was tested positive for serum GT1a IgG antibody. We discussed this case as the neurological presentation was severe, in the presence of GT1a IgG antibody. La encefalitis troncoencefálica de Bickerstaff es una enfermedad neurológica postinfecciosa autoinmune rara, que está normalmente asociada al anticuerpo GQ1b. Describimos el caso de un varón joven con infección reciente del tracto respiratorio superior, que acudió con aparición súbita de ptosis en el ojo izquierdo con oftalmoplejía internuclear, habla entrecortada con hemiplejía izquierda, aunque con reflejos tendinosos profundos normales. La imagen cerebral fue normal, y el análisis del líquido cefalorraquídeo no produjo disociación cito-albuminológica, siendo negativo el resultado del cultivo para la detección de tuberculosis y la PCR para la presencia de virus. El resultado de anticuerpos séricos GT1a IgG fue positivo. Debatimos este caso, dado que la presentación neurológica fue grave, en presencia de anticuerpos GT1a IgG.

An Anatomic, Imaging, and Clinical Review of the Medial Longitudinal Fasciculus

The medial longitudinal fasciculus (MLF) is a paired, highly specialized, and heavily myelinated nerve bundle responsible for extraocular muscle movements, including the oculomotor reflex, saccadic eye movements an smooth pursuit, and the vestibular ocular reflex. Clinically, lesions of the MLF are classically associated with internuclear ophthalmoplegia. However, clinical manifestations of a lesion in the MLF may be more complex and variable. We provide an overview of the neuroanatomy, neurologic manifestations, and correlative examples of the imaging findings on brain MRI of MLF lesions to provide the clinician and radiologist with a more comprehensive understanding of the MLF and potential clinical manifestations for an MLF lesion.

Atypical presentations of idiopathic intracranial hypertension.

Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.

Controversies in Ocular Myasthenia Gravis.

Myasthenia gravis (MG) with symptoms limited to eye muscles [ocular MG (OMG)] is a rare disease. OMG incidence varies according to ethnicity and age of onset. In recent years, both an increase in incidence rate, particularly in the elderly, and a lower risk for secondary generalization may have contributed to the growing disease prevalence in Western countries. OMG should be considered in patients with painless ptosis and extrinsic ophthalmoparesis. Though asymmetric muscle involvement and symptom fluctuations are typical, in some cases, OMG can mimic isolated cranial nerve paresis, internuclear ophthalmoplegia, and conjugate gaze palsy. Diagnostic confirmation can be challenging in patients negative for anti-acetylcholine receptor and anti-muscle-specific tyrosine kinase antibodies on standard radioimmunoassay. Early treatment is aimed at relieving symptoms and at preventing disease progression to generalized MG. Despite the absence of high-level evidence, there is general agreement on the efficacy of steroids at low to moderate dosage; immunosuppressants are considered when steroid high maintenance doses are required. The role of thymectomy in non-thymoma patients is controversial. Prolonged exposure to immunosuppressive therapy has a negative impact on the health-related quality of life in a proportion of these patients. OMG is currently excluded from most of the treatments recently developed in generalized MG.

Neuro-ophthalmic Complications of Immune Checkpoint Inhibitors: A Systematic Review.

ObjectiveImmune checkpoint inhibitors (ICIs) are novel cancer therapies that may be associated with immune-related adverse events (IRAEs) and come to the attention of neuro-ophthalmologists. This systematic review aims to synthesize the reported ICI-associated IRAEs relevant to neuro-ophthalmologists to help in the diagnosis and management of these conditions.MethodsA systematic review of the literature indexed by MEDLINE, Embase, CENTRAL, and Web of Science databases was searched from inception to May 2020. Reporting followed the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines. Primary studies on ICIs and neuro-ophthalmic complications were included. Outcomes included number of cases and incidence of neuro-ophthalmic IRAEs.ResultsNeuro-ophthalmic complications of ICIs occurred in 0.46% of patients undergoing ICI and may affect the afferent and efferent visual systems. Afferent complications include optic neuritis (12.8%), neuroretinitis (0.9%), and giant cell arteritis (3.7%). Efferent complications include myasthenia gravis (MG) (45.0%), thyroid-like eye disease (11.9%), orbital myositis (13.8%), general myositis with ptosis (7.3%), internuclear ophthalmoplegia (0.9%), opsoclonus-myoclonus-ataxia syndrome (0.9%), and oculomotor nerve palsy (0.9%). Pembrolizumab was the most common causative agent for neuro-ophthalmic complications (32.1%). Mortality was highest for MG (19.8%). Most patients (79.8%) experienced improvement or complete resolution of neuro-ophthalmic symptoms due to cessation of ICI and immunosuppression with systemic corticosteroids.ConclusionWhile incidence of neuro-ophthalmic IRAEs is low, clinicians involved in the care of cancer patients must be aware of their presentation to facilitate prompt recognition and management. Collaboration between oncology and neuro-ophthalmology teams is required to effectively manage patients and reduce morbidity and mortality.

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).

Ophthalmic manifestations of myelin oligodendrocyte glycoprotein-IgG-associated disorder other than optic neuritis: a systematic review

Background/AimsOptic neuritis (ON) is the primary ophthalmic manifestation of myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD), but numerous reports have expanded the visual manifestations of this condition. The goal of this study...

Trochlear nerve palsy and contralateral internuclear ophthalmoplegia: an unusual crossed syndrome

Trochlear nerve palsies and internuclear ophthalmoplegias are well documented neuro‐ophthalmological conditions.2017 However, cases of both of these conditions occurring together in the same patien...

The Medial Longitudinal Fasciculus and Internuclear Opthalmoparesis: There's More Than Meets the Eye.

Background and purposeThe classic sign of a lesion in the medial longitudinal fasciculus is internuclear opthalmoplegia. However, clinical presentation may vary depending on the type of pathology and the lesion location. The purpose of this study was to identify and classify the different lesions of the medial longitudinal fasciculus on MRI and review their clinical presentations. We also offer an overview of the pertinent imaging anatomy of the medial longitudinal fasciculus.Materials and methodsPatients with an abnormality affecting the medial longitudinal fasciculus were identified retrospectively using the keyword ‘medial longitudinal fasciculus’ included in radiology reports between 2010 and 2018 using the Nuance mPower software (Nuance Communications, Burlington, MA). The brain MRI examinations of these patients were reviewed by two neuroradiolgists. Detailed description of lesion location within the medial longitudinal fasciculus, pathology type, additional lesions, and clinical presentations were recorded along with pertinent demographic information.ResultsFive men and four women were identified with lesions in the medial longitudinal fasciculus on brain MRI. Five patients demonstrated demyelination in the medial longitudinal fasiculus and four patients demonstrated findings of an acute cerebrovascular accident. Two-thirds of medial longitudinal fasiculus lesions were located in the upper, mid, or lower pons with the remaining located in the midbrain. Of the patients presenting with a cerebrovascular accident, there was little to no additional evidence of acute stroke elsewhere in the brain. All patients were clinically symptomatic with 89% of patients demonstrating extraocular muscle dysfunction at presentation and 78% of patients experiencing dizziness. Additional symptoms included headache, weakness, and gait instability.Conclusions Lesions involving the medial longitudinal fasiculus may not always present with the classic sign of internuclear opthalmoplegia. Variations in lesion location may result in diplopia rather than internuclear opthalmoplegia, and additional brain lesions may produce clinical symptoms that confound extraocular muscle dysfunction. Lesions affecting the often-overlooked vestibular and otolithic reflexes, which run in the medial longitudinal fasiculus, may result in dizziness/weakness and mask the classic internuclear opthalmoplegia symptoms. The radiologist should carefully inspect the medial longitudinal fasiculus in all patients regardless of the supportive clinical history of extraocular muscle dysfunction since symptoms may be more general than classically described.