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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.

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.

Introduction Medial longitudinal fasciculus infarction is rare in clinical practice and generally accompanied by brain tissue damage around the medial longitudinal fasciculus. Isolated medial longitudinal fasciculus midbrain infarction was seldom reported. Case report An 81-year-old man with hypertension was admitted to our hospital because of sudden onset diplopia. A neurological examination revealed right adduction paresis without abducting nystagmus in the left eye, whereas the convergence reflex was normal. Diffusion-weighted magnetic resonance imaging demonstrated a small acute lacunar medial longitudinal fasciculus infarction in the right midbrain at the level of the inferior colliculus. Diffusion-tensor imaging showed a reduction of the right medial longitudinal fasciculus. Medial longitudinal fasciculus infarction is rare and occurs most commonly in the pons. The authors report on a case of isolated medial longitudinal fasciculus infarction that was diagnosed because of sudden diplopia and manifested as simple internal rectus paralysis with no abducting nystagmus on the contralateral side of the lesion. Conclusion Isolated midbrain-medial longitudinal fasciculus infarction without contralateral abducting nystagmus is a rare occurrence. It can be differentiated from partial oculomotor palsy by assessing the convergence reflex, primary gaze, and diffusion-tensor imaging.

Parinaud’s syndrome involves dysfunction of the structures of the dorsal midbrain. We investigated the pathophysiology related to the signs and symptoms to better understand the symptoms of Parinaud’s syndrome: diplopia, blurred vision, visual field defects, ptosis, squint, and ataxia, and Parinaud’s main signs of upward gaze paralysis, upper eyelid retraction, convergence retraction nystagmus (CRN), and pseudo-Argyll Robertson pupils. In upward gaze palsy, three structures are disrupted: the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), interstitial nucleus of Cajal (iNC), and the posterior commissure. In CRN, there is a continuous discharge of the medial rectus muscle because of the lack of inhibition of supranuclear fibers. In Collier’s sign, the posterior commissure and the iNC are mainly involved. In the vicinity of the iNC, there are two essential groups of cells, the M-group cells and central caudal nuclear (CCN) group cells, which are important for vertical gaze, and eyelid control. Overstimulation of the M group of cells and increased firing rate of the CCN group causing eyelid retraction. External compression of the posterior commissure, and pretectal area causes pseudo-Argyll Robertson pupils. Pseudo-Argyll Robertson pupils constrict to accommodation and have a slight response to light (miosis) as opposed to Argyll Robertson pupils were there is no response to a light stimulus. In Parinaud’s syndrome patients conserve a slight response to light because an additional pathway to a pupillary light response that involves attention to a conscious bright/dark stimulus. Diplopia is mainly due to involvement of the trochlear nerve (IVth cranial nerve. Blurry vision is related to accommodation problems, while the visual field defects are a consequence of chronic papilledema that causes optic neuropathy. Ptosis in Parinaud’s syndrome is caused by damage to the oculomotor nerve, mainly the levator palpebrae portion. We did not find a reasonable explanation for squint. Finally, ataxia is caused by compression of the superior cerebellar peduncle.

There are no literature data on brainstem arachnoid cysts in humans.To describe the clinical case of brainstem (pontomesencephalic) arachnoid cyst and to analyze classification, pathogenesis, differential diagnosis and treatment of this pathology considering literature data and own experience.A 29-year-old patient with pontomesencephalic arachnoid cyst is reported. The disease manifested in childhood with a headache aggravated by bending and pushing. Later, syncope, vegetative-visceral paroxysms, mild oculomotor disturbances, transient paresthesia and numbness of the left half of the face occurred. Headaches became significantly more severe and resulted nausea and vomiting. Magnetic resonance imaging (MRI) revealed a two-chambered arachnoid cyst. A smaller chamber was localized in interpeduncular cistern, a larger one - in brainstem.Differential diagnosis included cystic glioma and Virchow-Robin space enlargement. Fenestration of the cyst wall within interpeduncular cistern was performed via right-sided pterional approach. The diagnosis was verified by histological examination. The follow-up period was 14 months. We observed postoperative cyst reduction confirmed by MR data and regression of all symptoms except for minimal signs of medial longitudinal fasciculus dysfunction.Correct surgical approach for brainstem arachnoid cyst complicated by progressive neurological deterioration is confirmed by postoperative regression of cyst and symptoms.Арахноидальные кисты ствола мозга у человека не описаны.Представить описание клинического случая внутристволовой (понтомезенцефальной) арахноидальной кисты и обсудить вопросы патогенеза, дифференциальной диагностики и лечения данной патологии.Приводится описание клинического случая пациентки 29 лет с арахноидальной кистой, расположенной в толще ствола головного мозга в области перехода среднего мозга в мост. Заболевание проявилось в детстве головной болью, усиливающейся при наклонах и натуживании, затем присоединились обморочные состояния, вегетовисцеральные пароксизмы, легкие глазодвигательные нарушения, преходящие парестезии и онемение левой половины лица; со временем головная боль значительно усилилась, стала сопровождаться тошнотой и рвотой. При магнитно-резонансной томографии (МРТ) выявлена двухкамерная арахноидальная киста с меньшей по размеру камерой в межножковой цистерне и большей — в толще ствола головного мозга.Дифференциальная диагностика проведена с кистозной глиомой и расширением пространств Вирхова—Робина. Операция заключалась в фенестрации стенки кисты, выходившей в межножковую цистерну, и выполнена из правостороннего птерионального доступа. Диагноз верифицирован при гистологическом исследовании биоптата. Катамнез прослежен на протяжении 14 мес. После операции отмечено значительное уменьшение размеров кисты по данным МРТ, регресс всех симптомов, кроме минимальных признаков дисфункции медиального продольного пучка.Правильность выбора хирургической тактики при арахноидальной кисте ствола головного мозга, вызывающей прогредиентное нарастание клинической симптоматики, подтверждается регрессом кисты и симптомов после операции.

Martin Lubow (1931–2015)1 is credited with coining the term ‘wall-eyed bilateral internuclear ophthalmoplegia’ (WEBINO).2 Most readers will be familiar with internuclear ophthalmoplegia (INO) and the fact that it can occur bilaterally. So why is WEBINO, as in the case presented by Chen3 in this issue of Practical Neurology , special? INO is caused by damage to the medial longitudinal fasciculus (MLF). This interrupts the nerve fibres connecting the sixth and third nerve nuclei, which facilitate conjugate horizontal eye movement. A unilateral INO causes slowing, or absence, of adduction of the ipsilateral eye and, usually, gaze-evoked nystagmus of the abducting, contralateral eye during attempted contralateral gaze. Interestingly, adduction is not infrequently preserved in the context of convergence.4 Because these are not the only fibres that travel in the MLF, there may be additional abnormalities due to interruption of the vestibular/otolithic pathways, such as skew deviation or vertical nystagmus. If the INO is bilateral, vertical nystagmus is very common and the vertical vestibulo-ocular reflex is demonstrably impaired.5 INO can result from many different underlying pathologies,5 6 though demyelinating disease is the most common cause of bilateral INO and stroke is the most common cause of unilateral INO.6 In spite of the impaired adduction, many patients with an INO are orthophoric in primary position, that is, their eyes point straight ahead. Some exotropia occurs in up to 60% of cases of unilateral INO4 but diplopia is often absent or surprisingly mild.7 A few patients are esotropic (convergent), particularly early on, possibly due to excessive convergence tone. The number of patients who are markedly exotropic (divergent) is surprisingly small, but many of these demonstrate large, wide-angle, exotropia. Bilateral INOs that show this divergence are labelled WEBINO. Of note, …

The aim of this study is to investigate the trajectory of medial longitudinal fasciculus (MLF) and explore its anatomical relationship with the oculomotor nerve using tractography technique. The MLF and oculomotor nerve were reconstructed at the same time with preset three region of interests (ROIs): one set at the area of rostral midbrain, one placed on the MLF area at the upper pons, and one placed at the cisternal part of the oculomotor nerve. This mapping protocol was tested in an HCP‐1065 template, 35 health subjects from Massachusetts General Hospital (MGH), 20 healthy adults and 6 brainstem cavernous malformation (BCM) patients with generalized q‐sampling imaging (GQI)‐based tractography. Finally, the 200 μm brainstem template from Center for In Vivo Microscopy, Duke University (Duke CIVM), was used to validate the trajectory of reconstructed MLF. The MLF and oculomotor nerve were reconstructed in the HCP‐1065 template, 35 MGH health subjects, 20 healthy adults and 6 BCM patients. The MLF was in conjunction with the ipsilateral mesencephalic part of the oculomotor nerve. The displacement of MLF was identified in all BCM patients. Decreased QA, RDI and FA were found in the MLF of lesion side, indicating axonal loss and/or edema of displaced MLF. The reconstructed MLF in Duke CIVM brainstem 200 μm template corresponded well with histological anatomy. The MLF and oculomotor nerve were visualized accurately with our protocol using GQI‐based fiber tracking. This GQI‐based tractography is an important tool in the reconstruction and evaluation of MLF., The mapping protocol of medial longitudinal fasciculus using the generalized q‐sampling imaging based tractography.

Stroke can present with unique neurologic symptoms, which can be used to help determine the location of the stroke. Internuclear ophthalmoparesis (INO), also known as internuclearophthalmoplegia, is a distinct gaze abnormality with impaired horizontal eye movements with compromised adduction of the affected eye, and abduction nystagmus of the contralateral eye. Infarcts involving the medial longitudinal fasciculus in either the pons or midbrain can result in INO. We present two cases of midbrain ischemic stroke, which presented as isolated INO. The midbrain has a unique and intricate vascular supply including branches from the basilar, superior cerebellar, posterior cerebral, posterior communicating, anterior choroidal, and posterior choroidal arteries, which is reviewed. Infarcts involving the paramedian midbrain, which is supplied by short circumferential arteries and penetrating branches arising from the posterior cerebral artery and superior cerebellar artery, can result in INO.

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome has previously been reported in only 2 patients with progressive supranuclear palsy (PSP). Herein, we report a third case of WEBINO syndrome with PSP. The patient was an 81-year-old man who had experienced gradually increasing gait disturbance and occasional falls since the age of 78 years. At 80 years of age, he presented with cognitive impairment, parkinsonism, and oculomotor abnormalities. The oculomotor abnormalities consisted of vertical gaze palsy and loss of eye convergence. Brain magnetic resonance imaging demonstrated marked atrophy of the midbrain. He was diagnosed with PSP. At the age of 81 years, he presented with alternating extropia in his forward gaze and adduction paresis and outward nystagmus of the abducted eye in his horizontal gaze, both of which were compatible with WEBINO syndrome. Previously, we reported the first case of PSP with WEBINO syndrome, and another group recently reported a second case. In light of the previous cases and the present case, WEBINO syndrome in PSP should not be considered extremely rare. Furthermore, WEBINO syndrome has not been reported in other neurodegenerative disorders, which suggests that it might be a useful and specific diagnostic finding in PSP.

The one and a half Fisher syndrome is produced by a unilateral lesion in the dorsal and inferior part of the pons located specifically in the pontine tegmentum. This is an area that contains the medial longitudinal fasciculus (LMF), the paramedian pontine reticular formation (PPRF) and the nucleus of the sixth cranial nerve, responsible for the horizontal movements of the gaze. Clinically it manifests with conjugate gaze palsy with internuclear ophthalmoplegia. The case is reported on a 60 year-old patient, who presented with binocular diplopia, ophthalmoplegia and paralysis of the gaze conjugated to the left. Imaging studies were performed that showed a hemi-protuberant hematoma secondary to a cavernoma. As expected, he presented with several episodes of re-bleeding with clinical worsening: headache, diplopia, tetraparesis, and hypoaesthesia in the right side of the face, as such that the microsurgical exeresis of the protuberant cavernoma was considered.

Impaired bilateral conjugate horizontal saccades can occur from bilateral abducens nuclear lesions,1 as seen in our patient with multiple sclerosis (Video 1 and Figure). Each abducens nucleus is a horizontal gaze center and innervates the ipsilateral lateral rectus through the abducens fascicle and the contralateral medial rectus via the medial longitudinal fasciculus (Figure). Thus, bilateral lesions of the abducens nuclei cause complete horizontal gaze paresis.

Background and purpose The 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). Methods In 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. Results This 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. Conclusion This 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., This study provides an objective and quantitative model to investigate the clinico‐radiological paradox, by comparing the presence of internuclear ophthalmoplegia and medial longitudinal fasciculus lesions on magnetic resonance imaging.