Bilateral Internuclear Ophthalmoplegia Research Articles

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.
 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.
 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.
 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.

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.

Wernekink commissure syndrome secondary to a rare ‘V’-shaped pure midbrain infarction: a case report and review of the literature

Purpose: Wernekink commissure syndrome is a typical but extremely rare mesencephalic syndrome, and generally presents with bilateral cerebellar dysfunction, diverse oculomotor disorders and occasionally delayed-onset palatal myoclonus or tremor. However, it has been reported infrequently.Methods: We report a case of a 55-year-old man who suffered an acute paramedian midbrain infarction presenting with bilateral cerebellar ataxia, bilateral anterior internuclear ophthalmoplegia and unilateral pseudoabducens palsy, which is confirmed as Wernekink commissure syndrome by magnetic resonance imaging (MRI). We summarized the clinical data of this entity and performed a literature review of 20 previous reports of patients with this syndrome.Results: In combination with previous reports, we found that the most common symptom was bilateral cerebellar ataxia (100%) and other frequent symptoms were oculomotor disorders (81%), delayed palatal myoclonus or tremor (33%) and consciousness dysfunction (33%). Lesions on brain MRI of all patients affected the area of caudal paramedian midbrain (CPM).Conclusion: Bilateral cerebellar ataxia and lesions involved in the area of CPM on MRI are the major features of Wernekink commissure syndrome and should be the necessary conditions of diagnostic criteria. The simultaneous occurrence of bilateral cerebellar ataxia and oculomotor disorders is significant for localization diagnosis. Consciousness dysfunction is also a relatively frequent symptom of this syndrome. Moreover, pseudoabducens palsy might be attributed to a midbrain lesion. Clinicians should be familiar with and early to recognize this unique syndrome to avoid misdiagnosis.

38. Bilateral internuclear ophthalmoplegia and third nerve palsy in giant cell arteritis

IntroductionWe present a case of a gentleman with atypical headache symptoms clinically diagnosed as giant cell arteritis (GCA) and initiated on high dose oral steroids. He subsequently developed progressive neurological deficit including bilateral internuclear ophthalmoplegia (INO), as well as third cranial nerve involvement despite above treatment. He received IV methylprednisolone and demonstrated clinical response, temporal artery biopsy confirmed histological evidence of GCA. The nature of his presentation was atypical of cranial giant cell arteritis. Few reported cases describe INO in the context of GCA, with bilateral manifestation being rarer, especially with additional third cranial nerve involvement.Case descriptionA 66-year-old gentleman presented with a 7-day history of bilateral temporal headaches. He noted prominence of both temporal arteries with mild tenderness during this period. He denied any visual changes, PMR symptoms, and jaw claudication or weight loss. He had been experiencing generalised fatigue and myalgia for the preceding 4 months.He was noted to have raised inflammatory markers (CRP 194, ESR 74) and due to the non-specific nature of headache, concern was for possible meningitis. CT head scan was unremarkable and lumbar puncture and CT-CAP did not demonstrate any abnormality including evidence of infection.He was assessed by the rheumatology team and a clinical diagnosis of GCA was made. He was initiated on prednisolone 60mg daily, and described clinical improvement of headaches over the subsequent day. Inflammatory markers also initially responded to treatment.After 2 days of oral therapy, he developed double vision as well as intermittent headache. CRP remained static at 40. He was seen with the neurology team and was found to have divergent gaze of the left eye with ptosis. Eye movements demonstrated bilateral internuclear opthalmoplegia with involvement of the left third cranial nerve. MRI head scan demonstrated small vessel ischaemic changes but no obvious focal pathology. Due to new visual involvement despite prednisolone 60mg, IV methylprednisolone was administered for a period of 3 days. Ophthalmological symptoms did not progress and CRP reduced to 8. Temporal artery biopsy reported findings consistent with GCA.He was re-established on prednisolone 60mg however intermittent headache recurred and CRP gradually increased. Decision was made to increase prednisolone to 80mg (1mg/kg). At this dose headache resolved and CRP decreased to normal range. Methotrexate was introduced at 20mg weekly in order to facilitate with prednisolone weaning.Within 3 weeks of initial IV methylprednisolone administration, ophthalmological symptoms slowly improved to complete resolution.DiscussionTypical manifestion of cranial GCA consists of unilateral temporal headache. Patients can however exhibit other symptoms including bilateral involvement and features of systemic inflammation which may be non-specific.Cases of neurological involvement have also been described, with diplopia and cranial nerve involvement being widely reported. Few case reports have described bilateral internuclear ophthalmoplegia which is syndrome involving the medial longitudinal fasciculus of the brainstem. This is usually associated with multiple sclerosis however any pathology of this anatomical region can result in this clinical picture. This gentleman additionally demonstrated features of third nerve palsy of the left eye which would be in keeping with a more typical cranial nerve involvement in GCA. The hypothesis for his clinical picture would be of reversible localised ischaemia to the brain stem secondary to active inflammation of the supplying vessel. MRI imaging did not identify any focal pathology and as mentioned previously, following treatment, his clinical findings fully resolved in a gradual manner over a period of weeks.In regards to the management of his case, following exclusion of infection and in the absence of visual findings on presentation, he was started on prednisolone 60mg daily (40-60mg dose suggested within current BSR guidelines). He described improvement of his headache and CRP was seen to improve initially. Despite this treatment, he developed features of INO and third nerve palsy as described. Implementation of IV methylprednisolone therapy prevented further progression of his symptoms and subsequent resolution of raised CRP. However restarting prednisolone at 60mg after this appeared to demonstrate incomplete control of his condition, thus it was increased to 80mg. Decision to introduce methotrexate at an early stage was made in anticipation of likely difficulties in weaning prednisolone.He remains under close follow-up to monitor prednisolone weaning. He currently has not had any recurrence of his symptoms.Key learning pointsGCA can present in an atypical manner and should remain a differential in cases of unexplained headache with associated inflammation. A combination of INO and third nerve palsy is an atypical manifestation of this condition. Due to the nature of his presentation, our gentleman was seen by various specialties including the acute-medical team, rheumatologists and neurologists. Fortunately, a probable diagnosis of GCA was made early and appropriate treatment was initiated. However as this case demonstrates, response to treatment can vary and such adjustments were made to accommodate for this, potentially preventing long term disability.Conflicts of interestThe authors have declared no conflicts of interest.

Bilateral Internuclear Ophthalmoplegia in a Young Woman with Vertebral Artery Dissection.

BackgroundInternuclear ophthalmoplegia (INO) is an eye movement disorder caused by a lesion in the medial longitudinal fasciculus (MLF) located in the midbrain. Adduction paralysis of both eyes and bilateral abduction nystagmus are the main features of INO[1].Case presentationA 29-year-old Hispanic woman was admitted to the emergency department complaining of an intense holocranial headache lasting 9 days, associated with nausea and vomiting. She was discharged home with resolution of the headache but persistence of symptoms. However, she subsequently developed horizontal diplopia and gait abnormalities. She was readmitted to hospital because of anomalous eye movements and conjugate gaze palsy, manifested as bilateral INO. Magnetic resonance angiography (MRA) findings were consistent with dissection of the left V4 vertebral artery with multiple brain infarcts in the superior cerebellar artery territory, comprising both MLF tracts.ConclusionsIn young adults, bilateral INO is normally caused by demyelinating disease. In other patients, common causes include trauma, infections and autoimmune diseases with neurological symptoms. Vascular disease is implicated in over a third of cases.LEARNING POINTSA vascular aetiology should be suspected when internuclear ophthalmoplegia (INO) presents with an intense headache.Almost a third of patients with bilateral INO have an identified vascular cause.Magnetic resonance imaging is the gold standard for investigating INO

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).

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.

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.

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

Head Injury: A Rare Cause of Bilateral Internuclear Ophthalmoplegia

AbstractRare case of bilateral internuclear ophthalmoplegia (INO) following head injury in 25-year-old male is being reported. The site of the lesion in the mid-brain as detected in the magnetic resonance imaging (MRI) correlates well with the clinical presentation. The patient recovered on conservative treatment with a course of dexamethasone (for brainstem contusion).

Preferential Impairment of the Contralesional Posterior Semicircular Canal in Internuclear Ophthalmoplegia.

The vertical vestibulo-ocular reflex (VOR) may be impaired in internuclear ophthalmoplegia (INO) as the medial longitudinal fasciculus (MLF) conveys VOR-signals from the vertical semicircular canals. It has been proposed that signals from the contralesional posterior semicircular canal (PSC) are exclusively transmitted through the MLF, while for the contralesional anterior canal other pathways exist. Here, we aimed to characterize dysfunction in individual canals in INO-patients using the video-head-impulse test (vHIT) and to test the hypothesis of dissociated vertical canal impairment in INO. Video-head-impulse testing and magnetic resonance imaging were obtained in 21 consecutive patients with unilateral (n = 16) or bilateral (n = 5) INO and 42 controls. VOR-gains and compensatory catch-up saccades were analyzed and the overall function (normal vs. impaired) of each semicircular canal was rated. In unilateral INO, largest VOR-gain reductions were noted in the contralesional PSC (0.55 ± 0.11 vs. 0.89 ± 0.08, p < 0.001), while in bilateral INO both posterior (0.43 ± 0.11 vs. 0.89 ± 0.08, p < 0.001) and anterior (0.58 ± 0.19 vs. 0.88 ± 0.09, p < 0.001) canals showed marked drops. Small, but significant VOR-gain reductions were also found in the other canals in unilateral and bilateral INO-patients. Impairment of overall canal function was restricted to the contralesional posterior canal in 60% of unilateral INO-patients, while isolated involvement of the posterior canal was rare in bilateral INO-patients (20%). Reviewers correctly identified the INO-pattern in 15/21 (71%) patients and in all controls (sensitivity = 84.2% [95%-CI = 0.59.5-95.8]; specificity = 95.5% [95%-CI = 83.3-99.2]). Using a vHIT based overall rating of canal function, the correct INO-pattern could be identified with high accuracy. The predominant and often selective impairment of the contralesional posterior canal in unilateral INO further supports the role of the MLF in transmitting posterior canal signals. In patients with acute dizziness and abnormal vHIT-results, central pathologies such as INO should be considered as well, especially when the posterior canal is involved.

Vestibulo-ocular reflex deficits with medial longitudinal fasciculus lesions

The medial longitudinal fasciculus (MLF) is the final common pathway for all conjugate adducting horizontal eye movements, as well as for the vertical-torsional vestibulo-ocular reflex (VOR). MLF lesion causes adduction paresis of ipsilesional (adducting) eye with dissociated nystagmus of contralesional (abducting) eye-the well-known clinical syndrome of internuclear ophthalmoplegia (INO). We measured the VOR stimulation and also any catch-up saccades, from individual semicircular canal (SCC) evoked by the head impulse test (HIT), using head and binocular 3-dimensional scleral search coils in 27 multiple sclerosis (MS) patients, 8 with unilateral, 19 with bilateral INO. In unilateral INO, VOR gain (normal >0.90) from ipsilesional lateral SCC stimulation was 0.48 for the adducting eye and 0.81 for the abducting eye; 0.61 from contralesional anterior SCC stimulation and only 0.29 from contralesional posterior SCC stimulation. In bilateral INO, there were VOR gain deficits from all six SCCs: lateral SCC gains were asymmetrically reduced to 0.45 in the adducting eye and 0.66 in the abducting eye; anterior SCC gain was 0.48 and posterior SCC gain was only 0.19. Horizontal VOR versional dysconjugacy between adducting and abducting eyes at 0.66 was less severe than horizontal catch-up saccade versional dysconjugacy (0.44); normal >0.80. Unexpected partial preservation of horizontal VOR with greater catch-up saccade impairment from the adducting than abducting eye suggests that the ascending tract of Deiters(ATD), an extra-MLF pathway, also mediates the horizontal VOR, but not adducting horizontal saccades. Vertical VOR deficits will produce vertical oscillopsia with any vertical head movement and measurement of the vertical VOR could help with the diagnosis and quantitative evaluation of MLF lesions in suspected MS. Horizontal VOR deficits and catch-up saccade versional dysconjugacy in INO will cause gaze instability and horizontal oscillopsia during active horizontal head movements.

Neurological Complications Associated With Anti–Programmed Death 1 (PD-1) Antibodies

Neurological complications are an increasingly recognized consequence of the use of anti-programmed death 1 (PD-1) antibodies in the treatment of solid-organ tumors, with an estimated frequency of 4.2%. To date, the clinical spectrum and optimum treatment approach are not established. To investigate the frequency, clinical spectrum, and optimum treatment approach to neurological complications associated with anti-PD-1 therapy. This single-center, retrospective cohort study was conducted from either September or December 2014 (the approval dates of the study drugs by the US Food and Drug Administration) to May 19, 2016. All patients receiving anti-PD-1 monoclonal antibodies were identified using the Mayo Cancer Pharmacy Database. Patients with development of neurological symptoms within 12 months of anti-PD-1 therapy were included. Patients with neurological complications directly attributable to metastatic disease or other concurrent cancer-related treatments were excluded. Clinical and pathological characteristics, time to development of neurological symptoms, and modified Rankin Scale (mRS) score. Among 347 patients treated with anti-PD1 monoclonal antibodies (pembrolizumab or nivolumab), 10 (2.9%) developed subacute onset of neurological complications. Seven patients were receiving pembrolizumab, and 3 patients were receiving nivolumab. The patients included 8 men and 2 women. Their median age was 71 years (age range, 31-78 years). Neurological complications occurred after a median of 5.5 (range, 1-20) cycles of anti-PD-1 inhibitors. Complications included myopathy (n = 2), varied neuropathies (n = 4), cerebellar ataxia (n = 1), autoimmune retinopathy (n = 1), bilateral internuclear ophthalmoplegia (n = 1), and headache (n = 1). Peripheral neuropathies included axonal and demyelinating polyradiculoneuropathies (n = 2), length-dependent neuropathies (n = 1), and asymmetric vasculitic neuropathy (n = 1). The time to maximum symptom severity varied from 1 day to more than 3 months. The median mRS score was 2.5 (range, 1-5), indicating mild to moderate disability. Five patients experienced other systemic immune-mediated complications, including hypothyroidism (n = 3), colitis (n = 2), and hepatitis (n = 1). Treatment with anti-PD-1 antibodies was discontinued in 7 patients. Treatment included corticosteroids (n = 7), intravenous immunoglobulin (n = 3), and plasma exchange (n = 1). Nine patients improved, with a median mRS score of 2 (range, 0-6). One patient with severe necrotizing myopathy died. Neurological adverse events associated with anti-PD-1 therapy have a diverse phenotype, with more frequent neuromuscular complications. Although rare, they will likely be encountered with increasing frequency as anti-PD-1 therapy expands to other cancers. The time of onset is unpredictable, and evolution may be rapid and life-threatening. Prompt recognition and discontinuation of anti-PD-1 therapy is recommended. In some cases, immune rescue treatment may be required.

Ocular Motor Manifestations of Multiple Sclerosis.

Multiple sclerosis (MS) is a demyelinating disease of the central nervous system leading to disability, especially in young patients. Acute or chronic lesions of MS within the brainstem and the cerebellum frequently result in ocular motor disorders. This review encompasses the spectrum of ocular motor disorders in patients with MS emphasizing prevalence, examination findings, diagnostic features, functional consequences, classification of MS course, and management of these disturbances of ocular motility. Ocular motor manifestations of MS can occur acutely in relapse or chronically, the latter as a consequence of previous relapses or as a chronic course of the disease. The most frequent and specific acute ocular motor manifestation is uni- or bilateral internuclear ophthalmoplegia (INO). The most frequent chronic manifestations include INO and cerebellar ocular motor disorders such as gaze-evoked nystagmus, saccadic hypermetria, and lack of vestibulo-ocular reflex inhibition. The most disabling syndrome is pendular nystagmus. The high prevalence of ocular motor manifestations emphasizes the importance of neuro-ophthalmological examination among patients with MS. Because chronic manifestations may cause minimal or no symptoms, a systematic investigation of the most common manifestations should be performed in daily practice. Appropriate treatment may improve visual outcome in some of these ocular motor disorders.

Demographic and clinical profile of Multiple Sclerosis in Kashmir: A short report

BackgroundMultiple sclerosis (MS) is a chronic autoimmune and inflammatory disease of the central nervous system (CNS). There have been only few population/hospital based studies on MS in India, and at the same time there is no data on its profile in Kashmir. MethodsA total of 41 MS patients diagnosed on the basis of 2010 Revised Mc Donald criteria were enrolled in this study from Kashmir region of India. Clinical, demographic, radiological and biochemical parameters were analyzed for most of the patients. ResultsMale to female ratio was found to be 1:3.1 with mean age at the time of analysis 32.26±7.54 (range 18–48) years. The mean disease duration was found to be 3.2±3.6 years. The most common course was relapsing-remitting (RR) present in 87.80% of cases followed by secondary progressive (SP) in 9.76% and primary progressive (PP) in 2.44%. Numbness, weakness of limbs, prickling and tingling sensations, muscle stiffness, and visual disturbances were most common manifestations. Condition of bilateral internuclear ophthalmoplegia (INO) and vertigo was rarely observed. Oligoclonal bands (OCB) were present in cerebrospinal fluid (CSF) of majority of the patients. Symptomatic and steroidal treatment mode was given to majority of the patients (92.68%) and only 7.32% patients were given disease modifying drug. ConclusionThis is the first preliminary report on MS profile in Kashmir. The high prevalence of female patients and RR course of MS, low prevalence of progressive cases, predominance of OCB positive cases, insignificant family history in all cases, predominance of cases with low socio-economic status, and high rate of less educated and unemployed cases are the most important findings. By and large MS pattern in Kashmir was found to be relatively similar to West and rest of the Asia. Larger comprehensive studies are mandatory to completely understand MS pattern in Kashmir. There is utmost requirement to maintain a local MS registry in Kashmir so as to get an idea about the actual number of persons suffering from the disease and compare the data with other regions of India.

Webino Syndrome (Wall-Eyed Bilateral Internuclear Ophthalmoplegia)

Figure 1: T2W MRI image showing bilateral lesions in dorsum of rostral pons. Figure 2: Showing horizontal eye movement pathway. Figure 3: Showing bilateral horizontal gaze internuclear ophthalmoplegia (wall eyed bilateral internuclear ophthalmoplegia/WEBINO). Figure 4: Showing improvement in bilateral horizontal gaze movement after one month of treatment with interferon beta Clinical Image: Multiple Sclerosis is an autoimmune disease of the Central Nervous System characterized by chronic inflammation, demyelination, gliosis and neuronal loss. The disease course can be relapsing-remitting or progressive type. Symptoms of Multiple Sclerosis are extremely varied and depend on location and severity of lesion within CNS. This is a case of nineteen years old female with multiple sclerosis presented in neurology clinic with chief complaints of diplopia on horizontal gaze and urinary retention. On examination patient was found to have bilateral conjugate horizontal gaze palsy with inability to converge her eyes. MRI brain revealed lesions in dorsum of pons extending into midbrain. Treatment given with injection methylprednisolone for 5 days followed by disease modifying therapy with interferon beta. After 4 weeks of therapy, patient has improved symptomatically. This presentation of multiple sclerosis as wall eyed bilateral internuclear ophthalmoplegia is rare and diagnostic of Multiple Sclerosis until proven otherwise (Figures 1-4)

Pathways Linked to Internuclear Ophthalmoplegia on Diffusion-Tensor Imaging in a Case with Midbrain Infarction

Midbrain infarction shows diverse patterns of ophthalmoplegia; however, the association of ophthalmoplegia with a precise microanatomy has not been fully studied. Here, we report a patient with characteristic ophthalmoplegia and explore the associated pathologic fiber tracts using diffusion-tensor imaging (DTI). A 21-year-old woman with an 11-year history of mixed connective tissue disease (MCTD) abruptly developed bilateral internuclear ophthalmoplegia (INO) with upward gaze and convergence palsies. Plasma levels of U1-ribonucleoprotein, double-stranded-DNA antibodies, and cerebrospinal fluid interleukin-6 were all increased. Diffusion-weighted imaging showed an acute ischemic lesion in the periaqueductal gray matter. DTI exhibited a reduction of fractional anisotropy and an increase of apparent diffusion coefficient values in the tract involving the left medial longitudinal fasciculus (MLF) in the midbrain to the posterior commissure (PC) when compared to the right-sided tract in the midbrain and to the bilateral MLF in the upper pontine levels. Antiplatelet and immunosuppressant therapies dramatically improved her symptoms. We believe this is the first case of a patient with juvenile MCTD presenting with bilateral INO with an upward gaze and convergence palsies caused by midbrain infarction associated with vasculitis. It is suggested that DTI might identify the pathologic fiber tract connecting the left MLF in the midbrain to the PC.

Gaze palsy and exotropia in internuclear ophthalmoplegia

Background and purposeUnilateral gaze palsy associated with internuclear ophthalmoplegia (INO), i.e., one-and-a-half syndrome, is well known. Exotropia can also be associated with INO, but it has been reported only rarely. We sought to determine the frequencies and courses of gaze palsy and exotropia in INO. MethodsPatients hospitalized with acute-onset INO during the period January 2009 through December 2013 were identified from our clinical registry. Associated gaze palsy and exotropia were evaluated in the identified patients. ResultsTwenty-five patients with unilateral INO and 7 patients with bilateral INO were included in this study. Of the 25 patients with unilateral INO, 4 (16.0.0%) had ipsilateral gaze palsy (one-and-a-half syndrome), 8 (32.0%) had exotropia (non-paralytic pontine exotropia), and 6 (24.0%) had both ipsilateral gaze palsy and exotropia (paralytic pontine exotropia). Six (85.7%) of the 7 patients with bilateral INO had exotropia. The gaze palsy persisted more than 1week in 40.0% of patients, whereas the exotropia disappeared within 1week in 92.9% of patients when the INO was unilateral. ConclusionExotropia is not uncommon in the acute stage of INO. However, it is often overlooked because of its short duration.