Assessment the neurodegenaration process of post-geniculate optic pathway in thalamic tumors using optical coherence tomography: Post-geniculate optic pathway in thalamic tumors.

PURPOSE: To investigate the effect of thalamic tumors related to post-geniculate optic pathway and those of microsurgical removal on neurodegeneration process of visual pathway using OCT. METHODS: This cross-sectional study included 30 eyes of 15 patients with thalamic tumors and data compiled from 45 eyes of 45 healthy participants. The analysis of variables was divided into ipsilateral and contralateral eye depends on the laterality of brain tumors. The data gained at baseline, postoperative 3rd and postoperative 6th month period was analyzed in One-Way Repeated Measures ANOVA. RESULTS: The mean superior quadrant retinal nerve fiber layer (RNFL) thickness of ipsilateral eye was significantly thicker (p = 0.02), while inferior hemifield ganglion cell-inner plexiform layer (GC-IPL) thickness of contralateral eye was significantly higher compared to control groups (p = 0.02). Significant negative correlations were found between the mean preop. mean deviation (MD) and tumor volume and time interval between initial diagnosis and surgery (r = - 0.730, p = 0.002 and r = - 0.680, p = 0.005, respectively) in the ipsilateral eye. In repeated measure analysis, there was no unfavourable effect of thalamic microsurgery on peripapillary average RNFL thickness on both ipsilateral and contralateral eyes (Ipsilateral eye; f (1.5,21.7) 0.76 p = 0.51 and contralateral eye; f (1.4,20.4) 0.42 p = 0.59). CONCLUSION: This study suggests that thalamic tumors affecting post-geniculate optic pathway may lead to significant increase in RNFL and GC-IPL thicknesses and changes in VF parameters. Timely intervention in thalamic tumors may prevent irreversible loss of retinal axons secondary to neurodegeneration process.

Changes in the peripapillary and subfoveal choroidal vascularity index after transsphenoidal surgery for pituitary macroadenoma.

PURPOSE: To investigate the changes in peripapillary and subfoveal choroidal vascular indexes (CVI) before and after pituitary macroadenoma surgery by using a binarization method. METHOD: In this cross-sectional study, we examined 17 eyes in 9 patients with pituitary macroadenomas who had undergone transsphenoidal pituitary surgery due to chiasmal compression. We also compiled data from 17of in 17 healthy subjects. ImageJ 1.51 software processing (National Institutes of Health, Bethesda, Maryland, USA) was used for binarization of optical coherence tomography scans. The CVI was computed as the ratio of luminal area to total choroidal areal. The CVI, OCT and VF parameters were analyzed in One-Way Repeated Measures ANOVA to determine significant changes in measurements during the postoperative course. RESULTS: The mean peripapillary inferior and temporal quadrant CVIs were significantly lower in the eyes of patients with pituitary macroadenoma compared to controls (46.0 ± 0.03 versus. 42.8 ± 0.04, p = 0.02; 45.8 ± 0.03 Versus. 42.3 ± 0.04, p = 0.02). In repeated measure analysis, there was a significant effect of transsphenoidal microscobic pituitary surgery on peripapillary inferior quadrant CVI and BCVA, F(1.3, 21.5) = 6.62, p = 0.01 and F (1.8, 29.7) = 7.8, p < 0.005, respectively. CONCLUSION: This study suggests that PMa with chiasmal compression may lead to significant changes in the peripapillary CVI. Pituitary surgery had a favorable significant effect on peripapillary choroidal vascular network and BCVA. Furthermore, optical coherence tomography is a helpful technique for quantifying the alterations of peripapillary CVI during the preoperative and postoperative course.

Retinal nerve fibre changes in sports-related repetitive traumatic brain injury.

IMPORTANCE: There is limited literature on the use of optical coherence tomography in the assessment of retinal nerve fibre layer (RNFL) thickness in sports-related repetitive mild traumatic brain injury. BACKGROUND: To evaluate RNFL thickness in professional rugby league players. RNFL thinning may serve as a proxy for wider white matter degeneration. DESIGN: Cross-sectional observational study. PARTICIPANTS: Thirteen retired Australian professional rugby league players were recruited. METHODS: Participants underwent binocular optical coherence tomography to measure RNFL thickness. Each participant underwent a complete ophthalmic assessment to exclude concurrent disease. MAIN OUTCOME MEASURES: RNFL thickness of each eye were compared with a normative database. RESULTS: Participants had played professional Rugby League for 18 years on average and reported sustaining 15 sports-related concussions throughout their career. The RNFL in participants was four micrometres thinner than that of matched normative data. Cohort average RNFL thickness was reduced in 12 out of 14 optical coherence testing parameters. These findings were statistically significant in the left inferonasal [P = .013] and left nasal [P = .006] sectors. There was no statistically significant relationship between RNFL thickness and other visual measures. CONCLUSIONS AND RELEVANCE: This study is the first to demonstrate RNFL thinning in a cohort of retired Australian professional Rugby League players. RNFL changes have been shown to correlate with cerebral white matter loss and neurodegeneration. Optical coherence tomography may serve as a safe and economical means of screening for repetitive traumatic brain injury related neurodegeneration in contact sport athletes.

Late-onset Leber hereditary optic neuropathy.

BACKGROUND: While Leber hereditary optic neuropathy typically causes bilateral visual loss in the second through fourth decades, we highlight visual loss from Leber hereditary optic neuropathy in older patients to characterize the clinical features of this cohort. DESIGN: Retrospective case series. PARTICIPANTS: Patients seen between January 2003 and July 2012 at Baylor College of Medicine and between April 2010 and July 2012 at The Methodist Hospital in Houston, Texas. METHODS: Patients with visual loss from genetically confirmed Leber hereditary optic neuropathy were identified via retrospective chart review. MAIN OUTCOME MEASURES: Clinical courses of patients. RESULTS: Five patients with visual loss from genetically confirmed Leber hereditary optic neuropathy were greater than 60 years of age at the time of visual loss (range 62-70 years, mean 66.4 ± 3.0). CONCLUSION: This series reinforces the importance of including Leber hereditary optic neuropathy in the differential diagnosis of patients of any age with optic neuropathy.

Hypertensive Retinopathy as a Presenting Sign of Pheochromocytoma with Malignant Hypertension: A Child Case.

A 13-year-old was admitted to our clinic complaining about a vision loss of over 2 weeks. Bilateral optic disc edema, peripapillary flame-shaped hemorrhages, macular star pattern exudates, and cotton wool spots were found in fundoscopic examination. The OCT exam showed bilaterally serous retinal detachments in sub-foveal region with intraretinal exudates. A pediatric examination found a high systemic hypertension of 230/140 mm Hg, and laboratory tests revealed increased levels of plasmatic and urinary catecholamines. An abdominal MRI showed a left suprarenal pheochromocytoma and the child underwent an adrenalectomy urgently. Informed patient consent was obtained from legal guardians to publish clinical images. Malignant hypertension (MHT) as a result of a pheochromocytoma is an extremely rare condition in childhood. MHT crisis represents a potential life-threatening event and an immediate treatment can prevent a multi-organ failure, including the eyes. An early diagnosis of MHT by fundus examination can lead to a completely favorable ophthalmological evolution and entirely functional recovery.

Aberrant regeneration in an international registry of patients with 3rd-nerve palsy.

BACKGROUND/AIMS: To describe the patterns of pre-operative aberrant regeneration and motility outcomes reported in an international registry of patients with 3rd-nerve palsy treated with nasal transposition of the split lateral rectus muscle (NTSLR). METHODS: This cross-sectional study used data from an international, multicentre registry of patients with 3rd-nerve palsy treated with NTSLR. Patients with aberrant regeneration were identified, and patterns of innervation described. Demographics and postoperative success defined as horizontal alignment ≤15 PD were compared based on the presence, and type, of aberrant regeneration using Wilcoxon rank sum and Fisher's exact tests. RESULTS: Aberrant regeneration was reported in 16% (21/129) of patients. Age at diagnosis, sex, and aetiology of palsy were not significantly associated with aberrant regeneration. Abnormal movements were triggered by adduction in 52% (11/21), infraduction in 23% (5/21), and supraduction in 23% (5/21) of cases. Presentation patterns involved rectus muscle innervation in 29% (6/21) and levator muscle innervation in 71% (15/21) of cases. Although patients with aberrant regeneration had similar probability of success in comparison to those without following NTLSR (76% vs. 69%, p = 0.5), those with abnormal innervation of a rectus muscle had a lower success rate than those with abnormal innervation of the levator palpebrae superioris muscle (17% vs. 93%; p = 0.002). CONCLUSION: Successful treatment of a 3rd nerve palsy with NTSLR was not influenced by aberrant regeneration involving the levator muscle. Alternative surgical interventions should be considered when aberrant regeneration alters rectus muscle function given its adverse impact on motor outcomes with NTSLR.

Ocular factors predicting requirement for corneal protective oculoplastic surgery.

BACKGROUND: Currently there is no universally agreed schema for predicting ocular morbidity in facial nerve palsy. The House Brackmann Scale has limitations in assessing ocular morbidity from facial nerve palsy. Our aim was to create a scoring system to help quantify ocular morbidity to aid in decision making regarding the need for corneal protective oculoplastic surgery. METHODS: We conducted a large cohort study observing 606 patients attending the specialist facial palsy clinic in Manchester UK between March 2002 and October 2017. Retrospective multivariate analysis identified clinical predictors for the 316 patients that required oculoplastic surgery. ß coefficients generated in the multivariate analysis helped formulate a new facial nerve palsy scoring instrument to predict the need for corneal protective oculoplastic surgery. RESULTS: The House Brackmann Scale, corneal lagophthalmos and loss of corneal sensation proved clinically significant predictors for requiring corneal protective oculoplastic surgery. The scoring system derived from these factors provided an accurate and repeatable prediction tool demonstrated by validation studies on our patient population. The area under the ROC curve for the multivariate prediction model was 0.769 (0.726, 0.811). A score of 5 points out of a possible 8 was the best cut off score to recommend oculoplastic surgery, giving a sensitivity of 0.750 and a specificity of 0.671. CONCLUSION: This study demonstrates that corneal lagophthalmos, corneal sensation and the House Brackmann Scale are important in predicting the need for corneal protective oculoplastic surgery. Our scoring tool is an important clinical decision tool for ophthalmic and ENT colleagues.

Predictive value of OCT and MRI for postoperative visual recovery in patients with chiasmal compressive lesions.

PURPOSE: We aimed to investigate the predictive value of retinal thickness measured by optical coherence tomography (OCT) and mass biometrics measured using magnetic resonance image (MRI) for visual recovery after surgery for removal of a mass compressing the optic chiasm. METHODS: Consecutive patients who showed typical temporal visual field defect (VFD) with respect to the vertical meridian due to a chiasmal compressive mass and who underwent mass removal surgery were recruited. Ophthalmic examination was performed preoperatively and postoperatively. Retinal thickness was measured by the Cirrus OCT. The height and size of the mass and suprasellar extension (SSE) in both the sagittal and coronal planes were evaluated. Patients were divided into two groups based on the improvement in VFD (mean deviation [MD] change ≥ 5 dB: group R; others: group NR) and clinical characteristics were compared. RESULTS: Fifteen patients were included in the study. Eight (53.3%) patients were allocated into group R and others (7 patients, 46.7%) into group NR. Age, sex, initial visual acuity, initial MD was not different between the two groups. The retinal thicknesses were not different while tumor height, volume, and both sagittal and coronal SSE were significantly different between the two groups. (p = 0.029, 0.014, <0.001, and <0.001, respectively) All MRI parameters showed significant predictive value for the degree of MD recovery. CONCLUSION: MRI showed better predictive value than OCT in predicting postoperative VFD recovery in patients with temporal VFDs due to chiasmal compressive disorder.

How do intracranial tumors associated with visual pathways affect quadrant and sectoral based optical coherence tomography measurements: The effect of intracranial tumors on RNFL and GC-IPL thickness.

AIM: To investigate the quantitative sectoral and regional changes of retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GC-IPL) thickness in different types of intracranial tumors associated with visual pathways. METHODS: This comparative retrospective study included 58 eyes of 30 patients with intracranial tumors and the data of 62 eyes of 31 healthy subjects. The RNFL and GCIPL thicknesses were analyzed using spectral-domain optical coherence tomography (OCT). The tumors were classified into ones that affect pre-geniculate and post-geniculate pathway. RESULTS: The mean RNFL thickness in temporal quadrant was significantly thinner in eyes with intracranial tumors affecting the pre-geniculate optic pathway compared to controls (p = 0.04). In contrast, the mean superior quadrant RNFL thickness was significantly thicker in eyes with brain tumors associated with post-geniculate optic pathway (p = 0.01). The mean GC-IPL thicknesses of the inner ring superotemporal, superonasal, inferotemporal and inferionasal sectors and outer ring superonasal and inferonasal sectors were significantly thinner in eyes with intracranial tumors affecting the pre-geniculate optic pathway compared to control eyes (p = 0.02, p = 0.001, p = 0.02, p = 0.003, p = 0.008 and p = 0.03 respectively). CONCLUSION: The results of this study showed that significant changes can be seen in the different RNFL quadrants and GC-IPL sectors in eyes with intracranial tumors affecting pre-geniculate or post-geniculate optic pathway. OCT is a very useful imaging technique to quantify these structural changes which take place during the neurodegeneration process of visual pathways in intracranial tumors.

Direct and transsynaptic retrograde degeneration and optic nerve head microvascular changes in patients with hemianopia.

PURPOSE: To investigate optic nerve head (ONH) microvascular changes secondary to transsynaptic retrograde degeneration (TRD), comperatively with direct retrograde degeneration and healthy controls. METHODS: Patients with hemianopia due to intracranial lesion included in the study. Intracranial lesion was categorized by location: postgeniculate (causing TRD), chiasmal (causing direct retrograde degeneration). For the postgeniculate lesions, the eye on the same side of the lesion was defined as the ipsilateral eye and the eye on the opposite side as the contralateral eye. Optic disc microvasculature was evaluated with the help of optic coherence tomography angiography. RESULTS: Sixteen eyes of 16 patients with chiasmal lesion, 28 eyes of 14 patients with postgeniculate lesion, and 30 eyes of 30 healthy subjects were included in the study. Ipsilateral eyes of the patients with postgeniculate lesion had decreased vessel density at the temporal sectors compatible with the affected nasal side of the visual field. Contralateral eyes showed no reduction of the vessel density at the affected nasal sectors. The eyes with chiasmal lesions had decreased vessel density at the peripapillary region and nasal half of the ONH compatible with temporal hemianopia. Vascular changes in the chiasmal lesion were more prominent than those of the postgeniculate lesion. Retinal nerve fiber layer and ganglion cell complex thickness were reduced. CONCLUSION: Vessel density of ONH was reduced in patients with homonymous hemianopia, providing evidence for TRD secondary to acquired postgeniculate lesion. Direct retrograde degeneration was more prominent in affected sectors when compared to TRD.

Reproducibility of macular retinal nerve fiber layer and ganglion cell layer thickness measurements in a healthy pediatric population.

PURPOSE: To evaluate the reproducibility of Spectralis spectral domain-OCT segmented ganglion cell layer (GCL) and macular retinal nerve fiber layer (mRNFL) measurements in a healthy children population. METHODS: An observational, cross-sectional study was carried out on 79 children to measure the intraobserver reproducibility and the repeatability between a novel and an experienced operator of the segmented macular GCL and mRNFL. Interobserver and intraobserver reproducibility were defined by the intraclass correlation coefficient (ICC) and coefficients of variation (COV). Kruskal-Wallis test was used to determine statistical significance in the COV of three age groups children (younger than 6 years, between 6 and 12, and older than 12 years old). RESULTS: The results from the intraoperator GCL thickness analysis were highly reproducible (COV < 6%) and reliable (ICCs > 0.81). When the measurements were compared between a novel and an experienced examiner lower ICCs and higher COV were found. COVs ranged from 1.85% (total volume area) to 5.57% at the central ETDRS subfield while the ICC vary from 0.632 (outer inferior) to 0.832 (inner inferior). The repeatability and reproducibility of the mRNFL thickness were lower, with ICCs ranging from 0.428 to 0.872 in the interobserver analysis and from 0.897 to 0.346 in the interobserver one. CONCLUSION: In the present study, we establish substantial reliability of the GCL thickness in children with Spectralis® SD-OCT in all the sectors, albeit lower than the reported in the literature with other SD-OCTs and in adults. The reproducibility and repeatability of the mRNFL were significantly lower. We were unable to find consistent statistical significant differences between the COV of the three age groups.

Surgical management of a case of contractile peripapillary staphyloma with retinal detachment and macular hole.

A healthy 4-year-old male presented a fundus examination with a unilateral contractile peripapillary staphyloma surrounded by redundant retina and retinal pigment epithelium atrophy. Five years later, best-corrected visual acuity decreased to hand motion due to a retinal detachment with macular hole. One month after first vitrectomy, scleral buckle and intraocular gas, retina re-detached. Second surgery was performed with silicon oil tamponade and lensectomy without intraocular lens (IOL). Subretinal silicon oil was detected at the third month of follow-up when vitrectomy, inferior retinectomy, and laser photocoagulation of temporal border of staphyloma with silicon oil tamponade were performed. The retina remained attached and best-corrected visual acuity was 20/600 with intraocular silicon oil. A fourth surgery was performed for emulsified silicon oil extraction replaced with intraocular gas. At 6 months of follow-up, the retina re-detached again. This is a challenging vitreoretinal surgery in which re-detachments were due to retinal folds around the contractile staphyloma that raised macular hole. This is the first report of the combined presentation of contractile peripapillary staphyloma, retinal detachment and macular hole with a long-time follow-up period of years.

Saccadic movements assessment in eccentric fixation: A study in patients with Stargardt disease.

PURPOSE: To investigate saccadic movements in subjects with eccentric fixation due to a deep central scotoma in Stargardt disease (STGD). METHODS: We studied 10 patients with STGD and 10 healthy subjects (control group). Saccadic movements of all the 20 subjects were assessed by using the eye tracker technique Tobii Glasses Pro 2. Standard measurements of reading ability (MNREAD charts), visual acuity (ETDRS charts), contrast sensitivity (Pelli-Robson charts), reading contrast threshold and speed (REX charts), retinal sensitivity and stability and localization of the fixation (MP1 fundus perimetry) were obtained in all subjects. RESULTS: The saccadic movements time was significantly slower in STGD than in healthy subjects (699 ± 193 ms vs 299 ± 40 ms, p < 0.001). When STGD patients moved fixation to the target localized in retinal scotomatous areas, the movement was significantly slower compared to non scotomatous areas in the retina (1103 ± 798 ms vs 524 ± 187 ms, p = 0.039). There was a trend toward a correlation between slow saccadic movements in STGD subjects and the reading performance indices, although statistical significance was not achieved. CONCLUSION: Ocular saccades guided by eccentric fixation in STGD patients are significantly slower than in the control group, especially when the target corresponds to retinal areas with a deep scotoma. These results can explain the worse reading performance in STGD subjects, in particular when a non-viewing area on the right part of the text is present.

Systematic review and meta-analysis of the development of multiple sclerosis in clinically isolated syndrome.

Clinically isolated syndrome (CIS) usually describes a first clinical episode that suggest multiple sclerosis. One of the signs and symptoms of CIS is Optic neuritis (ON). Several early intervention drugs are being tested in clinical trials for the early intervention in CIS patients which may lead to multiple sclerosis (MS). This study aim (1) early intervention of the CIS progression to MS delay slow down the MS progression, (2) since that ON affects 30%-70% of MS patients, the available ON intervention delay the MS progression. PubMed was used as a search engine. Summary relative risks (RRs) and 95 % confidence intervals (CIs) were estimated using a random-effect model. For aim 1, it showed lower risk of progression to MS compared to non-treated group (Risk Ratio (RR) 0.64; 95%CI: 0.58, 0.69; p < 0.001). For aim 2, drug treated group showed lower risk of progression compared to non-treated group (RR 0.19, 95%CI: 0.06, 0.54, p < 0.002. Statistical heterogeneity was not significant in both outcomes and bias of publication was not found using inspection of funnel plot and Egger's statistical test in both outcomes. In conclusion, early treatment should be considered in CIS patients to prevent the progression of MS. Moreover, this data shows that early intervention (Erythropoietin, Ery) of ON decreases the conversion risk to MS.

Umbilical cord-derived mesenchymal stem cell implantation in patients with optic atrophy.

BACKGROUND: Optic nerve cells can be irreversibly damaged by common various causes. Unfortunately optic nerve and retinal ganglion cells have no regenerative capacity and undergo apoptosis in case of damage. In this study, our aim is to investigate the safety and efficacy of suprachoroidal umbilical cord-derived MSCs (UC-MSCs) implantation in patients with optic atrophy. METHODS: This study enrolled 29 eyes of 23 patients with optic atrophy who were followed in the ophthalmology department of our hospital. BCVA, anterior segment, fundus examination, color photography, and optical coherence tomography (OCT) were carried out at each visit. Fundus fluorescein angiography and visual field examination were performed at the end of the first, third, sixth months, and 1 year follow-up. RESULTS: After suprachoroidal UC-MSCs implantation there were statistically significant improvements in BCVA and VF results during 12 months follow-up (p < 0.05). When we evaluate the results of VF tests, the mean deviation (MD) value at baseline was -26.11 ± 8.36 (range -14.18 to -34.41). At the end of the first year it improved to -25.01 ± 8.73 (range -12.56 to -34.41) which was statistically significant (p < 0.05). When we evaluate the mean RNFL thickness measurements at baseline and at 12 month follow-up the results were 81.8 ± 24.9 µm and 76.6 ± 22.6 µm, respectively. There was not a significant difference between the mean values (p > 0.05). CONCLUSION: Stem cell treatment with suprachoroidal implantation of UCMSCs seems to be safe and effective in the treatment for optic nerve diseases that currently have no curative treatment options.

Estimation and interrater reliability of pupillography by digital mobile app: Digital movil pupillography app validity.

OBJECTIVE: The examination of pupillary light reflexes is widely employed to evaluate the autonomic system's balance, ocular, and neurological conditions, typically evaluated by qualitative observation. To describe and evaluate the inter-rater reliability and agreement of pupillometric values obtained with a novel semi-automatic mobile pupillography app. METHODS: Two examiners and two graders perform the measurements with an android app which corrects the device-face distance, measure the maximum contraction of the pupil, and calculates its slope, in a hospital room setting with three levels of environmental light intensity: <100, 101 to 200, and 201 to 300 lux, at 200, 400, 600, 900, 1200, and 1500 milliseconds. RESULTS: Sixty healthy individuals, aged 18 to 45, were included; 85% had brown eyes. The reliability of the pupil/iris ratio had Ri = 70% to 88%, and bland Altman graphics show a uniform agreement. The pupillography curves show a similar slope at different light intensities, during the contraction phase. CONCLUSION: There were very good repeatability and inter-rater reproducibility of the measurements at average levels of illumination in examination rooms, even in dark-eyed people. This app provides a highly promising approach to pupillary measurements in clinical practice.

Unilateral papilledema in idiopathic intracranial hypertension: A rare entity.

Idiopathic Intracranial Hypertension (IIH), also known as pseudotumor cerebri is a clinical syndrome of unknown etiology, which typically affects young, obese women of childbearing age and is characterized by increased intracranial pressure and bilateral papilledema. Unilateral and highly asymmetrical papilledema is a rare presentation in IIH occurring in less than 4% of patients with definite IIH and can poses a diagnostic challenge for the treating physician as it usually raises the suspicion of localized ocular pathology. As per current consensus, papilledema results in stasis of axoplasmic transport due to mechanical compression, leading to secondary vascular changes of venous dilation and hemorrhage. Given this mechanism, the underlying reasons for unilateral and asymmetrical papilledema remain unclear.

Methylphenidate-associated Alice in Wonderland syndrome.

INTRODUCTION: We describe the case of a child affected by typical symptoms of Alice in Wonderland syndrome (AIWS), related to the methylphenidate treatment he was taking for an attention deficit hyperactivity disorder (ADHD). To our knowledge, this is the first case of methylphenidate-associated AIWS. METHODS: Retrospective single center observational case report. CASE DESCRIPTION: A 12-year-old boy was complaining of micropsias and macropsias. Except a disruptive ADHD treated by methylphenidate for 2 years, his medical history was banal. His symptoms coincided with a change in his treatment regimen and ceased with methylphenidate discontinuation. Unfortunately, they recurred when the medication was reimplemented by his psychiatrist. The ophthalmological examination was unremarkable. We concluded to an AIWS and prescribed ancillary testing (including blood work, electroencephalogram, and brain MRI) to rule out conditions known to be associated with this syndrome. In the meanwhile, the methylphenidate dosage was readapted, and the symptoms disappeared again. Seen this clear dechallenge and rechallenge effect and the fact that all additional tests returned normal results, we deduced that our patient's symptoms were associated to methylphenidate. CONCLUSION: AIWS could be a potential side effect of methylphenidate. Given the frequency of methylphenidate prescription for ADHD and its widespread misuse, it is important to consider this peculiar adverse effect. Every physician should be aware of the condition to offer reassurance and to prescribe the appropriate additional examinations, as life-threatening disorders can cause this syndrome.

Complete bilateral ophthalmoplegia in malignant intracranial hypertension in a child.

PURPOSE: To describe a case of fulminant idiopathic intracranial hypertension (IIH) in a child with "malignant" presentation. CASE REPORT: A 16-year-old, previously healthy, girl presented with bilateral visual loss and bilateral global limitation of eye movements in the absence of headache. Extensive laboratory evaluation for infectious, inflammatory, autoimmune, and neoplastic conditions was negative. Magnetic resonance imaging (MRI) of the brain and lumbar puncture findings were consistent with a diagnosis of IIH. Extraocular motility improved in the next few days as well as optic disc edema but visual acuity remained poor. CONCLUSION: The authors believe that the acute, severe, and fulminant ("malignant") presentation with markedly elevated intracranial pressure may produce the unique presentation of severe vision loss and bilateral complete ophthalmoplegia. Interestingly, there was no headache. To our knowledge this is the first such case to be reported in the English language ophthalmic literature.

Optic atrophy in children: Current causes and diagnostic approach.

BACKGROUND/AIMS: Optic atrophy is one of the leading causes of sight impairment in children. It frequently poses a diagnostic challenge, as it can be caused by many ocular and systemic conditions. We aimed to determine the current causes of optic atrophy at our centre and to describe the use of investigations, including molecular genetic testing. METHODS: We reviewed the medical records of children with optic atrophy seen at Moorfields Eye Hospital between 2010 and 2015. We recorded demographic data, reason for referral, history, investigations and diagnosis. RESULTS: We studied 143 cases aged below 16 years. A cause could be identified in all cases. Taking a full history was the most important part of the diagnostic workup, identifying a cause in 96 (67%) children. A developmental disorder of the brain and/or optic nerve, sometimes with retinal involvement, was the commonest cause (n = 33, 23%), followed by inheritable optic neuropathies (n = 27, 19%). Other causes included perinatal insults (n = 18, 13%), post-infectious or post-inflammatory conditions (n = 18, 13%), accidental or abusive trauma (n = 14, 10%) and inheritable retinal dystrophies (n = 13, 9%). Rare conditions included neurodegenerative disorders (n = 7, 5%), skeletal developmental disorders such as rickets (n = 4, 3%), tumours (n = 4, 3%), ischaemic events including large optic nerve head drusen (n = 4, 3%) and toxic events/metabolic conditions (n = 1, 0.7%). CONCLUSION: In this series, an underlying cause could be identified in all cases. Taking a comprehensive antenatal, perinatal, postnatal and family history will indicate a probable diagnosis in two-thirds of children, and targeted ancillary tests may identify the cause in most remaining cases.