Pharmacologic Mydriasis Secondary to Topical Glycopyrronium Tosylate Cloths: Clinical Characterization From a Multicenter Analysis.

BACKGROUND: Topical glycopyrronium tosylate (GT) is an anticholinergic medication for treatment of axillary hyperhidrosis. Pharmacologic mydriasis and anisocoria from topical GT has been reported and may be underrecognized. This study aims to clinically characterize patients presenting with pharmacologic mydriasis from exposure to this medication. METHODS: This study is a retrospective observational case series. A multicenter chart review of 16 patients diagnosed with pharmacologic mydriasis secondary to topical GT was performed. RESULTS: Eight patients (50.0%) were age 18 years and younger, and 14 patients (87.5%) were female. Unilateral mydriasis (anisocoria) occurred in 14 patients (87.5%). Fourteen patients (87.5%) did not initially volunteer topical GT as a "medication," and the history of topical GT exposure needed to be elicited with further questioning. Hand hygiene details were known for 12 patients, and all reported that they did not wash their hands after GT application. Six patients (37.5%) were soft contact lens users. One patient had possible exposure through a family member's use of the medication. Ocular symptoms were common (blurry vision [11 patients, 68.8%] and eye dryness [7 patients, 43.8%]), but systemic anticholinergic symptoms were uncommon (such as constipation [1 patient, 6.3%] and urinary symptoms [3 patients, 18.8%]). CONCLUSIONS: Mydriasis associated with topical GT seems to be a consequence of local exposure rather than systemic toxicity. Because patients may not volunteer topical GT as a medication, eliciting a history of exposure often requires further specific questioning. Soft contact lens wear and poor postapplication hand hygiene seem to be associated with mydriasis in GT use.

Ocular lateral deviation with brief removal of visual fixation differentiates central from peripheral vestibular syndrome.

OBJECTIVE: Ocular lateral deviation (OLD) is a conjugate, ipsilesional, horizontal ocular deviation associated with brief (3-5 s) closing of the eyes, commonly linked to the lateral medullary syndrome (LMS). There is limited information regarding OLD in patients with the acute vestibular syndrome (AVS). In one case series 40 years ago OLD was suggested to be a central sign. Recently, horizontal ocular deviation on imaging (RadOLD) was frequently associated with anterior circulation stroke and horizontal gaze palsy. Similarly, RadOLD has been associated with posterior circulation stroke, e.g., LMS and cerebellar stroke, but without clinical correlation with OLD. METHODS: This is a prospective, cross-sectional diagnostic study of 151 acute AVS patients. Patients had spontaneous nystagmus. Horizontal gaze paralysis was an exclusion criterion. We noted the effect of brief 3-5 s eyelid closure on eye position, and then used the HINTS algorithm (the head-impulse test, nystagmus characteristics and skew deviation) and RadOLD, to establish a correlation between clinical and radiologic findings RESULTS: Of the 151 AVS patients, 100 had a central lesion and 51 a peripheral lesion; 29 of the central lesions were LMS, and 11 had OLD. Additionally, one lateral pontine syndrome had OLD. On opening the eyes 11 patients with OLD and LMS made multiple, hypometric corrective saccades to bring gaze back to straight ahead. 10/11 patients with LMS showed RadOLD. CONCLUSIONS: OLD with multiple hypometric corrective saccades on opening the eyes was infrequent but highly localizing and lateralizing. We emphasize how simple it is to test for OLD, with the caveat that to be specific, it must be present after just brief (3-5 s) eyelid closure.

Report on the Reunión Iberoamericana de Neuro-otologia 2018.

A report on the Reunión Iberoamericana de Neuro-otologia 2018 Fundacion Iberoamericana de Neuro-otologia.

Thrombolyic and Vasodilator Treatment in a Patient With Prolonged Retinal Ischemia.

Vasospastic transient monocular vision loss associated with systemic lupus erythematosus and antiphospholipid syndrome is typically short lasting and responsive to vasodilators. Virchow's triad of endothelial dysfunction, arterial stasis, and a hypercoagulable state are factors in systemic lupus erythematosus/antiphospholipid syndrome that may potentially contribute to prolonged retinal hypoperfusion and central retinal artery occlusion. Consequently, rapid intervention to address all components of Virchow's triad may increase the probability of a good outcome. Time of retinal viability should guide the management strategy. We report a systemic lupus erythematosus/antiphospholipid syndrome patient with prolonged monocular blindness coinciding with retinal arterial narrowing and rouleaux formation who responded favorably to sequential use of vasodilators and intravenous thrombolysis, addressing each component of Virchow's triad.

Primary Ocular Adnexal Extranodal Marginal Zone Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma Presenting as Orbital Apex Syndrome.

A 75-year-old female with a past medical history significant for prior stroke and atrial fibrillation presented with acute onset of orbital apex syndrome with chemosis and periorbital ecchymosis. Following initial treatment to relieve intraocular pressure, she began spontaneously haemorrhaging retro-orbitally. Preliminary investigation with neuroimaging demonstrated a left orbital mass with extension into the orbital apex. A provisional diagnosis of cavernous haemangioma was made. She was treated with transorbital resection of the orbital mass. Subsequent histopathology revealed a diagnosis of ocular adnexal non-Hodgkin lymphoma of histologic type extranodal marginal zone of mucosa-associated lymphoid tissue (MALT lymphoma). MALT lymphoma should be considered in cases of orbital apex syndrome.

Eyes and stroke: the visual aspects of cerebrovascular disease.

A large portion of the central nervous system is dedicated to vision and therefore strokes have a high likelihood of involving vision in some way. Vision loss can be the most disabling residual effect after a cerebral infarction. Transient vision problems can likewise be a harbinger of stroke and prompt evaluation after recognition of visual symptoms can prevent future vascular injury. In this review, we discuss the visual aspects of stroke. First, anatomy and the vascular supply of the visual system are considered. Then, the different stroke syndromes which involve vision are discussed. Finally, topics involving the assessment, prognosis, treatment and therapeutic intervention of vision-specific stroke topics are reviewed.

Fusiform Left Carotid-Ophthalmic Artery Aneurysm Presenting with Central Retinal Artery Occlusion.

Central retinal artery occlusion (CRAO) is a devastating ocular emergency characterized by acute painless visual loss in the ipsilateral eye. We describe the case of acute non-arteritic CRAO associated fusiform internal carotid-ophthalmic artery aneurysm with intraluminal thrombus. Despite the rarity of this condition, we suggest that carotid-ophthalmic artery aneurysm should be included in the differential diagnosis of CRAO.

Identification and validation of clinically meaningful benchmarks in the 12-item Multiple Sclerosis Walking Scale.

BACKGROUND: The 12-item Multiple Sclerosis Walking Scale (12-MSWS) is a validated questionnaire which assessed walking function; it has been widely adopted in multiple sclerosis (MS) clinical research. OBJECTIVE: Identify and validate clinically meaningful 12-MSWS benchmarks in MS. METHODS: Cross-sectional study of 159 MS patients permitted identification of clinically meaningful 12-MSWS benchmarks based on their relationship to real-life anchors. Identified 12-MSWS benchmarks were then validated in a second population of 96 subjects using measures of ambulation, cognition, and patient-reported outcomes. RESULTS: 12-MSWS score of 0-24.99 was associated with working outside the home and assistance-free mobility; 25-49.99 was associated with gait disability and difficulty doing housework; 50-74.99 was associated with unemployment, government healthcare, cane use, and difficulty performing instrumental activities of daily living (IADLs); and 75-100 was associated with change in occupation due to walking, mobility impairment requiring bilateral assistance, and inability to perform IADLs. During the validation step, strong linear associations were identified between 12-MSWS benchmarks and other MS-related disability outcome measures, including ambulatory and non-ambulatory measures. CONCLUSION: We have identified clinically meaningful 12-MSWS benchmarks which define four groups differentiated by increasing levels of mobility impairment and associated loss of functional independence. These data provide insight into how 12-MSWS translate to meaningful functional limitations in MS.

A Rare Case of Unilateral Progressive Vision Loss and Pachymeningitis.

We describe a 32-year-old man with presumed Vogt-Koyanagi Harada (VKH) syndrome, whose presenting symptoms were headache and progressive loss of vision in the right eye. Neuro-ophthalmic examination showed anterior and posterior uveitis, and retinal detachment in the right eye. Ocular coherence tomography (OCT) showed extensive submacular fluid in the right eye, while the fundus fluorescein angiogram (FFA) confirmed perifoveal retinal pigment epithelium (RPE) disruption and multifocal fluorescein leakage in the right eye. The brain MRI showed a small crescent of dependent fluid layering in the right posterior globe adjacent to the right optic nerve head, and pachymeningeal enhancement of the skull base dura along the clivus.This case demonstrates the utility of brain MRI and OCT findings in the early diagnosis of VKH syndrome, in the absence of prominent clinical signs of meningitis. Aggressive treatment is critical to preserve vision and prevent development of other systemic complications of the disease.

Detection of retinal blood vessel changes in multiple sclerosis with optical coherence tomography.

Although retinal vasculitis is common in multiple sclerosis (MS), it is not known if MS is associated with quantitative abnormalities in retinal blood vessels (BVs). Optical coherence tomography (OCT) is suitable for examining the integrity of the anterior visual pathways in MS. In this paper we have compared the size and number of retinal blood vessels in patients with MS, with and without a history of optic neuritis (ON), and control subjects from the cross-sectional retinal images from OCT. Blood vessel diameter (BVD), blood vessel number (BVN), and retinal nerve fiber layer thickness (RNFLT) were extracted from OCT images collected from around the optic nerves of 129 eyes (24 control, 24 MS + ON, 81 MS-ON) of 71 subjects. Associations between blood vessel metrics, MS diagnosis, MS disability, ON, and RNFLT were evaluated using generalized estimating equation (GEE) models. MS eyes had a lower total BVD and BVN than control eyes. The effect was more pronounced with increased MS disability, and persisted in multivariate models adjusting for RNFLT and ON history. Twenty-nine percent (29%) of MS subjects had fewer retinal blood vessels than all control subjects. MS diagnosis, disability, and ON history were not associated with average blood vessel size. The relationship between MS and lower total BVD/BVN is not accounted for by RNFLT or ON. Further study is needed to determine the relationship between OCT blood vessel metrics and qualitative retinal blood vessel abnormalities in MS.

Update on the evaluation of transient vision loss.

Transient vision loss may indicate underlying vascular disease, including carotid occlusion and thromboembolism, or it may have a more benign etiology, such as migraine or vasospasm. This review focuses on the differential diagnosis and workup of patients presenting with transient vision loss, focusing on several key areas: the relationship to thromboembolic vascular disease, hypercoagulable testing, retinal migraine, and bilateral vision loss. The objective is to provide the ophthalmologist with information on how to best manage these patients. Thromboembolic etiologies for transient vision loss are sometimes managed with medications, but when carotid surgery is indicated, earlier intervention may prevent future stroke. This need for early treatment places the ophthalmologist in the important role of expediting the management process. Hospital admission is recommended in patients presenting with transient symptoms within 72 hours who meet certain high-risk criteria. When the cause is giant cell arteritis, ocular ischemic syndrome, or a cardioembolic source, early management of the underlying condition is equally important. For nonthromboembolic causes of transient vision loss such as retinal migraine or retinal vasospasm, the ophthalmologist can provide reassurance as well as potentially give medications to decrease the frequency of vision loss episodes.

CSF pressure, papilledema grade, and response to acetazolamide in the Idiopathic Intracranial Hypertension Treatment Trial.

Previous reports suggest an association between the degree of optic nerve head edema and CSF pressure (CSFp) in idiopathic intracranial hypertension (IIH). We hypothesized that CSFp would be associated with Frisén papilledema grade (FPG) and other clinical features, and that FPG would modify the CSFp response to acetazolamide in participants in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). In the IIHTT, eligible patients underwent lumbar puncture (LP) prior to enrollment and were randomly assigned to one of two treatment groups: acetazolamide plus supervised diet or placebo plus supervised diet. Trial eligibility required baseline CSFp ≥250 mm H2O or ≥200 mm H2O with compelling clinical or imaging IIH findings. Associations between CSFp and FPG and other clinical features were examined at baseline. The effect of acetazolamide on 6-month change in CSFp was examined in those with low FPG (grades I-III) and those with high FPG (grades IV-V) at baseline. All 165 enrolled subjects had a baseline LP and 85 had an LP at 6 months. There was an association between CSFp and FPG at baseline: CSFp was more elevated in subjects with high FPG (378 ± 90 mm H2O, n = 50) than in subjects with low FPG (331 ± 77, n = 115, p = 0.002). At 6 months, acetazolamide had a similar effect on CSFp in subjects with high FPG (-79.9 mm H2O) and in subjects with low FPG (-50.9 mm H2O, p = 0.50). We found a modest association between CSFp and FPG. Acetazolamide had a beneficial effect on CSFp regardless of baseline FPG.

Using the Theories of Joseph Babinski to Manage Functional Vision Loss.

OBJECTIVE: To report our use of persuasion to treat patients with functional vision loss and to place them in the context of both Joseph Babinski's theories on hysteria and the current literature on management of functional vision loss. BACKGROUND: Conversion disorders, such as functional vision loss, can develop in response to a traumatic event. Recent functional imaging studies have found that, rather than being malingerers, patients with conversion disorders show changes within neuromodulatory pathways, suggesting organic dysfunction rather than a purely psychological disorder. METHODS: We conducted a retrospective analysis of 8 consecutive patients (7 female, 1 male; mean age 16 years) who had presented to a university-based neuro-ophthalmology clinic with vision loss from a conversion disorder. The patients had been given a lengthy visual task to perform at home, with the suggestion that their vision would improve by the time they completed the task. RESULTS: All 8 patients had evidence of a dissociation between real and perceived visual acuity and fields. All the patients performed the assigned visual task at home, and all either reported improvement or showed improved visual function at follow-up. CONCLUSIONS: A century after Joseph Babinski proposed using persuasion in managing patients with vision loss from a conversion disorder, we report that his method remains a viable and pathophysiologically sound option. Future case-controlled studies that include functional magnetic resonance imaging, other neurophysiologic imaging, and neuroradiographic evaluation may strengthen the correlation between persuasion therapy and recovery of normal vision.

Further validation of the Six-Spot Step Test as a measure of ambulation in multiple sclerosis.

BACKGROUND: There is preliminary evidence regarding the validity of the Six-Spot Step Test (SSST) as a promising measure of ambulatory function in persons with multiple sclerosis (MS). To date, this assessment has not been subject to the same rigor and extent of psychometric evaluation as other widely-accepted measures of ambulatory (e.g., timed 25-foot walk (T25FW)). OBJECTIVE: This study aimed to provide additional validity evidence for the SSST in 96 persons with MS, based on construct validity and precision. Construct validity involves examining the pattern of associations between the SSST and other measures, and precision involves comparing SSST performance relative to other valid measures of ambulation for differentiating between levels of disability status, MS clinical course, and fall risk based on balance confidence. METHODS: All participants completed the SSST, T25FW, Timed Up-and-Go (TUG), six-minute walk, Multiple Sclerosis Walking Scale-12, Late-Life Function and Disability Inventory, Activities-specific Balance Confidence, and Paced Auditory Serial Addition Test. All participants further underwent a neurological examination for generating EDSS scores, and then wore an ActiGraph accelerometer for the waking hours of a 7-day period. RESULTS: SSST performance was strongly associated with other valid measures of ambulation (|ρ| = .65-.90) and disability status (ρ = .73), moderately-to-strongly associated with balance confidence (ρ = .58), and weakly-to-moderately associated with cognitive processing speed and non-ambulatory measures (|ρ| = .35-.39). The SSST demonstrated stronger relative precision in discriminating between levels of disability status, MS clinical course, and fall risk based on balance confidence than the T25FW and TUG. CONCLUSIONS: We provide comprehensive validity evidence for the SSST that supports its consideration for inclusion alongside other highly-regarded objective measures of ambulatory function for clinical research and practice in persons with MS.

Silent sinus syndrome.

PURPOSE OF REVIEW: Symptoms of diplopia, abnormal eyelid signs (retraction, ptosis, absent crease), ocular asymmetry (hypoglobus, enophthalmos), and dry eyes from lagophthalmos could all indicate the diagnosis of silent sinus syndrome. It is important for the physician to be updated on this relatively rare clinical entity. RECENT FINDINGS: Silent sinus syndrome lies on a spectrum of other forms of sinus-related orbitopathy. Uncommon presentations and descriptions of sinus involvement other than the maxillary sinus add to the variability of presentation. Radiographic features of disease include an opacified and hypoplastic sinus, a lateralized uncinate process, depression of the orbital floor, and a blocked osteomeatal complex. Management of silent sinus syndrome may include a single or multistage surgical approach, or a nonsurgical approach. SUMMARY: This review discusses silent sinus syndrome from a neuro-ophthalmological perspective.

Longitudinally extensive optic neuritis in neuromyelitis optica spectrum disorder.

BACKGROUND: Neuomyelitis optica, sarcoid, and multiple sclerosis can all cause optic neuritis. Further means of distinguishing the causes of optic neuritis among these etiologies would be valuable for the clinician. METHODS: This is a retrospective, cohort study from a single university based hospital and neuro-ophthalmology clinic. Blinded interpretation of orbit MRIs was performed on patients with acute optic neuritis from multiple sclerosis (n=25), sarcoid (n=5) and neuromyelitis optica spectrum disorder (n=6). RESULTS: A length of >40 mm anterior visual pathway enhancement distinguished neuromyelitis optica spectrum disorder from multiple sclerosis (p=0.0376). No statistically significant differences were found for presence of pain or papillitis, however there was a trend for bilateral involvement and chiasmal involvement in neuromyelitis optica spectrum disorder compared to multiple sclerosis. CONCLUSIONS: In acute optic neuritis, enhancing anterior visual pathway lesion length >40 mm helps differentiate neuromyelitis optica spectrum disorder from multiple sclerosis. This degree of involvement can be considered longitudinally extensive optic neuritis. Further characterization is necessary as this degree of enhancement occurs in other clinical syndromes besides neuromyelitis optica.

Leg spasticity and ambulation in multiple sclerosis.

Background. Spasticity of the legs is common in multiple sclerosis (MS), but there has been limited research examining its association with ambulatory outcomes. Objective. This study examined spasticity of the legs and its association with multiple measures of ambulation in persons with MS. Methods. The sample included 84 patients with MS. Spasticity of the legs was measured using a 5-point rating scale ranging between 0 (normal) and 4 (contracted). Patients completed the 6-minute walk (6 MW), timed 25 foot walk (T25FW), and timed up-and-go (TUG), and O2 cost of walking was measured during the 6 MW. The patients undertook two walking trials on a GAITRite (CIR systems, Inc.) for measuring spatial and temporal parameters of gait. The patients completed the Multiple Sclerosis Walking Scale-12 (MSWS-12) and wore an accelerometer over a seven-day period. Results. 52% (n = 44) of the sample presented with spasticity of the legs. Those with leg spasticity had significantly worse ambulation as measured by 6 MW (P = 0.0001, d = -0.86), T25FW (P = 0.003, d = 0.72), TUG (P = 0.001, d = 0.84), MSWS-12 (P = 0.0001, d = 1.09), O2 cost of walking (P = 0.001, d = 0.75), average steps/day (P < 0.05, d = -0.45), and walking velocity (P < 0.05, d = -0.53) and cadence (P < 0.05, d = -0.46). Conclusion. Leg spasticity was associated with impairments in ambulation, including alterations in spatiotemporal parameters and free-living walking.