The Use of the Saccadometer to Identify Saccadic Characteristics in Myasthenia Gravis: A Pilot Study.

BACKGROUND: Myasthenia gravis (MG) often presents with ocular signs that mimic other forms of ocular defects, such as isolated cranial nerve palsy. Normal velocity or even hyperfast saccadic eye movements in the presence of deficits of smooth pursuit have been well described in the literature in myasthenic patients. The reason for these paradoxical clinical findings has been reported to be due to increased postsynaptic folding of the fast-twitch fibers responsible for the execution of a saccade which is absent in those fibers responsible for slower, smooth eye movement. Saccadic characteristics therefore offer a point of differential diagnosis between patients suspected of having ocular motility deficits as a result of MG and those caused by other neuropathies. The advent of portable quantitative saccadic assessment means that previously laboratory-based assessments that require specialist equipment and training may now be undertaken clinically, providing a noninvasive test that can aid the differential diagnosis of the condition. The aim of this pilot study was to investigate the feasibility of the saccadometer (Ober Consulting, Poznan, Poland) in detecting the saccadic characteristics associated with myasthenia, specifically normal peak velocity (PV) in a group of patients confirmed with myasthenia. METHODS: A group of 5 patients with a confirmed diagnosis of MG were recruited from a single site into the study along with 5 age-matched healthy volunteers. All myasthenic patients had ocular signs such as underaction or limitations of motility confirmed through ocular clinical examination. Healthy volunteers were screened for any underlying ocular motility or neurological defects before inclusion within the study. All participants undertook 100 trials of both 10 and 20° amplitude saccades, and mean PV, amplitude, and latency were recorded using the saccadometer for each individual. Overall, mean PV, amplitude, and latency were collated for both myasthenic and healthy control groups for each saccade size and compared. RESULTS: The mean PV was significantly greater (481 ± 103.5 deg/seconds) for myasthenic patients compared with healthy controls (384 ± 42.8 deg/seconds) (P < 0.05) in 10° saccades. PV was also greater in myasthenics for 20° saccades; however, this difference did not reach statistical significance for patients with MG (547 ± 89.8 deg/seconds vs 477 ± 104.5 deg/seconds) (P = 0.14). The latency of participants with MG was not significantly different from those of age-matched healthy participants in 10° saccades but was significantly different for 20° saccades. There was no difference in amplitude measured between the groups. CONCLUSIONS: PV for both 10 and 20° saccades was greater in myasthenic patients compared with healthy controls. All myasthenic patients produced normal velocity saccades in the presence of deficits of smooth ocular motility. The results from this small pilot study demonstrate the potential use of the saccadometer in a clinical setting to provide a noninvasive aid in the diagnosis of patients suspected with myasthenia.

Interventions for eye movement disorders due to acquired brain injury.

BACKGROUND: Acquired brain injury can cause eye movement disorders which may include: strabismus, gaze deficits and nystagmus, causing visual symptoms of double, blurred or 'juddery' vision and reading difficulties. A wide range of interventions exist that have potential to alleviate or ameliorate these symptoms. There is a need to evaluate the effectiveness of these interventions and the timing of their implementation. OBJECTIVES: We aimed to assess the effectiveness of any intervention and determine the effect of timing of intervention in the treatment of strabismus, gaze deficits and nystagmus due to acquired brain injury. We considered restitutive, substitutive, compensatory or pharmacological interventions separately and compared them to control, placebo, alternative treatment or no treatment for improving ocular alignment or motility (or both). SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (containing the Cochrane Eyes and Vision Trials Register) (2017, Issue 5), MEDLINE Ovid, Embase Ovid, CINAHL EBSCO, AMED Ovid, PsycINFO Ovid, Dissertations & Theses (PQDT) database, PsycBITE (Psychological Database for Brain Impairment Treatment Efficacy), ISRCTN registry, ClinicalTrials.gov, Health Services Research Projects in Progress (HSRProj), National Eye Institute Clinical Studies Database and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). The databases were last searched on 26 June 2017. No date or language restrictions were used in the electronic searches for trials. We manually searched the Australian Orthoptic Journal, British and Irish Orthoptic Journal, and ESA, ISA and IOA conference proceedings. We contacted researchers active in this field for information about further published or unpublished studies. SELECTION CRITERIA: We included randomised controlled trials (RCTs) of any intervention for ocular alignment or motility deficits (or both) due to acquired brain injury. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies and extracted data. We used standard methods expected by Cochrane. We employed the GRADE approach to interpret findings and assess the quality of the evidence. MAIN RESULTS: We found five RCTs (116 participants) that were eligible for inclusion. These trials included conditions of acquired nystagmus, sixth cranial nerve palsy and traumatic brain injury-induced ocular motility defects. We did not identify any relevant studies of restitutive interventions.We identified one UK-based trial of a substitutive intervention, in which botulinum toxin was compared with observation in 47 people with acute sixth nerve palsy. At four months after entry into the trial, people given botulinum toxin were more likely to make a full recovery (reduction in angle of deviation within 10 prism dioptres), compared with observation (risk ratio 1.19, 95% CI 0.96 to 1.48; low-certainty evidence). These same participants also achieved binocular single vision. In the injection group only, there were 2 cases of transient ptosis out of 22 participants (9%), and 4 participants out of 22 (18%) with transient vertical deviation; a total complication rate of 24% per injection and 27% per participant. All adverse events recovered. We judged the certainty of evidence as low, downgrading for risk of bias and imprecision. It was not possible to mask investigators or participants to allocation, and the follow-up between groups varied.We identified one USA-based cross-over trial of a compensatory intervention. Oculomotor rehabilitation was compared with sham training in 12 people with mild traumatic brain injury, at least one year after the injury. We judged the evidence from this study to be very low-certainty. The study was small, data for the sham training group were not fully reported, and it was unclear if a cross-over study design was appropriate as this is an intervention with potential to have a permanent effect.We identified three cross-over studies of pharmacological interventions for acquired nystagmus, which took place in Germany and the USA. These studies investigated two classes of pharmacological interventions: GABAergic drugs (gabapentin, baclofen) and aminopyridines (4-aminopyridines (AP), 3,4-diaminopyridine (DAP)). We judged the evidence from all three studies as very low-certainty because of small numbers of participants (which led to imprecision) and risk of bias (they were cross-over studies which did not report data in a way that permitted estimation of effect size).One study compared gabapentin (up to 900 mg/day) with baclofen (up to 30 mg/day) in 21 people with pendular and jerk nystagmus. The follow-up period was two weeks. This study provides very low-certainty evidence that gabapentin may work better than baclofen in improving ocular motility and reducing participant-reported symptoms (oscillopsia). These effects may be different in pendular and jerk nystagmus, but without formal subgroup analysis it is unclear if the difference between the two types of nystagmus was chance finding. Quality of life was not reported. Ten participants with pendular nystagmus chose to continue treatment with gabapentin, and one with baclofen. Two participants with jerk nystagmus chose to continue treatment with gabapentin, and one with baclofen. Drug intolerance was reported in one person receiving gabapentin and in four participants receiving baclofen. Increased ataxia was reported in three participants receiving gabapentin and two participants receiving baclofen.One study compared a single dose of 3,4-DAP (20 mg) with placebo in 17 people with downbeat nystagmus. Assessments were made 30 minutes after taking the drug. This study provides very low-certainty evidence that 3,4-DAP may reduce the mean peak slow-phase velocity, with less oscillopsia, in people with downbeat nystagmus. Three participants reported transient side effects of minor perioral/distal paraesthesia.One study compared a single dose of 4-AP with a single dose of 3,4-DAP (both 10 mg doses) in eight people with downbeat nystagmus. Assessments were made 45 and 90 minutes after drug administration. This study provides very low-certainty evidence that both 3,4-DAP and 4-AP may reduce the mean slow-phase velocity in people with downbeat nystagmus. This effect may be stronger with 4-AP. AUTHORS' CONCLUSIONS: The included studies provide insufficient evidence to inform decisions about treatments specifically for eye movement disorders that occur following acquired brain injury. No information was obtained on the cost of treatment or measures of participant satisfaction relating to treatment options and effectiveness. It was possible to describe the outcome of treatment in each trial and ascertain the occurrence of adverse events.

Botulinum toxin for the treatment of strabismus.

BACKGROUND: The use of botulinum toxin as an investigative and treatment modality for strabismus is well reported in the medical literature. However, it is unclear how effective it is in comparison to other treatment options for strabismus. OBJECTIVES: The primary objective was to examine the efficacy of botulinum toxin therapy in the treatment of strabismus compared with alternative conservative or surgical treatment options. This review sought to ascertain those types of strabismus that particularly benefit from the use of botulinum toxin as a treatment option (such as small angle strabismus or strabismus with binocular potential, i.e. the potential to use both eyes together as a pair). The secondary objectives were to investigate the dose effect and complication rates associated with botulinum toxin. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Trials Register) (2016, Issue 6), Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid MEDLINE Daily, Ovid OLDMEDLINE (January 1946 to July 2016), Embase (January 1980 to July 2016), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to July 2016), the ISRCTN registry (www.isrctn.com/editAdvancedSearch), ClinicalTrials.gov (www.clinicaltrials.gov), and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). We did not use any date or language restrictions in the electronic searches for trials. We last searched the electronic databases on 11 July 2016. We handsearched the British and Irish Orthoptic Journal, Australian Orthoptic Journal, proceedings of the European Strabismological Association (ESA), International Strabismological Association (ISA) and International Orthoptic Association (IOA) (www.liv.ac.uk/orthoptics/research/search.htm) and American Academy of Paediatric Ophthalmology and Strabismus meetings (AAPOS). We contacted researchers who are active in this field for information about further published or unpublished studies. SELECTION CRITERIA: We included randomised controlled trials (RCTS) of any use of botulinum toxin treatment for strabismus. DATA COLLECTION AND ANALYSIS: Two review authors independently selected studies and extracted data. We used standard methods expected by Cochrane and assessed the certainty of the evidence using GRADE. We defined ocular alignment as an angle of deviation of less than or equal to 10 prism dioptres. MAIN RESULTS: Six RCTs were eligible for inclusion. We judged the included studies as at a mixture of low, unclear and high risk of bias. We did not consider any of the included studies as at low risk of bias for all domains.Two trials conducted in Spain (102 people, number of eyes not specified) compared botulinum toxin with surgery in children that required retreatment for acquired or infantile esotropia. These two studies provided low-certainty evidence that children who received botulinum toxin may have a similar or slightly reduced chance of achieving ocular alignment (pooled risk ratio (RR) 0.91, 95% confidence interval (CI) 0.71 to 1.16), binocular single vision (RR 0.88, 95% CI 0.63 to 1.23), sensory fusion (RR 0.88, 95% CI 0.63 to 1.23) and stereopsis (RR 0.86, 95% CI 0.59 to 1.25) compared with children who received surgery. One trial from Canada compared botulinum toxin with surgery in 30 adults (30 eyes) with horizontal strabismus and reported a reduced chance of ocular alignment with botulinum toxin (RR 0.38, 95% CI 0.17 to 0.85; low-certainty evidence).One trial in the UK suggested that botulinum toxin may result in a similar or slightly improved chance of ocular alignment in people with acute onset sixth nerve palsy compared with observation (RR 1.19, 95% CI 0.96 to 1.48; 47 participants, low-certainty evidence).Very low-certainty evidence from one trial from Brazil suggested that adjuvant botulinum toxin in strabismus surgery may increase the chances of ocular alignment compared with strabismus surgery alone (RR 1.83, 95% CI 0.41 to 8.11; 23 participants).One trial from China of 47 participants (94 eyes) suggested that people receiving botulinum toxin combined with sodium hyaluronate may have a similar or slightly reduced chance of achieving ocular alignment compared with botulinum toxin alone (RR 0.81, 95% CI 0.36 to 1.82; low-certainty evidence).Reported complications in people given botulinum toxin in the included trials included ptosis (range 9% to 41.66%) and vertical deviation (range 8.3% to 18.51%). Ptosis occurred less frequently when treated with botulinum toxin combined with sodium hyaluronate compared to botulinum toxin alone. AUTHORS' CONCLUSIONS: Most published literature on the use of botulinum toxin in the treatment of strabismus consists of retrospective studies, cohort studies or case reviews. Although these provide useful descriptive information, clarification is required as to the effective use of botulinum toxin as an independent treatment modality. Six RCTs on the therapeutic use of botulinum toxin in strabismus, graded as low and very low-certainty evidence, have shown varying responses. These include a lack of evidence for effect of botulinum toxin on reducing visual symptoms in acute sixth nerve palsy, poor response in people with horizontal strabismus without binocular vision, similar or slightly reduced achievement of successful ocular alignment in children with esotropia and potential increased achievement of successful ocular alignment where surgery and botulinum toxin are combined. Further high quality trials using robust methodologies are required to compare the clinical and cost effectiveness of various forms of botulinum toxin (e.g. Dysport, Xeomin, etc), to compare botulinum toxin with and without adjuvant solutions and to compare botulinum toxin to alternative surgical interventions in strabismus cases with and without potential for binocular vision.

A Randomised Controlled Trial of Treatment for Post-Stroke Homonymous Hemianopia: Screening and Recruitment.

The authors report the screening process and recruitment figures for the VISION (Visual Impairment in Stroke; Intervention Or Not) trial. This is a prospective, randomised, single-blinded, three-arm controlled trial in 14 UK acute hospital stroke units. Stroke teams identified stroke survivors suspected as having homonymous hemianopia. Interventions included Fresnel prisms versus visual search training versus standard care (information only). Primary outcome was change in visual field assessment from baseline to 26 weeks. Secondary measures included change in quality-of-life questionnaires. Recruitment opened in May 2011. A total of 1171 patients were screened by the local principal investigators. Of 1171 patients, 178 (15.2%) were eligible for recruitment: 87 patients (7.4%) provided consent and were recruited; 91 patients (7.8%) did not provide consent, and 993 of 1171 patients (84.8%) failed to meet the eligibility criteria. Almost half were excluded due to complete/partial recovery of hemianopia (43.6%; n = 511). The most common ineligibility reason was recovery of hemianopia. When designing future trials in this area, changes in eligibility criteria/outcome selection to allow more patients to be recruited should be considered, e.g., less stringent levels of visual acuity/refractive error. Alternative outcomes measurable in the home environment, rather than requiring hospital attendance for follow-up, could facilitate increased recruitment.

Care provision for poststroke visual impairment.

BACKGROUND: We sought to explore the care provision for poststroke visual impairment and variations in this in the United Kingdom. METHODS: Survey questions were developed and piloted with clinicians, academics, and users. Questions addressed types of visual problems; how these were identified, treated, and followed up; care pathways in use; links with other professions; and referral options. The survey was accessed via a Weblink, which was circulated through UK professional organizations to multiprofessional members of ophthalmic and stroke teams. RESULTS: A total of 548 completed electronic surveys were obtained. In all, 49.5% of respondents represented stroke teams, 42.5% eye teams, and 8% from other teams, for example, emergency care. Many respondents (41%) saw patients within 1 week of stroke. Nineteen percent did not personally test vision: 11% had a visiting clinician to test vision, and 22% used screening tools. Validated tests were used for the assessment of visual acuity (39.5%), visual field (57.5%), eye movement (48.5%), and visual function (58.5%). Visual problems suspected by family or professionals were high (88.5%). Typical overall follow-up period of vision care was less than 3 months. In all, 46% of respondents used designated care pathways for stroke survivors with visual problems; 33.5% of respondents did not provide visual information leaflets. CONCLUSIONS: Significant inequality exists in care for stroke survivors who experience visual problems. There is great variability in how vision screening is undertaken, which vision tests are used, methods of referral to eye care services, how visual problems are managed, and what vision information is provided to stroke survivors/carers. Further work is required to ensure equality and effective care.

Detection of Visual Field Loss in Pituitary Disease: Peripheral Kinetic Versus Central Static.

Visual field assessment is an important clinical evaluation for eye disease and neurological injury. We evaluated Octopus semi-automated kinetic peripheral perimetry (SKP) and Humphrey static automated central perimetry for detection of neurological visual field loss in patients with pituitary disease. We carried out a prospective cross-sectional diagnostic accuracy study comparing Humphrey central 30-2 SITA threshold programme with a screening protocol for SKP on Octopus perimetry. Humphrey 24-2 data were extracted from 30-2 results. Results were independently graded for presence/absence of field defect plus severity of defect. Fifty patients (100 eyes) were recruited (25 males and 25 females), with mean age of 52.4 years (SD = 15.7). Order of perimeter assessment (Humphrey/Octopus first) and order of eye tested (right/left first) were randomised. The 30-2 programme detected visual field loss in 85%, the 24-2 programme in 80%, and the Octopus combined kinetic/static strategy in 100% of eyes. Peripheral visual field loss was missed by central threshold assessment. Qualitative comparison of type of visual field defect demonstrated a match between Humphrey and Octopus results in 58%, with a match for severity of defect in 50%. Tests duration was 9.34 minutes (SD = 2.02) for Humphrey 30-2 versus 10.79 minutes (SD = 4.06) for Octopus perimetry. Octopus semi-automated kinetic perimetry was found to be superior to central static testing for detection of pituitary disease-related visual field loss. Where reliant on Humphrey central static perimetry, the 30-2 programme is recommended over the 24-2 programme. Where kinetic perimetry is available, this is preferable to central static programmes for increased detection of peripheral visual field loss.

Losing sight under pressure.

A non-communicative patient with vascular dementia who was admitted to hospital with non-specific symptoms. Ophthalmic emergencies are rare, however they should be considered as part of a systemic work-up in unexplained non-specific presentations, particularly in patients who are not able to communicate as in the case we present here.

Botulinum toxin for the treatment of strabismus.

BACKGROUND: The use of botulinum toxin as an investigative and treatment modality for strabismus is well reported in the medical literature. However it is unclear how effective its use is in comparison to other treatment options for strabismus. OBJECTIVES: To evaluate the efficacy of botulinum toxin in the treatment of strabismus compared with alternative treatment options, to investigate dose effect and complication rates. SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2011, Issue 11), MEDLINE (January 1950 to December 2011), EMBASE (January 1980 to December 2011), Latin American and Caribbean Literature on Health Sciences (LILACS) (January 1982 to December 2011), the metaRegister of Controlled Trials (mRCT) (www.controlled-trials.com), ClinicalTrials.gov (www.clinicaltrials.gov) and the WHO International Clinical Trials Registry Platform (ICTRP) (www.who.int/ictrp/search/en). There were no date or language restrictions in the electronic searches for trials. The electronic databases were last searched on 5 December 2011. We manually searched the Australian Orthoptic Journal and British and Irish Orthoptic Journal and ESA, ISA and IOA conference proceedings. We attempted to contact researchers who are active in this field for information about further published or unpublished studies. SELECTION CRITERIA: We included randomised controlled trials (RCTS) of any use of botulinum toxin treatment for strabismus. DATA COLLECTION AND ANALYSIS: Each review author independently assessed study abstracts identified from the electronic and manual searches. Author analysis was then compared and full papers for appropriate studies were obtained. MAIN RESULTS: We found four RCTs that were eligible for inclusion. Two trials found that there was no difference between the use of botulinum toxin and surgery for patients requiring retreatment for acquired esotropia or infantile esotropia. There was no evidence for a prophylactic effect of botulinum toxin in a treatment trial of acute onset sixth nerve palsy. Botulinum toxin had a poorer response than surgery in a trial of patients requiring treatment for horizontal strabismus in the absence of binocular vision. Reported complications included ptosis and vertical deviation and ranged from 24% in a trial using Dysport™ to 52.17% and 55.54% in trials using Botox™. AUTHORS' CONCLUSIONS: The majority of published literature on the use of botulinum toxin in the treatment of strabismus consists of retrospective studies, cohort studies or case reviews. Although these provide useful descriptive information, clarification is required as to the effective use of botulinum toxin as an independent treatment modality. Four RCTs on the therapeutic use of botulinum toxin in strabismus have shown varying responses ranging from a lack of evidence for prophylactic effect of botulinum toxin in acute sixth nerve palsy, to poor response in patients with horizontal strabismus without binocular vision, to no difference in response in patients that required retreatment for acquired esotropia or infantile esotropia. It was not possible to establish dose effect information. Complication rates for use of Botox™ or Dysport™ ranged from 24% to 55.54%.

Botulinum toxin for the treatment of strabismus.

BACKGROUND: The use of botulinum toxin as an investigative and treatment modality for strabismus is well reported in the medical literature. However it is unclear how effective its use is in comparison to other treatment options for strabismus. OBJECTIVES: To evaluate the efficacy of botulinum toxin in the treatment of strabismus compared with alternative treatment options, to investigate dose effect and complication rates. SEARCH STRATEGY: We searched CENTRAL, MEDLINE, EMBASE and LILACS up to 15 December 2008. We manually searched the Australian Orthoptic Journal and British and Irish Orthoptic Journal and ESA, ISA and IOA conference proceedings. We attempted to contact researchers who are active in this field for information about further published or unpublished studies. SELECTION CRITERIA: We included randomised controlled trials (RCTS) of any use of botulinum toxin treatment for strabismus. DATA COLLECTION AND ANALYSIS: Each review author independently assessed study abstracts identified from the electronic and manual searches. Author analysis was then compared and full papers for appropriate studies were obtained. MAIN RESULTS: We found four RCTs that were eligible for inclusion. Two trials found that there was no difference between the use of botulinum toxin and surgery for patients requiring retreatment for acquired esotropia or infantile esotropia. There was no evidence for a prophylactic effect of botulinum toxin in a treatment trial of acute onset sixth nerve palsy. Botulinum toxin had a poorer response than surgery in a trial of patients requiring treatment for horizontal strabismus in the absence of binocular vision. Reported complications included ptosis and vertical deviation and ranged from 24% in a trial using Dysport to 52.17% and 55.54% in trials using Botox. AUTHORS' CONCLUSIONS: The majority of published literature on the use of botulinum toxin in the treatment of strabismus consists of retrospective studies, cohort studies or case reviews. Although these provide useful descriptive information, clarification is required as to the effective use of botulinum toxin as an independent treatment modality. Four RCTs on the therapeutic use of botulinum toxin in strabismus have shown varying responses ranging from a lack of evidence for prophylactic effect of botulinum toxin in acute sixth nerve palsy, to poor response in patients with horizontal strabismus without binocular vision, to no difference in response in patients that required retreatment for acquired esotropia or infantile esotropia. It was not possible to establish dose effect information. Complication rates for use of Botox or Dysport ranged from 24% to 55.54%.

Accuracy of kinetic perimetry assessment with the Humphrey 850; an exploratory comparative study.

AIM: To compare kinetic perimetry on the Humphrey 850 and Octopus 900 perimeters for assessment of visual fields, uniocular rotations and fields of binocular single vision. METHODS: Prospective cross section study comparing Humphrey 850 kinetic perimetry to kinetic perimetry using the Octopus 900. Results were compared for both perimeters for the measurement of visual field boundaries, uniocular rotations and fields of binocular single vision in subjects with normal visual function, with comparisons of mean vector extremity values and duration of testing. A visual field boundary overlay was used to assess detection potential of Humphrey 850 kinetic perimetry using I4e and I2e targets in results of known abnormal visual fields. RESULTS: Fifteen subjects (30 eyes) with normal parameters of visual function underwent dual perimetry assessment. Mean visual field boundaries and ocular rotation extremity values were similar for Humphrey and Octopus kinetic perimetry along horizontal meridians. Measurements for Humphrey perimetry were significantly smaller for superior and inferior visual field and rotations with ceiling effects at approximately 40 and 50 degrees, respectively. Use of visual field boundary overlays for 140 patient results showed high detection of the known abnormal visual field results by the Humphrey 850 perimeter (91.4% with I4e target; 95% with I2e target) but with notable exceptions for peripheral superior visual field defects. CONCLUSIONS: The Humphrey perimeter's aspheric bowl introduces a ceiling effect for measurements in the superior and inferior visual field at approximately 40 and 50 degrees respectively. This results in potential diagnostic accuracy issues when measuring uniocular rotations, fields of binocular single and visual field boundaries in conditions that specifically impair superior and/or inferior ocular motility (e.g., thyroid eye disease) or visual fields (e.g., chiasmal compression).

Neuromyelitis optica spectrum disorders.

Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with high-dose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immunosuppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD.

Visual Function Questionnaire as an outcome measure for homonymous hemianopia: subscales and supplementary questions, analysis from the VISION trial.

BACKGROUND: We conduct supplementary analyses of the NEI VFQ-25 data to evaluate where changes occurred within subscales of the NEI VFQ-25 leading to change in the composite scores between the three treatment arms, and evaluate the NEI VFQ-25 with and without the Neuro 10 supplement. METHODS: A prospective, multicentre, parallel, single-blind, three-arm RCT of fourteen UK acute stroke units was conducted. Stroke survivors with homonymous hemianopia were recruited. Interventions included: Fresnel prisms for minimum 2 h, 5 days/week over 6-weeks (Arm a), Visual search training for minimum 30 min, 5 days/week over 6-weeks (Arm b) and standard care-information only (Arm c). Primary and secondary outcomes (including NEI VFQ-25 data) were measured at baseline, 6, 12 and 26 weeks after randomisation. RESULTS: Eighty seven patients were recruited (69% male; mean age (SD) equal to 69 (12) years). At 26 weeks, outcomes for 24, 24 and 22 patients, respectively, were compared to baseline. NEI VFQ-25 (with and without Neuro 10) responses improved from baseline to 26 weeks with visual search training compared to Fresnel prisms and standard care. In subscale analysis, the most impacted across all treatment arms was 'driving' whilst the least impacted were 'colour vision' and 'ocular pain'. CONCLUSIONS: Composite scores differed systematically for the NEI VFQ-25 (Neuro 10) versus NEI VFQ-25 at all time points. For subscale scores, descriptive statistics suggest clinically relevant improvement in distance activities and vision-specific dependency subscales for NEI VFQ-25 scores in the visual search treatment arm. TRIAL REGISTRATION: Current Controlled Trials ISRCTN05956042.

Bilateral combined resection and recession of the medial rectus muscle for convergence excess esotropia.

Five patients with true convergence excess esotropia were surgically managed with combined resection and recession of the medial rectus muscles. This technique was chosen to address the near/distance angle disparity. The use of this surgical technique produced full asymptomatic binocular control at near and distance fixation in four patients. One patient developed a consecutive exotropia but regained full binocular control following a second surgical procedure.

Surgical treatment for progressive esotropia in the setting of high-axial myopia.

Acquired esotropia in association with high myopia may be secondary to displacement of the lateral rectus muscle pulley system or to the mechanical restriction due to the contact between the posterior elongated globe and the orbital apex limiting rotation. We present the clinical findings and surgical management of a patient with this condition.

Bilateral optic disc edema as a presentation of an obstructing spinal plasmacytoma.

PURPOSE: To report a rare case of bilateral optic disc edema as presentation of an obstructing spinal plasmacytoma. METHODS: Case report. RESULTS: A 41-year-old healthy man presented with distortion of his peripheral vision for 9 months. He denied headaches or neurologic symptoms. Examination showed bilateral optic disc swelling, radial disc hemorrhages, and absent spontaneous venous pulsations. Brain magnetic resonance imaging (MRI) and magnetic resonance venography were unrevealing. Lumbar puncture showed a normal opening pressure of 19 cm cerebrospinal fluid (CSF). CSF protein was significantly elevated at 3.22 g/L (0.10-0.45 g/L). Spinal MRI with contrast revealed a tumor in the T9 vertebral body extending through the disc spaces into T8 and T10, compressing the spinal cord. Computed tomography-guided biopsy confirmed a spinal plasmacytoma. He received radiotherapy to the spine T7-T11 to reduce spinal cord compression followed by a 6-month course of chemotherapy with dexamethasone, cyclophosphamide, and thalidomide. Disc swelling improved on starting radiotherapy with complete resolution 8 months posttreatment. MRI spine showed reduction of tumor. CONCLUSIONS: This case highlights the importance of spinal imaging in patients with normal cranial scans and raised CSF protein levels who lack the typical idiopathic intracranial hypertension phenotype.

Delivery of high quality stroke and vision care: experiences of UK services.

PURPOSE: We sought to identify exemplars of high quality care provision from established stroke vision services. METHODS: We identified areas of high quality services across the UK, judged as having integrated stroke/vision care provision for stroke survivors. Healthcare professionals were selected to participate in 1:1 interviews or focus groups. A strengths-weaknesses-opportunities-threats (SWOT) framework was used to lead the discussion in a semi-structured format. Thematic analysis was undertaken. RESULTS: Interviewees (n = 24) from 14 NHS Trusts included eye clinic managers, nurses, orthoptists, occupational therapists and physiotherapists. Identified strengths of their services included established communication, training provision for stroke team staff, "open access" for referrals, use of standardised screening/referral forms, provision of lay summaries and information sheets, patients assessed on the stroke unit with continued follow-up and initial visual assessments made within 1 week of stroke onset. Weaknesses included lack of funding, insufficient orthoptic cover, and time consuming retraining of stroke staff because of staff rotation and changes. Opportunities included increasing the number (or length) of orthoptic sessions and training of stroke staff. Perceived threats related to funding and increased appointment waiting times. CONCLUSIONS: Practical elements for improved stroke and vision care provision are highlighted which can be implemented with relatively little financial inputs. Implications for Rehabilitation Integrated vision services within stroke units can improve the detection of visual problems in stroke survivors leading to earlier visual rehabilitation. Orthoptists within core stroke teams are beneficial to the delivery of a high quality service. This study illustrates clear practical elements to support the provision of high quality integrated stroke and vision services. Relatively little financial inputs are required to fund such services but with larger potential to improve patient care.

Combined optical and atropine penalization for the treatment of strabismic and anisometropic amblyopia.

INTRODUCTION: The treatment of amblyopia by occlusion of the fellow eye is beset by problems related to compliance, stigmatization, and regression of visual acuity (VA). Atropine or optical penalization has been used as an alternative treatment for amblyopia and might be synergistic as a combination therapy. Combined optical and atropine penalization treatment (COAT) was therefore assessed in patients with anisometropic and strabismic amblyopia. METHODS: Patients with a hypermetropic refractive error in whom occlusion had failed were prescribed daily atropine 1% and a plano spectacle lens to the fellow eye. Outcome measures included VA (LogMAR units), interocular VA difference, adverse events, and adverse reactions. COAT was continued until VA failed to improve after 2 consecutive visits, and then patients were offered occlusion treatment again. RESULTS: Forty-two patients (mean age, 4.73 yrs) were treated with COAT. Patients had attempted occlusion for a mean period of 36 weeks before commencement of COAT. The mean VA of the amblyopic eyes improved after 10 weeks of COAT, from 0.85 (20/113) to 0.28 (20/37) (P <.001), an overall success (doubling of VA) rate of 76%. There was no significant change in the mean VA of the fellow eye as a result of COAT (P =.13). Twenty-two patients recommenced occlusion after COAT for regression (10), lack of further improvement (2), or parental choice (10). The remaining 20 patients maintained their VA without treatment at 93% of post-COAT levels, at a mean follow-up 6.4 months. Overall regression rate was 36%, adverse reaction rate was 2%, adverse event rate was 21%, and presumed compliance rate was 83%. DISCUSSION: COAT is an effective treatment method when occlusion therapy initially fails. COAT is well tolerated and should be considered as an alternative and/or supportive therapy in the management of amblyopia.

Comparison of octopus semi-automated kinetic perimetry and humphrey peripheral static perimetry in neuro-ophthalmic cases.

Aim. To compare semikinetic perimetry (SKP) on Octopus 900 perimetry to a peripheral static programme with Humphrey automated perimetry. Methods. Prospective cross-section study comparing Humphrey full field (FF) 120 two zone programme to a screening protocol for SKP on Octopus perimetry. Results were independently graded for presence/absence of field defect plus type and location of defect. Results. 64 patients (113 eyes) underwent dual perimetry assessment. Mean duration of assessment for SKP was 4.54 minutes ±0.18 and 6.17 ± 0.12 for FF120 (P = 0.0001). 80% of results were correctly matched for normal or abnormal visual fields using the I4e target versus FF120, and 73.5% were correctly matched using the I2e target versus FF120. When comparing Octopus results with combined I4e and I2e isopters to the FF120 result, a match for normal or abnormal fields was recorded in 87%. Conclusions. Humphrey perimetry test duration was generally longer than Octopus SKP. In the absence of kinetic perimetry, peripheral static suprathreshold programme options such as FF120 may be useful for detection of visual field defects. However, statokinetic dissociation may occur. Octopus SKP utilising both I4e and I2e targets provides detailed information of both the defect depth and size and may provide a more representative view of the actual visual field defect.

The psychosocial effects of adult strabismus: a review.

Correction of adult strabismus is not a cosmetic procedure but one that restores normality to an individual's appearance that has been altered by a disease process. Two fundamental principles underpinning facial attractiveness are symmetry and averageness-manifest strabismus affects both of these giving an unconscious signal of poor genetic history. The presence of manifest strabismus adversely affects many aspects of patients' lives including finding a partner, job prospects and interaction with peers, and may manifest more seriously as psychiatric disorders. Surgical correction has been shown to be safe and effective for the functional problems of strabismus in adults but the hugely positive effects on the psychosocial aspects are only now becoming apparent. The advent of a new adult strabismus specific quality-of-life questionnaire and its subsequent validation will make this quantification of improvement easier. The wider medical community and the public at large should be made aware of the benefits of corrective strabismus surgery in adults.

Relatively spared central multifocal electroretinogram responses in acute quinine toxicity.

A 71-year-old man was investigated with electrodiagnostic testing 4 months after a deliberate quinine overdose. Initially he was admitted to intensive care unit with visual acuity (VA) of perception of light in both eyes. VA recovered to 6/6 right eye and 6/12 left eye, though severely constricted fields were noted. Slow stimulus (base period of 83 ms) multifocal electroretinogram (ERG) showed electronegative responses outside the inner 5 degrees, with a reduced but electropositive response seen in this central area. It appears that in this case of bilaterally negative ERGs that the macula/fovea (which has a vascular supply through the choroid) is relatively spared as is seen in bilateral vascular electronegative ERGs. This may indicate that quinine toxicity to the retina may be secondary to effects similar to vascular occlusion or severe ischemia during the acute phase of quinine poisoning.