Surgical treatment of unilateral acquired superior oblique muscle palsy in adults by inferior oblique belly transposition: a retrospective analysis.

PURPOSE: Evaluate and analyze the efficacy of inferior oblique belly transposition (IOBT) in treating adult patients with diplopia and small-angle hypertropia caused by mild to moderate inferior oblique overaction (IOOA) secondary to acquired superior oblique palsy (SOP). METHODS: Nine adult patients with diplopia and small-angle hypertropia associated with mild to moderate IOOA secondary to unilateral acquired SOP were included in the current retrospective study. All patients received the IOBT procedure between February 2019 and May 2023 at The Second People's Hospital of Jinan and were followed up for more than 6 months after the surgery. During the procedure, the belly of the inferior oblique muscle was fixed to the sclera at 5 mm posterior to the temporal insertion of the inferior rectus muscle. The following indicators were reviewed pre- and post-surgery: the vertical deviation (VD) in the primary position and in the Bielschowsky test, the fovea disc angle (FDA) of the affected eye, changes in IOOA, and diplopia. RESULTS: After IOBT, the VD in the primary position decreased from 7.22△ ± 1.72△ (range 4△-10△) to 1.22△ ± 1.30△ (range 0△-3△). The VD in the Bielschowsky test decreased from 13.00△ ± 1.80△ to 3.22△ ± 1.09△. The FDA decreased from 10.02° ± 3.34° to 6.26° ± 1.91°. The grade of IOOA was reduced from 2.00 (1.00, 2.00) to 0.00 (0.00, 1.00). All changes were statistically significant (P < 0.001 or P = 0.006). Diplopia was resolved completely for all patients. CONCLUSIONS: IOBT can effectively treat adults with diplopia and small-angle hypertropia caused by mild to moderate IOOA secondary to acquired SOP.

The Challenge of Strabismus Surgery for Patients with Hemianopic Visual Field Defects Associated with Ocular Deviations.

Homonymous and heteronymous hemianopias associated with strabismus are a therapeutic challenge because surgery may result in diplopia, decrease the visual field (VF), and/or lead to recurrence of the deviation. We present four cases: two homonymous hemianopias and two heteronymous hemianopias. Of the four patients, three had exotropia and one had esotropia. The origin of hemianopias was neoplastic in two cases and traumatic in two cases. Strabismus surgery was performed in three cases, but only one case had a good result; recurrence of the deviation and diplopia occurred in the other two cases. In conclusion, exotropia may develop as a compensatory mechanism in a patient with congenital or early-onset homonymous hemianopia and realigning the eyes could reduce the binocular VF and cause diplopia. In heteronymous hemianopias, there is little risk of the surgery causing diplopia, but the strabismus is unstable because of the absence of binocular vision and vergence reflexes if VF loss includes the macula. Further, strabismus, in association with hemianopia, may be caused by coexistent cranial nerve and/or gaze palsy. The prognosis, objective, and results of the strabismus operation should be clearly discussed with the patients or their guardians before it is performed if hemianopias are present.

Review: Binocular double vision in the presence of visual field loss.

Binocular double vision in strabismus is marked by diplopia (seeing the same object in two different directions) and visual confusion (seeing two different objects in the same direction). In strabismus with full visual field, the diplopia coexists with visual confusion across most of the binocular field. With visual field loss, or with use of partial prism segments for field expansion, the two phenomena may be separable. This separability is the focus of this review and offers new insights into binocular function. We show that confusion is necessary but is not sufficient for field expansion. Diplopia plays no role in field expansion but is necessary for clinical testing of strabismus, making such testing difficult in field loss conditions with confusion without diplopia. The roles of the three-dimensional structure of the real world and the dynamic of eye movements within that structure are considered as well. Suppression of one eye's partial view under binocular vision that develops in early-onset (childhood) strabismus is assumed to be a sensory adaption to diplopia. This assumption can be tested using the separation of diplopia and confusion.

The effect of teprotumumab infusion on ocular alignment in patients with symptomatic thyroid eye disease.

BACKGROUND: Thyroid eye disease (TED) can result in proptosis and ocular misalignment, leading to eye pain, diplopia, and vision loss. Teprotumumab, a humanized antibody against insulin-like growth factor 1 receptor, was approved in 2020 for the treatment of TED. The purpose of this study was to describe the effect of a full course of teprotumumab on ocular misalignment. METHODS: The medical records of patients who underwent treatment with teprotumumab for active moderate-to-severe TED at a single institution from April 2020 to September 2023 were reviewed retroactively. Sensorimotor examination was performed at each visit using simultaneous prism-cover testing. Demographic information and previous history of radioactive iodine, steroids, strabismus surgery, and smoking were extracted from the record for analysis. RESULTS: A total of 19 patients were treated during the study period, of whom 11 had strabismus and diplopia. The initial absolute horizontal misalignment in these 11 was 6.0Δ ± 1.5Δ, vertical misalignment was 7.7Δ ± 2.4Δ, and total misalignment was 11.5Δ ± 2.0Δ. On completion of treatment, these measurements decreased by 2.0Δ ± 1.5Δ, 2.2Δ ± 1.0Δ, and 3.2Δ ± 1.6Δ, respectively (P = 0.10, 0.02, and 0.04, resp.). Eight patients (73%) had a decrease in their strabismus, and 5 (46%) reported complete resolution of their diplopia at the final visit. No factors were predictive of which patients would have resolution of their misalignment. Of the remaining 3 patients who had no improvement in ocular alignment, 2 (66%) underwent strabismus surgery. Of the 8 patients with improvement of strabismus, only a single patient (13%) underwent strabismus surgery for persistent diplopia. CONCLUSIONS: In our study cohort, a full course of teprotumumab coincided with complete resolution of diplopia in 46% of patients and a decrease in strabismus in 73% of patients.

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

BACKGROUND AND OBJECTIVES: We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. METHODS: In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. RESULTS: Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. DISCUSSION: The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. TRIAL REGISTRATION INFORMATION: ClinicalTrials.gov: NCT03049956.

Doses of medial rectus muscle recessions for divergence insufficiency-type esotropia.

We evaluated whether doses of bilateral medial rectus recessions greater than Parks's tables yielded superior outcomes for adult-onset divergence insufficiency. Forty-two patients underwent bilateral medial rectus recessions. Dose was analyzed as the average total per muscle (surgery + suture adjustment if performed) and compared with the standard dose tables (based on preoperative distance esodeviation), as difference between dose performed and dose indicated by Parks's tables. Each participant was classified as having received either Parks's dose (within 0.5 mm) or a dose greater than Parks's dose. Success was defined as "rarely" or "never" diplopia in distance straight-ahead gaze and reading. For patients classified as success, the mean difference between actual surgical dose performed and Parks's dose was calculated. Success was 91% (29/32) in those receiving greater than Parks's dose versus 67% (6/9) with Parks's dose (difference = 24%; 95% CI, -5% to 60%). The mean surgical dose was 1.0 mm greater than Parks's tables for the 35 successes (at 10 weeks) versus 0.7 mm greater for the 6 failures (difference = 0.4 mm; 95% CI, -0.2 to 0.9). For medial rectus recessions in adult-onset divergence insufficiency-type esotropia, a surgical dose 1 mm greater than Parks's tables, for each muscle, is a reasonable strategy.

Comparison of simultaneous prism and cover test (SPCT) and prism and alternate cover test (PACT) measurements with final power of Press-On prism power dispensed.

PURPOSE: To investigate the association of strabismic angle obtained by simultaneous prism and cover test (SPCT) and prism alternate cover test (PACT) with the final power of Press-On prism used in adults with symptomatic strabismus. METHODS: The medical records of consecutive patients seen by a single orthoptist at an urban, tertiary care hospital over a 36-month period were reviewed retrospectively. All subjects had been prescribed Press-On prism for diplopia after evaluation with SPCT and PACT measurements at distance fixation. Prism dispensed was chosen by the patient using a trial Press-On prism set. RESULTS: A total of 244 charts were reviewed. Of these, 108 adult patients had prism dispensed, and 32 had both SPCT and PACT measurements. Mean SPCT measurement was 7Δ (median, 6Δ; range, 3Δ-14Δ); mean PACT, 13Δ (median, 11Δ; range, 8Δ-20Δ). The mean prism power dispensed was 7Δ (median, 6Δ; range, 1Δ-15Δ); this was not significantly different from mean SPCT (P = 0.35). There was a large difference (6Δ) between mean PACT measurements and mean prism dispensed (P < 0.001). At follow-up of 1 year, 21 patients (66%) remained in prism. Nine patients (4%) elected to undergo eye muscle surgery, 7 with horizontal strabismus, 6 of whom had SPCT measurements of >10Δ and PACT measurements of >15Δ. CONCLUSIONS: Press-On prism power used was more closely tied to the angle of strabismus obtained by SPCT. Overall success rate of Press-On prism was good when the prism power dispensed was close to the SPCT measurement, suggesting that it is reasonable to initiate treatment with a weaker prism, approximating the SPCT measurement, building up only if there are continuing symptoms.

An Update on Dragged-Fovea Diplopia Syndrome.

Dragged-fovea diplopia syndrome (DFDS) is a type of binocular double vision caused by a displacement of the fovea in one or both eyes due to retinal disorders including epiretinal membranes or other maculopathies. DFDS induces diplopia through a mismatch between peripheral motor fusion and central (foveal) fusion. It can be diagnosed by utilizing the Lights on - Lights off test. While there is no cure, there are treatments for DFDS including monocular occlusion or blurring (tape, lenses, IOL), Bangerter filter, and Fresnel prisms. While this syndrome has been identified in the literature by multiple names including central-peripheral Rivalry (CPR)-type diplopia, macular diplopia, and foveal displacement syndrome, this article works to summarize the current known characteristics, diagnostic tests, and treatment for this syndrome.

Age-related nonparalytic hypertropia: Clinical features.

PURPOSE: Age-related nonparalytic hypertropia (ARNH) is reported to be involved in sagging eye syndrome, where excyclotorsion is large and the lower eye is more extorted. The primary aim of this study was to describe the clinical profiles of patients with ARNH. The secondary aim was to compare cyclotorsion in patients with ARNH with that in normal individuals. METHODS: Inclusion criteria for ARNH were insidious onset of diplopia at distance after 50 years of age, nonparalytic hyperdeviation and follow-up >6 months. Objective cyclotorsion was measured as the disc-to-fovea angle (DFA) on fundus photographs obtained from two groups: 75 patients with ARNH (age, 74.1 ± 7.9 years) and 75 sex- and age-matched normal controls (73.9 ± 8.1 years). RESULTS: The hypertropia angle was 4.6 ± 2.8 prism dioptres. One patient also experienced diplopia at near-gaze. DFA in ARNH (right/left eye; 11.0 ± 4.8°/11.6 ± 3.9°) was larger than that of the control (6.6 ± 3.7°/9.2 ± 3.5°) (p < 0.0001). The DFA in the non-dominant eye (12.5 ± 4.1°) was larger than that in the dominant eye (10.2 ± 4.3°) (p = 0.0003). The lower eye did not have the larger DFA in 29 patients. CONCLUSION: The angle of hyperdeviation in patients with ARNH was small. The DFA in the ARNH group was larger than that in the normal group. The lower eye was not the eye with a larger DFA in 39% of ARNH, inconsistent with sagging eye syndrome. Decreased phoria adaptation (fusional ability) may trigger diplopia in patients with ARNH.

Unusual and Rare Causes of Monocular Elevation Deficit.

INTRODUCTION: To study the rare and unusual causes of monocular elevation deficit. METHODS: Five patients presenting to us with diplopia and elevation deficit were thoroughly examined and were found to have monocular elevation deficit due to rare causes. OBSERVATIONS: All five were found to have different underlying etiologies - iatrogenic, sphenoid wing meningioma, cysticercosis, sarcoidosis and mid brain infarct, and were managed appropriately. DISCUSSION: Monocular Elevation Deficit can occur due to a variety of causes. Having a high index of suspicion for the more serious etiologies is of utmost importance. Thorough clinical examination and imaging help clinch the diagnosis.

Ocular Neuromyotonia: Clinical Features, Diagnosis, and Outcomes.

PURPOSE: The purpose of this study was to describe the clinical features, management, outcomes, and diagnostic pitfalls in a large series of patients with ocular neuromyotonia. DESIGN: Retrospective cohort. METHODS: Patients diagnosed with ocular neuromyotonia from January 1, 2004, through January 1, 2023, seen at one of the 3 Mayo Clinic sites in Rochester, MN, Scottsdale, AZ, and Jacksonville, FL, comprised the study population. We ascertained patients with ocular neuromyotonia through a search using the medical records database. Only patients with an observed episode of ocular neuromyotonia were included and the medical records were reviewed. The main outcome measures were clinical features and outcomes of patients with ocular neuromyotonia. RESULTS: Forty-two patients who were diagnosed with ocular neuromyotonia were included. The median age was 58 years (range, 16-80 years). A history of cranial radiation therapy was present in 39 patients (93%). The sixth cranial nerve was involved in 31 patients (74%). Bilateral disease was found in 2 patients (5%). The median time from onset of diplopia to diagnosis was 8 months (range, 1 month-25 years), with a high rate of initial misdiagnosis in 52%. Twenty of 42 patients (48%) were treated with oral medication, of whom 95% had significant improvement or resolution of symptoms. CONCLUSION: Prior cranial irradiation is the most common cause for ocular neuromyotonia, affecting the sixth cranial nerve most often. Although delayed and initial misdiagnosis is common, most patients show improved symptoms on medical treatment.

Outcomes of Strabismus Surgery Following Teprotumumab Therapy.

PURPOSE: To investigate the results of patients undergoing surgical treatment for strabismic diplopia in thyroid eye disease (TED) following teprotumumab. DESIGN: Multicenter, retrospective, case series. METHODS: We report 28 patients who underwent extraocular muscle surgery for strabismic diplopia after treatment with teprotumumab at 7 different academic centers. Elapsed time from last teprotumumab dose to the date of surgery, previous orbital decompression, primary preoperative horizontal and vertical deviation, surgical procedure, and 2-month postoperative results were collected from the patient records. RESULTS: Sixteen (57%) patients were diplopia-free after 1 surgery. Three (11%) chose prism spectacles to correct residual diplopia, 2 (7%) used compensatory head posture to resolve diplopia, and 1 (4%) had intermittent diplopia and was functionally improved (choosing no prisms or further surgery). These were considered treatment successes. Three (11%) patients required reoperation, and all were diplopia-free after their second procedure. CONCLUSIONS: Most patients requiring surgery for strabismic diplopia following teprotumumab achieve good outcomes with success rates comparable to series published before the availability of teprotumumab.

Reactivation After Teprotumumab Treatment for Active Thyroid Eye Disease.

PURPOSE: To determine the recurrence and reactivation rates after teprotumumab therapy for active thyroid eye disease. DESIGN: Retrospective consecutive case series. METHODS: This was a study of all patients followed for active thyroid eye disease at the Cole Eye Institute, Cleveland Clinic, treated with teprotumumab between May 2020 and May 2021. Patients with less than 6 months follow-up after completion of infusions were excluded. The primary outcome measure was reactivation, defined as a regression in proptosis (increase of ≥2 mm in either eye and to within ≤2 mm of pre-treatment level and Clinical Activity Score [CAS] worsening of 2 points or greater). Secondary outcome was diplopia response. RESULTS: A total of 21 patients were included in the study. The average long-term improvement in proptosis in the eye with more proptosis after teprotumumab was 1.57mm (range, -3 to 4 mm). Of the 17 initial responders, there were 8 reactivations (47%) and 2 isolated proptosis regressions (12%); Overall, 7 of 21 patients (33%) responded throughout the study period. Average time to regression was 12.25 months (range, 2-22.5 months). There was no statistically significant change in diplopia at final visit in any subgroup (P = 0.68 to >.99). CONCLUSIONS: At most, 33% of patients demonstrate continued response 2 years after teprotumumab treatment. The proptosis and CAS regression occurs in the setting of disease reactivation in 80% of regressions. Teprotumumab treatment appears to offer minimal long-term improvement in diplopia.

Outcomes of the intraoperative relaxed muscle positioning technique in strabismus surgery for thyroid eye disease.

BACKGROUND: The intraoperative relaxed muscle positioning (IRMP) technique for strabismus surgery in thyroid eye disease (TED) involves recessing muscles' insertions to positions where they rest without tension. We evaluate the long-term outcomes of patients who underwent surgery using this technique. METHODS: The medical records of patients with TED and diplopia who underwent strabismus surgery with IRMP between 1999 and 2021 were reviewed retrospectively. Excellent outcomes were defined as no diplopia in primary and reading gazes without the use of prisms; good outcomes, as residual deviation of <10Δ with diplopia in primary and reading gazes. Good or excellent outcomes were considered successes. Poor outcomes were defined as residual deviation >10Δ with diplopia in primary and reading gazes or the inability of patients to tolerate prismatic correction. RESULTS: A total of 129 patients were followed for an average of 4.24 ± 5.13 years (longest follow-up 20 years): 96 (73.6%) underwent a single surgery, and 33 (26.4%) underwent additional interventions. Seven of these had been planned as staged procedures, and all were successful. Five patients experienced disease reactivation and underwent additional surgery, with successful outcomes in 4 (80%). Of the remaining 21 patients, 6 underwent a third procedure, and a single patient underwent a fourth. Overall, 93.6% of patients had a successful outcome: 77.5% excellent and 16.3% good. CONCLUSIONS: In our study cohort, the IRMP approach resulted in durable relief from large-angle strabismus and diplopia in a large majority of patients.

Slab-off prism for symptomatic, vertical heterophoria in the reading position.

PURPOSE: To describe uncommon techniques that induce compensatory strabismus utilizing slab-off spectacle prism to treat symptomatic incomitant vertical strabismus in adults with diplopia in the reading position only. METHODS: The medical records of consecutive patients having a chief complaint of diplopia in the reading position that was treated with slab-off prism correction were reviewed retrospectively. Success of the slab-off spectacle prism correction was determined at the follow-up visit or by phone call for any patients who did not return for a follow-up examination. RESULTS: Five adult subjects were identified, ranging in age from 55 to 75 years (mean age, 65.6 years). All 5 patients complained of vertical diplopia during reading. Symptoms resolved with slab-off prism in all 5 cases. Three patients had symptomatic incomitant vertical strabismus occurring only in the reading position. All 3 of these patients remained in slab-off prism at in-office follow-up. CONCLUSIONS: Slab-off spectacle prism in non-digitally manufactured multifocal lens prescriptions was an effective nonsurgical treatment option in this descriptive study of 5 patients with diplopia in the reading position only, 3 of whom had symptoms related to small-angle vertical strabismus in the reading position only, without anisometropia.

Interocular suppression in primary visual cortex in strabismus: impact of staggering the presentation of stimuli to the eyes.

Diplopia (double vision) in strabismus is prevented by suppression of the image emanating from one eye. In a recent study conducted in two macaques raised with exotropia (an outward ocular deviation) but having normal acuity in each eye, simultaneous display of stimuli to each eye did not induce suppression in V1 neurons. Puzzled by this negative result, we have modified our protocol to display stimuli in a staggered sequence, rather than simultaneously. Additional recordings were made in the same two macaques, following two paradigms. In trial type 1, the receptive field in one eye was stimulated with a sine-wave grating while the other eye was occluded. After 5 s, the occluder was removed and the neuron was stimulated for another 5 s. The effect of uncovering the eye, which potentially exposed the animal to diplopia, was quantified by the peripheral retinal interaction index (PRII). In trial type 2, the receptive field in the fixating eye was stimulated with a grating during binocular viewing. After 5 s, a second grating appeared in the receptive field of the nonfixating eye. The impact of the second grating, which had the potential to generate visual confusion, was quantified by the receptive field interaction index (RFII). For 82 units, the mean PRII was 0.48 ± 0.05 (0.50 = no suppression) and the mean RFII was 0.46 ± 0.08 (0.50 = no suppression). These values suggest mild suppression, but the modest decline in spike rate registered during the second epoch of visual stimulation might have been due to neuronal adaptation, rather than interocular suppression. In a few instances neurons showed unequivocal suppression, but overall, these recordings did not support the contention that staggered stimulus presentation is more effective than simultaneous stimulus presentation at evoking interocular suppression in V1 neurons.NEW & NOTEWORTHY In strabismus, double vision is prevented by interocular suppression. It has been reported that inhibition of neuronal firing in the primary visual cortex occurs only when stimuli are presented sequentially, rather than simultaneously. However, these recordings in alert macaques raised with exotropia showed, with rare exceptions, little evidence to support the concept that staggered stimulus presentation is more effective at inducing interocular suppression of V1 neurons.

Clinical Outcome and Technical Nuances After Resection of Orbital Cavernous Venous Malformations-A Single-Center Experience.

BACKGROUND: Cavernous venous malformations (CVMs) represent the most common benign intraorbital lesions. Enlarging or symptomatic CVMs (progressive proptosis or visual disturbances) are treated by surgical resection. For this, a variety of different surgical approaches have been described. The aim of this study was to present a contemporary series of orbital CVMs treated via open microsurgical approaches. METHODS: In this study, patients who underwent resection of orbital CVMs between 2002 and 2019 were included. Presenting symptoms were noted and neuro-ophthalmologic examinations performed pre- and postoperatively. For surgical resection, the location of the orbital CVM and its relation to the orbital anatomy led to decision-making for appropriate approaches. A comparison between anatomical location and surgical outcome was performed. RESULTS: Overall, 35 patients with orbital CVMs were included. Most common presenting symptoms were progressive proptosis (43%) and visual disturbances (34%). Most common location was the lateral quadrant (37%) followed by the superior quadrant (20%). A subfrontal craniotomy was performed in 40% of cases followed by a supraorbital craniotomy including the orbital rim in 34% of cases. For surgical excision, a cryo-probe was used in 30 patients, and complete resection was feasible in all cases. Location of a CVM within the superior quadrant was associated with improved postoperative recovery of visual acuity. No differences for clinical outcomes were observed depending on the surgical approach. CONCLUSIONS: Resection of orbital CVMs is indicated in patients with visual disturbances or progressive proptosis. In these, microsurgical approaches can be used with minimal morbidity for complete removal of these well-circumscribed lesions.

Efficacy and safety of tripterygium glycosides for active moderate to severe Graves' ophthalmopathy: a randomised, observer-masked, single-centre trial.

BACKGROUND: Tripterygium glycosides (TG) has been used to treat a spectrum of inflammatory and autoimmune diseases. Our preliminary studies have shown that TG is effective in the treatment of active Graves' ophthalmopathy (GO). OBJECTIVE: We aimed to compare the efficacy and tolerability of TG with intravenous methylprednisolone (iv.MP) in patients with active moderate-to-severe GO. METHODS: This study was an observer-masked, single-centre, block-randomised trial. Patients with active moderate-to-severe GO were randomly assigned to receive iv.MP (500 mg once per week for 6 weeks followed by 250 mg per week for 6 weeks) or with TG (20 mg tablet three times per day for 24 weeks). The primary endpoints were the overall response rate and the patients' quality of life at 12 and 24 weeks. RESULTS: In this study, 161 patients were enrolled and randomised from 2015 to 2019. A total of 79 were randomly assigned to receive iv.MP and 82 to receive TG. A greater overall response rate was found in the TG group compared with the iv.MP group at week 24 (90.2% vs 68.4%, P = 0.000). Similarly, the patients' quality of life of the TG group showed a significantly higher response than the iv.MP group at week 24 (89.02% vs 72.15%, P = 0.001). The TG therapy showed a better CAS response than the iv.MP (91.5% vs 70.9% improved, P < 0.05), and up to 91.2% of patients were inactive. Also, the TG group showed a significantly higher improved rate of diplopia, proptosis, visual acuity, soft tissue involved and the decrease of eye muscle motility than the iv.MP group at week 24. Significantly more patients in the iv.MP group than the TG group experienced adverse events. CONCLUSION: Compared with iv.MP treatment, TG therapy is more effective and safer for patients with active moderate to severe GO.

Double Vision and Gait Ataxia in an Immunocompetent 9-Year-Old Girl With Intracranial Phaeohyphomycosis.

ABSTRACT: A 9-year-old girl presented with morning headaches associated with vomiting, gait ataxia, and facial and ocular motor nerve palsies. Her initial imaging was concerning for demyelinating disease. After extensive infectious and rheumatologic workup returned negative, she was treated twice with intravenous immunoglobulin and intravenous steroids with near-complete resolution each time. She returned, however, with worsening neurologic deficits and imaging revealing focal ischemic infarction in the brainstem as well as new-onset hydrocephalus. A multispecialty workup was initiated without conclusive diagnosis. A novel, noninvasive test for plasma cell-free DNA established a diagnosis of Cladophialophora bantiana that was confirmed and validated by a brain biopsy taken during a clinical decompensation. Treatment was initiated with systemic voriconazole and intraventricular amphotericin B.