In adults with optic disc drusen and type-2-diabetes or obesity, glucagon-like peptide-1 receptor agonists exposure was not associated with increased nonarteritic anterior ischemic optic neuropathy hazard, though moderate risk increases could not be excluded statistically.

Purpose We demonstrate the potential role of acalabrutinib, a second-generation selective Bruton tyrosine kinase (BTK) inhibitor, as monotherapy in treating optic nerve infiltration by chronic lymphocytic leukemia (CLL). Observations A 68-year-old male, initially diagnosed with CLL 13 years earlier, presented with a decrease in vision and intermittent floaters for 5 days. On fundus examination, there was optic disc swelling in both eyes (OU) concerning for CLL infiltration and vitreous haze in the left eye (OS). The MRI of the brain and orbit was normal, and his infectious and uveitis workups were unremarkable. He was prescribed acalabrutinib 100 mg twice daily by mouth. Five days later, the optic disc edema OU and vitreous haze OS improved and completely subsided two months later. Conclusions and importance Early initiation of acalabrutinib may be sufficient therapy for CLL optic nerve infiltration.

BACKGROUND:Uveitis and optic neuritis related to demyelinating disease share similar immune mechanisms and can have deleterious visual consequences. Data on uveitis incidence in patients with central nervous system (CNS) demyelinating diseases remain limited. Our objective was to assess the 5-year incidence of uveitis among patients with CNS demyelinating diseases with and without optic neuritis. METHODS:Retrospective cohort study using a health records aggregate. We identified patients aged ≥18 years using diagnostic codes to arrange into 3 study groups: a composite CNS demyelinating disease cohort, a multiple sclerosis (MS) cohort, and a neuromyelitis optica/myelin oligodendrocyte glycoprotein antibody-associated disease cohort, each stratified by presence or absence of optic neuritis. We matched each study group based on demographics, clinical comorbidities, medications, and encounter visits. RESULTS:After matching, the 5-year cumulative incidence of any uveitis among patients with optic neuritis in the CNS demyelinating disease group was 1.09% (95% confidence intervals [CI] 0.950-1.25) compared with 0.610% (95% CI 0.501-0.743) in those without optic neuritis (log-rank P < 0.0001), with a hazard ratio (HR) of 1.99 (95% CI 1.57-2.52). The 5-year cumulative incidence of any uveitis among patients with optic neuritis in patients with MS was 0.986% (95% CI 0.846-1.15) compared to 0.543% (95% CI 0.437-0.676) in those without optic neuritis (log-rank P < 0.0001), with a HR of 1.95 (95% CI 1.51-2.53). Although patients with neuromyelitis optica/myelin oligodendrocyte glycoprotein antibody-associated disease showed similar trends, there was no statistical significance. Intermediate uveitis demonstrated the strongest associations with optic neuritis across study groups. CONCLUSIONS:Optic neuritis is a clinically meaningful risk factor for uveitis in patients with CNS demyelinating disease, underscoring the need for careful ophthalmic surveillance in this population.

Postoperative vision loss (POVL) can be devastating and permanent. We present three unique cases of POVL after shoulder surgery, and we provide recommendations on patient positioning and eye protection perioperatively based on our experience and a review of the literature. In Case 1, our patient underwent an uncomplicated right arthroscopic rotator cuff repair and acromioclavicular (AC) joint resection in the beach-chair (BC) position. He developed bilateral vision loss (right eye greater than the left eye) in the postoperative period, with visual field deficits noted on Humphrey visual field (HVF) 24-2, and decreased retinal function on electroretinography (ERG) in the right eye (OD). In Case 2, our patient underwent an uncomplicated left total shoulder arthroplasty with open biceps tenodesis and implant removal in the BC position. He developed vision loss in the left eye (OS) in the postoperative period, with visual field deficits noted on HVF 24-2, and ERG findings that pointed to an ischemic injury to the retina and optic nerve in the left eye (OS). In Case 3, our patient underwent an uncomplicated right arthroscopic rotator cuff repair in the BC position. He developed vision loss OS in the postoperative period and was diagnosed with a macular-sparing central retinal artery occlusion (CRAO) OS, with eventual foveal recovery. POVL after shoulder surgery is a serious condition with a risk of permanent vision loss. Although POVL after shoulder surgery is rare and likely multifactorial, it is important to minimize risks and optimize both patient positioning and eye protection to promote patient safety and favorable outcomes.

Purpose To assess the risk of developing aortic aneurysms (AAs) and carotid artery stenosis (CAS) in patients with giant cell arteritis (GCA), particularly among those presenting with and without visual symptoms. Design Retrospective cohort study. Subjects A total of 7294 patients aged ≥50 years with biopsy-proven GCA (temporal artery biopsy within 2 weeks of diagnosis and ≥3 prednisone refills) were identified and compared with 265 948 control patients presenting with tension-type headache using the TriNetX US Collaborative Network. A secondary comparison was performed between patients with GCA with (n = 2390) and without (n = 5222) visual symptoms (eg, diplopia, amaurosis fugax, vision loss). Methods GCA was defined using International Classification of Diseases, 10th Revision codes M31.5 and M31.6. Patients with a history of other vasculitides, prior AAs, or major thrombotic events were excluded. Propensity score matching was used to balance demographics, socioeconomic factors, comorbidities, substance use, and laboratory/vital parameters, resulting in matched cohorts for each comparison. Adjusted hazard ratios (aHRs) and 95% CIs were estimated using Cox proportional hazards models to account for time to onset of vascular complications. Main Outcome Measures Primary outcomes were the 5-year risks of (1) thoracic AAs, (2) thoracoabdominal AAs, (3) abdominal AAs, and (4) CAS. Results After matching, 7252 patients remained in each arm for the primary GCA versus control comparison. Patients with GCA had a significantly higher 5-year risk of any AA (3.59% vs 1.75%; aHR, 1.98; 95% CI, 1.59-2.45), including thoracic (2.23% vs 1.02%; aHR, 2.01; 95% CI, 1.59-2.77), thoracoabdominal (0.32% vs 0.14%; aHR, 3.68; 95% CI, 1.50-9.05), and abdominal (1.80% vs 0.82%; aHR, 2.03; 95% CI, 1.49-2.77). CAS was also elevated in GCA (7.20% vs 4.37%; aHR, 1.59; 95% CI, 1.38-1.84). In the subanalysis of patients with GCA, the 5-year risk of any AA was comparable between those with and without visual symptoms (3.58% vs 3.23%; aHR, 1.14; 95% CI, 0.83-1.57). However, CAS occurred more frequently in patients with GCA presenting with visual symptoms (8.95% vs 7.43%; aHR, 1.24; 95% CI, 1.01-1.53). Conclusions Patients with GCA demonstrate a substantially increased risk of AAs, particularly thoracic AAs, compared with matched controls. Although having visual symptoms did not correlate with additional aortic risk, they were associated with a higher risk of CAS.

Purpose: To present a case of molecularly confirmed oculocutaneous albinism (OCA) and retinitis pigmentosa (RP). Observations: A 46-year-old male with a lifelong established diagnosis of OCA and baseline best corrected visual acuity (BCVA) of 20/200, presented for worsening visual acuity over the last few years. BCVA was light perception and hand motion at face for the right and left eye, respectively. Fundus exam showed hypopigmented fundi with visible choroidal vessels and blunted foveal reflexes in both eyes. Optical coherence tomography showed foveal hypoplasia and outer retinal degenerative changes not typical of OCA. Fundus autofluorescence (FAF) imaging showed focal areas of decreased signal at the fovea, similar to areas of atrophy in an age matched patient with PDE6A-RP. Genetic testing identified a homozygous disease-causing variant in TYR c.1467dup, p. (Ala490Cysfs*20) causing OCA, and a homozygous pathogenic variant c.304C > A, p. (Arg102Ser) in PDE6A causing autosomal recessive RP. Conclusions and importance: This is the first report of a patient with OCA and RP. The lack of pigmentary changes can make the diagnosis of RP challenging in patients with albinism. FAF can show features suggestive of RP and genetic testing can establish the diagnosis. The findings described herein may help physicians diagnose an extremely rare phenotype.

BACKGROUND AND OBJECTIVE:Retrospective analysis correlating serologic titers of ocular syphilis with posterior segment manifestations. PATIENTS AND METHODS:This study consisted of 40 patients (80 eyes imaged, 68 affected) with positive rapid plasma reagin (RPR) and Treponema Pallidum immunoglobulin G. We collected demographic and presentation data including HIV status, absolute CD4 count, RPR, cerebrospinal fluid-venereal disease research laboratory (CSF-VDRL) test, and retinal zone. We categorized imaging into syphilitic outer retinopathy (SOR), acute syphilitic posterior placoid chorioretinopathy, retinitis/chorioretinitis (RC), and papillitis. Multivariate analysis correlated HIV status, RPR, and VDRL titers with posterior segment findings and zone. RESULTS:Mean age of 42.8 ± 10.7 years, with 70% male patients. Presenting visual acuity (logMAR) 0.66 ± 0.74 did not correlate with RPR, nor was it associated with papillitis, RC, or acute syphilitic posterior placoid chorioretinopathy. Higher RPR (≥ 1:128) positively associated with SOR (P = 0.031) and zone 1 (odds ratio [OR], 1.62; P = 0.02), but negatively associated with zone 2 (OR 0.35; P = 0.005). HIV positivity increased RC odds (OR, 4.45; P = 0.047). CONCLUSION:Higher RPR correlated with SOR and zone 1, whereas HIV positivity correlated with RC. [Ophthalmic Surg Lasers Imaging Retina 2024;55:511-516.].

Department of Ophthalmology, Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas. Address correspondence to Joseph G. Chacko, MD, Department of Ophthalmology, Jones Eye Institute, University of Arkansas for Medical Sciences, 4301 W. Markham Street, Slot #523, Little Rock, AR 72205; E-mail: [email protected] The authors report no conflicts of interest.

We investigated the relationship between self-reported history of visual impairment in youth and the development of dementia in later life using data from the Panel Study of Income Dynamics (PSID) household survey, which included the Eight-item Informant Interview to Differentiate Aging and Dementia (AD-8) screen. Adults with a reported history of childhood visual impairment were found to have significantly higher odds of positive dementia screening. After adjusting for confounders, we found a twofold increase in those reporting early visual impairment compared with those who did not report early visual impairment.

BACKGROUND Age-related distance esotropia (ARDE), is an acquired, small, comitant esodeviation that is greater at distance than at near. It occurs in older adult patients without a history of neurological event or prior strabismus. It has been observed more frequently in White adults than in other racial groups. The purpose of this study was to assess the demographic and clinical characteristics of patients with ARDE presenting at a tertiary neuro-ophthalmology clinic.METHODS In this retrospective study, ICD-9/10 (ICD-9 378.85 and ICD-10 H51.8) codes were used to identify all patients with ARDE from 2005 to 2020 seen in a single tertiary neuro-ophthalmology clinic. ARDE was defined as esotropia greater at distance than near with associated clinical signs of adnexal tissue laxity. Patients with history or findings compatible with other etiologies of strabismus, such as thyroid eye disease, neuromuscular disorders, sensory deviations, sudden onset of diplopia, and high myopia, as well as those with prior strabismus surgery, were excluded.RESULTS A total of 89 patients (59 females [66%]) met inclusion criteria. Mean patient age was 76.6 years. All patients were White except for a single patient of African descent. Mean follow-up time was 25.2 months. Mean esodeviation at distance on presentation was 6.6(?). Of the 87 patients electing nonsurgical treatment, 80 achieved remission of diplopia symptoms with prism therapy alone. Of the 89 patients, 59 had no neuroimaging.CONCLUSIONS ARDE in our neuro-ophthalmology clinic population was diagnosed almost exclusively in older White adults. Prism therapy was effective for a majority of our patients.

Central retinal artery occlusion (CRAO) can result in devastating permanent vision loss. Presently, there is no evidence-based treatment for CRAO that is widely accepted. In the literature, multiple studies propose intravenous (IV) prostaglandin E1 (IV PGE1) as a potential treatment option for patients with CRAO. We illustrate 2 cases of CRAO successfully treated with IV PGE1. In both cases, our patients with vascular risk factors were diagnosed with CRAO of the left eye. They were started on twice daily IV 40 μg PGE1 in 100 mL normal saline, with each dose administered over 3 h. In the first case, we documented reperfusion of the retina on fluorescein angiography after administration of IV PGE1. In the second case, our patient improved from no light perception visual acuity (VA) to count fingers VA within 48 h of treatment with IV PGE1. Our study highlights the vasodilatory effect of IV PGE1. Due to its mechanism of action and safety profile, it should be considered a potential treatment option for CRAO. Further randomized controlled trials are necessary to determine the overall therapeutic effect of IV PGE1 for CRAO.

Amiodarone-associated optic neuropathy (AAON) is a complex clinical diagnosis, requiring distinction from non-arteritic ischemic optic neuropathy (NAION) due to a shared at-risk patient population. Diagnosis of AAON is complicated by a varied clinical presentation and incomplete pathophysiologic mechanisms. This article reviews pertinent literature for describing and clinically delineating AAON from NAION, as well as newly reported protective mechanisms of insulin-like growth factor 1 (IGF-1) and PI3K/Akt against amiodarone-induced oxidative and apoptotic injury in retinal ganglion and pigment epithelial cells. These studies offer a basis for exploring mechanisms of amiodarone toxicity in the optic nerve.

Carotid-cavernous fistula (CCF) is an aberrant communication between the main trunk or branches of carotid artery and the cavernous sinus. Most of the cases of CCF occur following head trauma, but congenital and spontaneous cases have been reported. We report an interesting case of bilateral CCF with no history of trauma, thus most likely spontaneous form. Since it is rare, it was a diagnostic challenge. The suspicion of this diagnosis was made due to clinical features of headache, signs of increased Intracranial Pressure (ICP) (nausea, vomiting, and worsening headaches during Valsalva), exophthalmos, periorbital edema, periorbital erythema, chemosis, and conjunctival injection in both eyes. It was diagnosed with a 4-vessel angiography (digital subtraction angiography) which is the gold standard and was managed successfully with endovascular coil embolization.

Neuroretinitis is an inflammatory condition with rapid unilateral vision loss, optic disc edema, and macular star formation. While neuroretinitis is commonly due to infectious causes such as Bartonella henselae, neuroretinitis due to toxoplasmosis is uncommon. A 29-year-old male presents to our neuro-ophthalmology clinic on December 7, 2021, at the University of Arkansas for Medical Sciences with symptoms of left eye pain and blurred vision. Subsequent workup led to the diagnosis and treatment of toxoplasma neuroretinitis. The fundus exam eventually demonstrated a notable macular star. Treatment was well tolerated, and the patient regained total visual acuity in the affected eye. Toxoplasma neuroretinitis is known for a characteristic appearance of optic disc edema prior to appearance of stellate maculopathy with vitreous inflammation and peripheral chorioretinal scars. Although loss of vision due to toxoplasmosis is rare, it should be included as part of the differential diagnosis with pertinent history.

The purpose of this work is to identify mitochondrial optic nerve (ON) lipid alterations associated with sonication-induced traumatic optic neuropathy (TON). Briefly, a mouse model of indirect TON was generated using sound energy concentrated focally at the entrance of the optic canal using a laboratory sonifier (Branson Digital Sonifier 450, Danbury, CT, USA) with a microtip probe. We performed an analysis of a previously generated dataset from high-performance liquid chromatography-electrospray tandem mass spectrometry (LC-MS/MS). We analyzed lipids from isolated mitochondria from the ON at 1 day, 7 days, and 14 days post-sonication compared to non-sonicated controls. Lipid abundance alterations in post-sonicated ON mitochondria were evaluated with 1-way ANOVA (FDR-adjusted significant p-value < 0.01), debiased sparse partial correlation (DSPC) network modeling, and partial least squares-discriminant analysis (PLS-DA). We find temporal alterations in triglyceride metabolism are observed in ON mitochondria of mice following sonication-induced optic neuropathy with notable depletions of TG(18:1/18:2/18:2), TG(18:1/18:1/18:1), and TG(16:0/16:0/18:1). Depletion of mitochondrial triglycerides may mediate ON damage in indirect traumatic optic neuropathy through loss energy substrates for neuronal metabolism.

Purpose: To identify optic nerve (ON) lipid alterations associated with sonication-induced traumatic optic neuropathy (TON). Design: Experimental study. Subjects: A mouse model of indirect TON was generated using sound energy concentrated focally at the entrance of the optic canal using a laboratory sonifier with a microtip probe. Methods: Analyses of datasets generated from high-performance liquid chromatography-electrospray tandem mass spectrometry of ONs dissected from the head of the ON to the optic chiasm at 1 day, 7 days, and 14 days postsonication compared with that in nonsonicated controls. Main Outcome Measures: Lipid abundance alterations in postsonicated ONs were evaluated using 1-way analysis of variance (false discovery rate-adjusted significant P value < 0.01), lipid-related gene sets, biochemical properties, and receiver operating characteristic to identify lipids associated with optic neuropathy. Results: There were 28 lipid species with significantly different abundances across the control and postsonication groups. The 2 most significantly upregulated lipids included a sphingomyelin (SM) species, SM(d40:7), and a hexosylceramide (CerG1) species, CerG1(d18:1/24:2). Hexosylceramide (d18:1/24:2) was noted to have a stepwise increasing trend from day 1 to day 14 after sonication-induced optic neuropathy. Investigation of biophysical properties showed notable enrichment of lipids with high and above-average transition temperatures at day 14 after sonication. Lipid-related gene set analysis revealed enrichment in sphingolipid and glycosphingolipid metabolic processes. The best classifier to differentiate day 14 postsonication from controls, based on area under the receiver operating characteristic curve, was CerG1(d18:1/24:2) (area under the receiver operating characteristic curve: 1). Conclusions: Temporal alterations in sphingolipid metabolism and biochemical properties were observed in the ON of mice after sonication-induced optic neuropathy, with notable elevations in sphingomyelin and hexosylceramide species. Hexosylceramide (d18:1/24:2) may be associated with damage after indirect trauma, indicating that lipid membrane abnormalities may be a mediator of pathology due to trauma.

Chauhan, Muhammad Z. MS, MA; Chacko, Joseph G. MD; Phillips, Paul H. MD; Siddiqui, Mohammad Z. MD; Uwaydat, Sami H. MDEditor(s): Avery, Robert DO; Golnik, Karl C. MD; Froment, Caroline MD, PhD Author Information