Systemic and ocular outcomes in patients with young-onset type 2 diabetes.

PURPOSE: To analyze the systemic and ocular outcomes in patients with young-onset type 2 diabetes (YO-DM2) based on grade of presenting diabetic retinopathy (DR). METHODS: Retrospective cohort study analysis of empaneled patients with type 2 diabetes <40 years old with retinopathy screening within the Los Angeles Department of Health Services between 01/01/2017-07/01/2021 were included. Patients were stratified based on presenting severity of DR determined on fundus photographs or clinical examination. Patient's systemic co-morbidities and ocular outcomes were then compared across each group. Procedural (e.g. intravitreal injections) and surgical interventions (e.g. pars plana vitrectomy) were documented as performed by the treating physician. RESULTS: 2795 patients were screened from 12,456 patients diagnosed with diabetes younger than age 40 (22.4 %). Of these, 1496 patients were diagnosed with type 2 DM. 1084 (72.4 %) of patients presented without DR, 307 (20.5 %) presented with non-proliferative diabetic retinopathy (NPDR), and 105 (7.0 %) of patients presented with proliferative diabetic retinopathy (PDR). Increasing presenting diabetic retinopathy severity was associated with longer duration of diabetes, greater systemic comorbidities (e.g. diabetic foot disease, neuropathy, chronic kidney or end stage renal disease), worse baseline and final visual acuity, and required more procedural and surgical interventions. CONCLUSIONS: Worse presenting DR severity in patients young-onset type 2 diabetes was associated with greater comorbid systemic and ocular disease with worse visual acuity outcomes. <1 % of patients without diabetic retinopathy or with mild NPDR were likely to progress to PDR. Diabetic kidney disease was an independent risk factor for developing neovascular glaucoma and retinal detachments. Prompt evaluation and intervention in patients with YO-DM2 may help reduce the associated systemic and ocular morbidity.

The relationship of diabetic retinopathy severity scales with frequency and surface area of diabetic retinopathy lesions.

PURPOSE: To assess the relationship between qualitative diabetic retinopathy (DR) scales with the precise numbers and surface area of DR lesions within the Early Treatment Diabetic Retinopathy Study (ETDRS) standard seven field (S7F) region on ultrawide-field (UWF) color fundus images. METHODS: In this study, we collected UWF images from adult patients with diabetes. Poor-quality images and eyes with any pathology precluding assessment of DR severity were excluded. The DR lesions were manually segmented. DR severity was graded according to the International Clinical Diabetic Retinopathy (ICDR) and AA protocol by two masked graders within the ETDRS S7F. These lesions' numbers and surface area were computed and correlated against the DR scores using the Kruskal-Wallis H test. Cohen's Kappa was performed to determine the agreement between two graders. RESULTS: One thousand five hundred and twenty eyes of 869 patients (294 females, 756 right eyes) with a mean age of 58.7 years were included. 47.4% were graded as no DR, 2.2% as mild non-proliferative DR (NPDR), 24.0% as moderate NPDR, 6.3% as severe NPDR, and 20.1% as proliferative DR (PDR). The area and number of DR lesions generally increased as the ICDR level increased up to severe NPDR, but decreased from severe NPDR to PDR. There was perfect intergrader agreement on the DR severity. CONCLUSION: A quantitative approach reveals that DR lesions' number and area generally correlate with ICDR-based categorical DR severity levels with an increasing trend in the number and area of DR lesions from mild to severe NPDR and a decrease from severe NPDR to PDR.

A MULTICENTER STUDY OF RETINOPATHY OF PREMATURITY FOLLOW-UP ADHERENCE.

PURPOSE: Characterize clinical and socioeconomic factors that impact follow-up to complete retinal vascularization and subsequent pediatric ophthalmology follow-up in neonates with retinopathy of prematurity. METHODS: Medical records of 402 neonates diagnosed with retinopathy of prematurity from neonatal intensive care units at the University of California, Los Angeles Mattel Children's Hospital and the University of California, Los Angeles Santa Monica Hospital, both academic medical centers, and the Harbor-University of California, Los Angeles Medical Center, a safety-net county hospital, were reviewed. Primary study outcomes were the rate of follow-up to complete retinal vascularization and adequate pediatric ophthalmology follow-up. Secondary outcome was the rate of nonretinal ocular comorbidity. RESULTS: In whole-cohort analysis, 93.6% of neonates were followed to complete retinal vascularization, and 53.5% had adequate pediatric ophthalmology follow-up. Public insurance was associated with lower rates of pediatric ophthalmology follow-up (Odds ratio 0.66, 95% confidence interval 0.45-0.98, P = 0.04). Participants screened at the academic medical center had lower rates of pediatric ophthalmology follow-up compared with the safety-net county hospital (50.7% vs. 63.5%, P = 0.034). In subgroup analysis, academic medical center participants with public insurance were less likely to have pediatric ophthalmology follow-up than safety-net county hospital participants with public insurance (36.5% vs. 63.8%, P < 0.001) or those with private insurance at the academic medical center (36.5% vs. 59.2%, P< 0.001). CONCLUSION: This study identified high follow-up rates to complete retinal vascularization, lower pediatric ophthalmology follow-up rates, and nonretinal ocular comorbidity at all hospitals. Insurance status relative to hospital type was identified as a risk factor for loss to follow-up. This demonstrates a need to further study health care disparities in retinopathy of prematurity infants.

Purtscher-like retinopathy in a 19-year-old with maturity-onset diabetes of the young: a case report.

BACKGROUND: We report the first case of Purtscher-like retinopathy in a patient with 17q12 deletion-associated maturity-onset diabetes of the young. CASE PRESENTATION: A 19-year-old diabetic Hispanic male with history of cataracts and toe amputations presented with sudden onset of painless bilateral vision loss for 1 week with no associated trauma. Visual acuity was counting fingers at six feet in both eyes. Dilated retinal examination revealed bilateral peripapillary cotton wool spots and intraretinal hemorrhages, and significant subretinal and intraretinal fluid on optical coherence tomography. Fluorescein angiography revealed arteriolar staining and leakage around the disc with areas of capillary nonperfusion, supporting the diagnosis of Purtscher-like retinopathy. Systemic workup revealed multiple diabetic complications including chronic osteomyelitis of multiple toes, nonhealing diabetic foot ulcers, neurogenic bladder and bowel, and bilateral lower-extremity muscular neuropathies. Genetic evaluation revealed a 17q12 deletion, which is associated with maturity-onset diabetes of the young 5. On follow-up examination, he received a single intravitreal antivascular endothelial growth factor injection in the left eye (off label) for persistent macular edema. Although his retinal edema improved, his visual acuity remained poor. CONCLUSIONS: The presentation of our patient's multiple diabetic complications along visual symptoms suggests Purtscher-like retinopathy can be a sequela of uncontrolled diabetes. Purtscher-like retinopathy is a rare but possible consideration in diabetic patients who present with acute-onset vision loss.

Ethnic Variation in Diabetic Retinopathy Lesion Distribution on Ultra-widefield Imaging.

PURPOSE: To evaluate whether the distribution of diabetic retinopathy (DR) lesions differs among various ethnicities. DESIGN: Multi-center, retrospective cohort study. METHODS: We accrued a cohort of 226 eyes with DR consisting of 51 East Asian eyes, 102 South Asian eyes, 30 Caucasian eyes, and 43 Latino eyes, all evaluated with ultrawide field pseudocolor images. Images were manually annotated for DR lesions and were classified as having predominantly peripheral lesions (PPL) or predominantly central lesions (PCL) using 4 quantitative methods. The percent distribution of PCL to PPL was compared among different ethnicities. RESULTS: Using a single-field lesion frequency-based method, East Asian eyes more frequently demonstrated a PPL distribution (86.3%), whereas South Asian eyes more frequently demonstrated a PCL distribution (64.7%). These findings were also observed when considering only the subset of treatment-naïve eyes. Furthermore, in treatment-naïve eyes without proliferative DR, the percent distribution of PPL to PCL in East Asian eyes was significantly different when compared to other ethnicities (P < .0001 South Asian, P = .035 Caucasian, P = .0003 Latino). The majority of patients (60%-78%) in all ethnic groups had moderate nonproliferative diabetic retinopathy(NPDR), and the same difference between East Asian and South Asian eyes was observed in this subgroup. CONCLUSIONS: The distribution of DR lesions appears to vary among different ethnicities. DR lesions tend to be distributed more peripherally in East Asian eyes compared to other ethnic groups, particularly South Asian eyes, which tend to have more central disease. The prognostic implications of these ethnic differences in DR lesion distribution require further investigation.

MULTIMODAL IMAGING OF RECURRENT CYSTOID MACULAR EDEMA ASSOCIATED WITH POEMS SYNDROME RESPONSIVE TO INTRAVITREAL DEXAMETHASONE IMPLANT.

PURPOSE: To describe the multimodal imaging findings of cystoid macular edema (CME) in POEMS syndrome and to demonstrate the effectiveness of the dexamethasone intravitreal implant in this condition. METHODS: A case report of a patient with POEMS syndrome. Multimodal imaging, including optical coherence tomography and ultra-wide field fluorescein angiography, was used to evaluate the CME and its response to treatment. RESULTS: A 66-year-old man with a history of POEMS syndrome in clinical remission after chemotherapy presented with blurry vision in the left eye. Vitreomacular traction and CME were noted on optical coherence tomography. After pars plana vitrectomy, the patient had persistent CME in the left eye and developed new CME in the right eye, which worsened over the next year. Visual acuities at this time were 20 of 60 in both eyes. The CME was unresponsive to topical ketorolac and prednisolone acetate and intravitreal bevacizumab but partially response to intravitreal and sub-Tenon's triamcinolone acetate injections. Intravitreal dexamethasone implant was given bilaterally with full resolution and visual acuities improvement to 20 of 40 in the right eye and 20 of 30 in the left eye. However, CME recurred after 3 months. Ultra-wide field fluorescein angiography demonstrated bilateral CME and diffuse peripheral vascular leakage. Serum vascular endothelial growth factor levels were normal. CONCLUSION: Cystoid macular edema may develop in POEMS syndrome in the absence of systemic findings, elevated serum vascular endothelial growth factor, or optic nerve edema. Previously unreported peripheral vascular leakage was demonstrated on ultra-wide field fluorescein angiography. Dexamethasone implant was the most effective of used therapies to treat CME.

RETINAL DETACHMENT IN EYES WITH BOSTON TYPE 1 KERATOPROSTHESIS: Surgical Techniques and Mid-Term Outcomes.

PURPOSE: To evaluate the mid-term outcomes of pars plana vitrectomy performed for retinal detachment (RD) repair after Boston Type 1 keratoprosthesis (KPro) implantation. METHODS: Retrospective review of medical records of KPro implanted at the Stein Eye Institute presenting with RD and treated by pars plana vitrectomy. Functional success was defined as a postoperative visual acuity maintained within 2 Snellen lines of the corrected distance visual acuity measured before the development of the RD (baseline) and anatomical success as an attached retina after the pars plana vitrectomy. Kaplan-Meyer survival analyses were performed. RESULTS: Among the 224 KPro performed, 28 (15.2%) RD were identified; of which, 21 (9.4%) were included. The mean follow-up was 42.5 ± 27.3 months. Vitreoretinal proliferation was present in 18 of 21 eyes (85.7%). Surgical techniques were adapted to the complex anterior segment anatomy of KPro eyes. Anatomical success was achieved in 18 of 21 eyes (85.7%). Functional success occurred in 17 of 21 eyes (81.0%), and 5 of 21 eyes (23.8%) reached 20/400 or better visual acuity at the final follow-up. The KPro was retained in 11 in 21 eyes (52.4%). The retention rate decreased from 94.7% at 1 year to 53.5% at 5 years. The most frequent complications were retroprosthetic membrane (47.6%) and corneal melt (23.8%). CONCLUSION: Modified pars plana vitrectomy techniques resulted in relatively good mid-term anatomical, functional, and retention rate outcomes, given the severity of RD at presentation and the numerous preoperative comorbidities of KPro eyes.

Spontaneous Conversion of Lamellar Macular Holes to Full-Thickness Macular Holes: Clinical Features and Surgical Outcomes.

PURPOSE: To describe the clinical features and surgical outcomes of patients experiencing a spontaneous conversion of a lamellar macular hole (LMH) to a full-thickness macular hole (FTMH). DESIGN: Retrospective, multicenter, observational case series. PARTICIPANTS: Patients with LMH who experienced a spontaneous conversion to FTMH and underwent FTMH surgery. METHODS: Clinical charts and OCT features of 20 eyes of 20 patients were reviewed. MAIN OUTCOME MEASURES: OCT features and surgical outcomes of FTMH derived from LMH. RESULTS: The mean baseline visual acuity (VA) was 0.21 ± 0.19 logarithm of the minimum angle of resolution (logMAR) (20/32 Snellen equivalent [SE]). Epiretinal proliferation was noted in 18 eyes (90%), and 14 eyes (75%) had an epiretinal membrane. At the diagnosis of FTMH, the mean VA decreased to 0.61 ± 0.50 logMAR (20/81 SE) (P = 0.001). The mean FTMH diameter was 224.4 ± 194.8 µm, with 15 (75%) small (≤250 µm), 2 (10%) medium (>250-≤400 µm), and 3 (15%) large (>400 µm) FTMHs. Eighteen (90%) FTMHs were sealed after 1 surgery, and 2 (10%) required an additional procedure. At the last follow-up, the mean VA was increased to 0.29 ± 0.23 logMAR (20/38 SE) (P = 0.003), but did not significantly differ from the baseline VA (P = 0.071). CONCLUSIONS: Patients with LMH may develop an FTMH with no evidence of vitreomacular traction. A tangential traction from an epiretinal membrane may contribute to its genesis, but a progressive loss of retinal tissue and an inherent weakness of the foveal architecture in LMH eyes could be sufficient. Most FTMHs derived from LMH had a small diameter, showed epiretinal proliferation, showed limited retinal hydration, and were associated with relatively poor surgical outcomes compared with idiopathic FTMH.

DETECTION OF NEUROSENSORY RETINAL DETACHMENT COMPLICATING DEGENERATIVE RETINOSCHISIS BY ULTRA-WIDEFIELD FUNDUS AUTOFLUORESCENCE IMAGING.

PURPOSE: To determine whether neurosensory retinal detachment complicating degenerative retinoschisis (RS) can be reliably detected with ultra-widefield fundus autofluorescence evaluation. METHODS: Consecutive patients diagnosed with RS who had ultra-widefield fundus autofluorescence imaging were included in this retrospective case series. According to the fundus autofluorescence patterns, we divided the eyes into two groups: 1) eyes with RS and a hyperautofluorescent leading edge and 2) eyes with RS and without hyperautofluorescence. Peripheral spectral domain optical coherence tomography images at the level of RS were obtained. RESULTS: Thirty-eight eyes that met eligibility criteria were identified. Review of ultra-widefield fundus autofluorescence demonstrated 21/39 (55%) eyes with distinctive hyperautofluorescence over the area of RS (Group A) and 17/38 (45%) eyes without any form of hyperautofluorescence (Group B). Spectral domain optical coherence tomography images confirmed the presence of full-thickness neurosensory retina separation from the underlying retinal pigment epithelium in the areas of hyperautofluorescence in 10/10 eyes (100%) from Group A. None (0/11; 0%) of the eyes from Group B showed full-thickness neurosensory retina separation on the spectral domain optical coherence tomography imaging of the retina-RS interface. CONCLUSION: Hyperautofluorescent findings suggest the presence of a neurosensory retinal detachment. Retinal detachment associated with RS can be reliably detected on ultra-widefield fundus autofluorescence and may be a useful diagnostic imaging modality.

NREM Sleep and Antiepileptic Medications Modulate Epileptiform Activity by Altering Cortical Synchrony.

INTRODUCTION: The activating role of non-rapid eye movement (NREM) sleep on epileptic cortex and conversely, the seizure remission brought about by antiepileptic medications, has been attributed to their effects on neuronal synchrony. This study aims to understand the role of neural synchrony of NREM sleep in promoting interictal epileptiform discharges (IEDs) in patients with epilepsy (PWE) by assessing the peri-IED phase synchrony during awake and sleep states. It also studies the role played by antiepileptic drugs (AEDs) on EEG desynchronization in the above cohort. METHODS: A total of 120 PWE divided into 3 groups (each n = 40; juvenile myoclonic epilepsy [JME], temporal lobe epilepsy [TLE]. and extratemporal lobe epilepsy [Ex-TLE]) were subjected to overnight polysomnography. Each patient group was subdivided into drug-naive and on treatment (Each n = 20). EEG phase synchronization analysis was performed to compare peri-IED phase synchronization indices (SI) during awake and sleep stages and between drug naïve and on treatment groups in 4 frequency bands, namely delta, theta, alpha, and beta. The mean ± SD of peri-IED SI among various subgroups was compared employing a multilevel mixed effects modeling approach. RESULTS: Patients with JME had increased peri-IED cortical synchrony in N3 sleep stage, whereas patients with partial epilepsy had increased IED cortical synchrony in N1 sleep stage. On the other hand, peri-IED synchrony was lower during wake and REM sleep. We also found that peri-IED synchronization in patients with JME was higher in drug-naive patients compared with those on sodium valproate monotherapy in theta, alpha, and beta bands. CONCLUSION: The findings of this study suggest that sleep stages can alter cortical synchrony in patients with JME and focal epilepsy, with NREM IEDs being more synchronized and wake/REM IEDs being less synchronized. Furthermore, it also suggests that AEDs alleviate seizures in PWE by inhibiting cortical synchrony.

Heightened Background Cortical Synchrony in Patients With Epilepsy: EEG Phase Synchrony Analysis During Awake and Sleep Stages Using Novel Ensemble Measure.

INTRODUCTION: Excessive cortical synchrony within neural ensembles has been implicated as an important mechanism driving epileptiform activity. The current study measures and compares background electroencephalographic (EEG) phase synchronization in patients having various types of epilepsies and healthy controls during awake and sleep stages. METHODS: A total of 120 patients with epilepsy (PWE) subdivided into 3 groups (juvenile myoclonic epilepsy [JME], temporal lobe epilepsy [TLE], and extra-temporal lobe epilepsy [Ex-TLE]; n = 40 in each group) and 40 healthy controls were subjected to overnight polysomnography. EEG phase synchronization (SI) between the 8 EEG channels was assessed for delta, theta, alpha, sigma, and high beta frequency bands using ensemble measure on 10-second representative time windows and compared between patients and controls and also between awake and sleep stages. Mean ± SD of SI was compared using 2-way analysis of variance followed by pairwise comparison ( P ≤ .05). RESULTS: In both delta and theta bands, the SI was significantly higher in patients with JME, TLE, and Ex-TLE compared with controls, whereas in alpha, sigma, and high beta bands, SI was comparable between the groups. On comparison of SI between sleep stages, delta band: progressive increase in SI from wake ⇒ N1 ⇒ N2 ⇒ N3, whereas REM (rapid eye movement) was comparable to wake; theta band: decreased SI during N2 and increase during N3; alpha band: SI was highest in wake and lower in N1, N2, N3, and REM; and sigma and high beta bands: progressive increase in SI from wake ⇒ N1 ⇒ N2 ⇒ N3; however, sigma band showed lower SI during REM. CONCLUSION: This study found an increased background cortical synchronization in PWE compared with healthy controls in delta and theta bands during wake and sleep. This background hypersynchrony may be an important property of epileptogenic brain circuitry in PWE, which enables them to effortlessly generate a paroxysmal EEG depolarization shift.

Optical Coherence Tomography Angiography Reveals Choriocapillaris Flow Reduction in Placoid Chorioretinitis.

PURPOSE: To elucidate the origin of disease in acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and related placoid disorders, and to determine the principle tissue level of involvement: retinal pigment epithelium (RPE) versus choriocapillaris (CC). To determine the prevalence, extent, and persistence of choroidal flow reduction in placoid chorioretinitis using en face optical coherence tomography (OCT) and OCT angiography (OCTA). DESIGN: Multicenter, prospective case series. PARTICIPANTS: Patients with a clinical diagnosis of APMPPE, persistent placoid maculopathy (PPM), or relentless placoid chorioretinitis (RPC). METHODS: Morphologic evaluation of en face structural OCT and OCTA images with customized segmentation through the deep capillary plexus, outer nuclear layer, inner segment ellipsoid band, choriocapillaris, and outer choroid. MAIN OUTCOME MEASURES: Segmented images were graded by 3 masked readers with regard to reduction of flow and signal attenuation, and intergrader agreement was determined by mean unweighted kappa analysis. RESULTS: In this study, 24 eyes of 15 patients with APMPPE, PPM, or RPC were recruited and 60% of patients were male (N = 9) and the mean age was 33.6 years (range, 19-73 years). Of the 24 eyes, 96% (23/24) were graded as definite (18/24, 75%) or questionable (5/24, 21%) flow reduction within the choriocapillaris on OCTA, and 58% (14/24) were graded as definite decreased flow within the outer choroid. Mean weighted kappa analysis among readers was 0.655 for OCTA of the choriocapillaris and 0.684 for OCTA of the outer choroid. Areas of choriocapillaris flow deficit correlated closely with ischemic lesions seen with fluorescein angiography and indocyanine green angiography but were more extensive with OCTA and significantly improved with treatment or nontreatment follow-up. Corresponding zones of outer retinal disruption also were identified and colocalized with the areas of choriocapillaris flow reduction seen with OCTA. CONCLUSIONS: Optical coherence tomography angiography indicates that the inner choroid is the primary site of disease pathogenesis in APMPPE and related placoid disorders with secondary photoreceptor disruption. Optical coherence tomography angiography may be used to enhance diagnosis of placoid disorders and to monitor the progression of choriocapillaris ischemia and its response to therapy.

Phase Synchronization Analysis of Natural Wake and Sleep States in Healthy Individuals Using a Novel Ensemble Phase Synchronization Measure.

PURPOSE: Electrical activity in the brain is presumed to arise from a combination of tonic asynchronous neuronal firing during wake and a synchronized, burst-pause firing of large number of neurons during sleep. This study aims to compare the phase synchronization index (SI) across multiple channels during wake and various sleep stages on scalp electroencephalographic recordings. METHODS: Forty healthy subjects were subjected to overnight polysomnography using 8-channel electroencephalography. Electroencephalographic phase synchronization during awake, non-rapid eye movement (N1, N2, N3), and rapid eye movement sleep states was studied using ensemble measure (multichannel measure across all the eight channels based on Hilbert transformation between any two pairs). RESULTS: With the progression of states of wakefulness to non-rapid eye movement sleep, there was progressive increase in phase SI in delta band while SI decreased in alpha band (P < 0.001). The SI in delta band during rapid eye movement was comparable with that of awake state (P < 0.001). In theta band, SI tends to decrease in N2 and increase in N3 (P < 0.001). In beta band, there was progressive increase in SI from awake to non-rapid eye movement stages that decreased in rapid eye movement stage (P < 0.001). CONCLUSIONS: This is the first study that has used an ensemble measure to assess the long-range cortical phase synchronization during awake and various sleep stages. The findings support the previous view of increased delta synchrony during non-rapid eye movement sleep and alpha synchrony during wakefulness. Rapid eye movement stage was characterized by marked desynchrony in all frequency bands. These findings suggest the possible role of cortical synchronization in influencing the occurrence of epileptic activity during sleep and awake states.

RETINAL VEIN OCCLUSION IN A PATIENT WITH MATERNALLY INHERITED DIABETES AND DEAFNESS.

PURPOSE: To report a case of maternally inherited diabetes and deafness complicated by branch retinal vein occlusion and cystoid macular edema. METHODS: Retrospective case report. Multimodal imaging including spectral domain optical coherence tomography, en face optical coherence tomography, and fundus autofluorescence was preformed, and the findings are presented. FINDINGS: A 58-year-old female with a history of diabetes mellitus, hearing loss, and a previous diagnosis of age-related macular degeneration presented with decreased vision in the right eye. Clinical examination and multimodal imaging demonstrated a right inferior branch retinal vein occlusion complicated by cystoid macular edema and bilateral maculopathy suspicious for maternally inherited diabetes and deafness. Genetic testing confirmed an A3243G mitochondrial mutation. CONCLUSION: Multimodal retinal imaging is a key to guide diagnosis of rare genetic diseases such as maternally inherited diabetes and deafness. We report the unusual presentation of maternally inherited diabetes and deafness complicated by branch retinal vein occlusion and cystoid macular edema.

DIDANOSINE RETINAL TOXICITY.

PURPOSE: To report nine new cases of retinal degeneration secondary to didanosine toxicity and to summarize the previously reported cases in the literature. METHODS: This was a multicenter, retrospective, observational case study from seven institutions. Medical records of patients who demonstrated well-demarcated severe midperipheral chorioretinal degeneration and who were previously treated with didanosine therapy were collected and the following information was reviewed: age, gender, medical history, detailed medication history including current and previous antiretroviral use, ocular and retinal examination findings, and multimodal imaging findings with optical coherence tomography, fundus photography, wide-field fundus autofluorescence, and wide-field fluorescein angiography. When available, findings with electrophysiology testing and automated perimetry were also collected and reviewed. A literature review was also performed to collect all reported cases of chorioretinal degeneration secondary to didanosine toxicity. RESULTS: Nine patients were identified who had findings consistent with peripheral retinal toxicity secondary to didanosine use. Eight of the 9 patients were men, and the median age was 54 years at the time of presentation (mean: 55 years, range, 42-71 years). Snellen distance acuity ranged from 20/20 to 20/32. At least three of the cases in the series demonstrated progression of the peripheral retinal pigment epithelium and photoreceptor atrophy despite didanosine cessation. A review of the literature revealed 10 additional cases of didanosine toxicity. Seven of the 10 cases were in men (70%), and the average age was 26 years with a wide range (2-54 years). Chorioretinal findings were very similar to this cohort. CONCLUSION: Herein, we report the largest series of nine cases of peripheral chorioretinal degeneration secondary to didanosine toxicity in adults. When combined with the cases in the literature, 19 cases of didanosine toxicity, 4 of which occurred in children, were collected and analyzed. Three of the new cases presented showed clear progression of degeneration despite didanosine cessation. Newer nucleoside reverse transcriptase inhibitors may potentiate mitochondrial DNA damage and lead to continued chorioretinal degeneration.

Primary angiitis of the central nervous system presenting as unilateral optic neuritis.

A middle-aged woman who experienced recurrent episodes of unilateral vision loss and eye pain. On presentation, magnetic resonance imaging (MRI) demonstrated left optic nerve enhancement with patchy hyperintensities in the white matter of both frontal lobes and ill-defined enhancement in a lenticulostriate distribution. Ophthalmologic examination revealed left optic disc edema with a macular scar consistent with neuroretinitis. Her subsequent clinical course was notable for 2 episodes of painful vision loss, without associated neurologic symptoms, which resolved with intravenous and oral steroids. More than 1 year after her initial presentation, the patient developed right facial weakness and slurred speech, and shortly thereafter suffered a fatal intracerebral hemorrhage. Histopathology on autopsy confirmed a diagnosis of primary angiitis of the central nervous system (PACNS). This is an unusual case of PACNS presenting with recurrent unilateral optic neuritis. The vascular enhancement pattern on MRI suggesting inflamed cerebral blood vessels is a rarely described pattern, which likely reflects intracerebral extension of the ocular pathology. The combination of neuroretinitis and perivascular MRI enhancement pattern may represent a subtype of PACNS.