A carotid-cavernous fistula (CCF) is an abnormal communication between arteries and veins within the cavernous sinus and may be classified as either direct or dural. Direct CCFs are characterized by a direct connection between the internal carotid artery (ICA) and the cavernous sinus, whereas dural CCFs result from an indirect connection involving cavernous arterial branches and the cavernous sinus. Direct CCFs frequently are traumatic in origin and also may be caused by rupture of an ICA aneurysm within the cavernous sinus, Ehlers-Danlos syndrome type IV, or iatrogenic intervention. Causes of dural CCFs include hypertension, fibromuscular dysplasia, Ehlers-Danlos type IV, and dissection of the ICA. Evaluation of a suspected CCF often involves non-invasive imaging techniques, including standard tonometry, pneumotonometry, ultrasound, computed tomographic scanning and angiography, and/or magnetic resonance imaging and angiography, but the gold standard for classification and diagnosis remains digital subtraction angiography. When a direct CCF is confirmed, first-line treatment is endovascular intervention, which may be accomplished using detachable balloons, coils, liquid embolic agents, or a combination of these tools. As dural CCFs often resolve spontaneously, low-risk cases may be managed conservatively. When invasive treatment is warranted, endovascular intervention or stereotactic radiosurgery may be performed. Modern endovascular techniques offer the ability to successfully treat CCFs with a low morbidity and virtually no mortality.

IMPORTANCE Recent studies suggest that maintenance intravenous immunoglobulin (IVIG) may be an effective treatment to prevent relapses in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD); however, most of these studies had pediatric cohorts, and few studies have evaluated IVIG in adult patients.OBJECTIVE To determine the association of maintenance IVIG with the prevention of disease relapse in a large adult cohort of patients with MOGAD. DESIGN, SETTING, AND PARTICIPANTSThis was a retrospective cohort study conducted from January 1, 2010, to October 31, 2021. Patients were recruited from 14 hospitals in 9 countries and were included in the analysis if they (1) had a history of 1 or more central nervous system demyelinating attacks consistent with MOGAD, (2) had MOG-IgG seropositivity tested by cell-based assay, and (3) were age 18 years or older when starting IVIG treatment. These patients were retrospectively evaluated for a history of maintenance IVIG treatment. EXPOSURES Maintenance IVIG.MAIN OUTCOMES AND MEASURES Relapse rates while receiving maintenance IVIG compared with before initiation of therapy. RESULTSOf the 876 adult patients initially identified with MOGAD, 59 (median [range] age, 36 [18-69] years; 33 women [56%]) were treated with maintenance IVIG. IVIG was initiated as first-line immunotherapy in 15 patients (25%) and as second-line therapy in 37 patients (63%) owing to failure of prior immunotherapy and in 7 patients (12%) owing to intolerance to prior immunotherapy. The median (range) annualized relapse rate before IVIG treatment was 1.4 (0-6.1), compared with a median (range) annualized relapse rate while receiving IVIG of 0 (0-3) (t 108 = 7.14; P < .001). Twenty patients (34%) had at least 1 relapse while receiving IVIG with a median (range) time to first relapse of 1 (0.03-4.8) years, and 17 patients (29%) were treated with concomitant maintenance immunotherapy. Only 5 of 29 patients (17%) who received 1 g/kg of IVIG every 4 weeks or more experienced disease relapse compared with 15 of 30 patients (50%) treated with lower or less frequent dosing (hazard ratio, 3.31; 95% CI, 1.19-9.09; P = .02). At final follow-up, 52 patients (88%) were still receiving maintenance IVIG with a median (range) duration of 1.7 (0.5-9.9) years of therapy. Seven of 59 patients (12%) discontinued IVIG therapy: 4 (57%) for inefficacy, 2 (29%) for adverse effects, and 1 (14%) for a trial not receiving therapy after a period of disease inactivity.CONCLUSIONS AND RELEVANCE Results of this retrospective, multicenter, cohort study of adult patients with MOGAD suggest that maintenance IVIG was associated with a reduction in disease relapse. Less frequent and lower dosing of IVIG may be associated with treatment failure. Future prospective randomized clinical trials are warranted to confirm these findings.

Pain management is an important consideration in the promotion of patients' comfort. However, research continues to indicate patients' pain management is poor. The nursing literature cites nurses' lack of knowledge as a significant determinant of poor pain management practices. The impetus for this study arose from poor attendance by nurses at inservice sessions discussing pain assessment and management. Knowledge of existing nursing practice and accompanying beliefs and attitudes in relation to pain management is paramount in the development of relevant continuing education for registered nurses. The aim of this investigation was to study nurses' intention to treat pain in different patients. A 10-page questionnaire with eight different patient scenarios was distributed to 886 nurses across all clinical divisions of an acute tertiary facility. Results indicate knowledge deficits regarding optimum pain relief for patients. This article highlights the need for innovative teaching strategies and approaches in the clinical context to heighten nurses' awareness of their lack of knowledge of pain assessment and management.

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PurposeTo compare a new method for steady-state pattern electroretinogram (PERGx) with a validated method (PERGLA) in normal controls and in patients with optic neuropathy.MethodsPERGx and PERGLA were recorded in a mixed population (n = 33, 66 eyes) of younger controls (C1; n = 10, age 38 ± 8.3 years), older controls (C2; n = 11, 57.9 ± 8.09 years), patients with early manifest glaucoma (G; n = 7, 65.7 ±11.6 years), and patients with nonarteritic ischemic optic neuropathy (N; n = 5, mean age 59.4 ± 8.6 years). The PERGx stimulus was a black-white horizontal grating generated on a 14 × 14 cm LED display (1.6 cycles/deg, 15.63 reversals/s, 98% contrast, 800 cd/m2 mean luminance, 25° field). PERGx signal and noise were averaged over 1024 epochs (∼2 minutes) and Fourier analyzed to retrieve amplitude and phase. Partial averages (16 successive samples of 64 epochs each) were also analyzed to quantify progressive changes over recording time (adaptation).ResultsPERGLA and PERGx amplitudes and latencies were correlated (Amplitude R2 = 0.59, Latency R2 = 0.39, both P < 0.0001) and were similarly altered in disease. Compared to PERGLA, however, PERGx had shorter (16 ms) latency, higher (1.39×) amplitude, lower (0.37×) noise, and higher (4.2×) signal-to-noise ratio. PERGx displayed marked amplitude adaptation in C1 and C2 groups and no significant adaptation in G and N groups.ConclusionsThe PERGx high signal-to-noise ratio may allow meaningful recording in advanced stages of optic nerve disorders. In addition, it quantifies response adaptation, which may be selectively altered in glaucoma and optic neuropathy.Translational RelevanceA new PERG method with increased dynamic range allows recording of retinal ganglion cell function in advanced stages of optic nerve disorders. It also quantifies the response decline during the test, an autoregulatory adaptation to metabolic challenge that decreases with age and presence of disease.

IMPORTANCE Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Its incidence has been carefully studied in white populations, yet its relevance among other racial and ethnic groups is less well known. OBJECTIVE To examine the incidence of biopsy-proven GCA (BP-GCA) in a tertiary care center-based population with a sizeable proportion of black patients. DESIGN, SETTING, AND PARTICIPANTS This retrospective cohort study identified all patients who underwent temporal artery biopsy (TAB) from July 1, 2007, through September 30, 2017, using the electronic medical record system at the Johns Hopkins Wilmer Eye Institute. Associations between self-reported race, sex, and age were explored and compared with all other patients attending the hospital over the same period. Data were analyzed from November 1, 2017, through July 31, 2018. MAIN OUTCOMES AND MEASURES Estimated incidence rates of BP-GCA in black and white patients. RESULTS Among 586 patients who underwent TAB (mean [SD] age, 70.5 [11.1] years; age range, 32-103 years; 423 [72.2%] women), 167 (28.5%) were black, 382 (65.2%) were white, and 37 (6.3%) were other or unknown. Of 573 individuals 50 years and older, 92 (16.1%) had BP-GCA; 14 were black (8.4% of all black patients undergoing testing) and 75 were white (19.6% of all white patients undergoing testing). Crude annual incidence rates for BP-GCA were 2.9 (95% CI, 1.3-5.5) per 100 000 for black and 4.2 (95% CI, 3.0-5.6) per 100 000 for white patients within the study population. Population-adjusted age-and sex-standardized incidence rates were 3.1 (95% CI, 1.0-5.2) and 3.6 (95% CI, 2.5-4.7) per 100 000 for black and white patients, respectively (difference, 0.5; 95% CI, −1.7 to 2.7; P = .70). The incidence rate ratio was 1.9 in women compared with men (95% CI, 1.1-3.4; P = .03) but was not significant in white compared with black patients (1.2; 95% CI, 0.6-2.4; P = .66). CONCLUSIONS AND RELEVANCE In our cohort, BP-GCA occurred more commonly in women, but rates were similar between races. These findings do not appear to support the conclusion that GCA occurs more frequently in white compared with black patients.

Purpose Ocular funduscopic examination is difficult in young children and is rarely attempted by nonophthalmologists. Our objective was to determine the feasibility of reliably obtaining high-quality nonmydriatic fundus photographs in children. Methods Nonmydriatic fundus photographs were obtained in both eyes of children seen in a pediatric ophthalmology clinic. Ease of fundus photography was recorded on a 10-point Likert scale (10 = very easy). Quality was graded from 1 to 5 (1, inadequate for any diagnostic purpose; 2, unable to exclude all emergent findings; 3, only able to exclude emergent findings; 4, not ideal, but still able to exclude subtle findings; and 5, ideal quality). The primary outcome measure was image quality by age. Results A total of 878 photographs of 212 children (median age, 6 years; range,1-18 years) were included. Photographs of at least one eye were obtained in 190 children (89.6%) and in both eyes in 181 (85.3%). Median rating for ease of photography was 7. Photographs of some clinical value (grade ≥2) were obtained in 33% of children <3 years and 95% >3 years. High-quality photographs (grade 4 or 5) were obtained in both eyes in 7% of children <3 years, 57% of children ≥3 to <7 years, 85% of children ≥7 to <9 years, and 65% of children ≥9 years. The youngest patient with high-quality photographs in both eyes was 22 months. Conclusions Nonmydriatic fundus photographs of adequate quality can be obtained in children over age 3 and in some children as young as 22 months.

Background: Ophthalmic involvement in acute leukemia is common, with 36% of patients having ophthalmic involvement at the time of diagnosis. However, neuro-ophthalmic involvement is relatively rare. We present a characterization of neuro-ophthalmic findings in patients with acute leukemia and discuss the implications of these findings on patient management and prognosis. Methods: We performed a retrospective review of cases of acute leukemia with central nervous system (CNS) involvement and neuro-ophthalmic manifestations that were evaluated at the Wilmer Eye Institute between January 2013 and September 2019. Data collected included demographic information, leukemia details, results of diagnostic testing, and features of associated neuro-ophthalmic manifestations.Results: Twelve patients with mean age 42 years (range 9-65, median 39) were included. Seven (58%) patients were men and 5 (42%) women. Eight (67%) were diagnosed with acute myeloid leukemia and 4 (33%) with acute lymphoid leukemia. Neuro-ophthalmic findings included 4 patients with isolated sixth nerve palsies, 2 with multiple cranial nerve palsies, 2 with orbital lesions with proptosis, 4 with optic disc swelling, and 1 with isolated fourth nerve palsy. Five (42%) neuro-ophthalmic presentations were associated with known CNS disease, 3 (25%) were associated with active disease but heralded the discovery of CNS involvement, 3 (25%) were the presenting features of relapse, and 1 (8%) led to the original leukemia diagnosis. Neuroimaging showed 4 with leptomeningeal enhancement, 4 with cranial nerve enhancement/thickening, 3 with optic nerve/sheath enhancement, 1 with lytic lesion of bone, 1 with soft tissue mass, and 1 with cytotoxic brain edema. One case had normal neuroimaging. Overall, patients had a poor prognosis, with 7 patients dying from leukemia or its complications and only 1 achieving a sustained remission. In 58% of the cases in our series, the discovery of neuro-ophthalmic leukemic involvement directly led to a change in leukemia treatment.Conclusions: Neuro-ophthalmic manifestations of leukemia may occur as presenting features of diagnosis, relapse, or CNS involvement, and portend a poor prognosis. Detection of neuro-ophthalmic involvement often triggers a prompt change in management. Therefore, familiarity with potential neuro-ophthalmic presentations of acute leukemia may avoid delayed diagnosis, and resultant inadequate treatment, of primary disease, relapse, or CNS involvement.