Guanfacine for the Treatment of Attention-Deficit/Hyperactivity Disorder in Children and Adolescents with Down Syndrome: A Retrospective Chart Review.

Study Design: Retrospective case series. Objectives: The objective of this study was to provide naturalistic data on the use of guanfacine for the treatment of attention-deficit/hyperactivity disorder (ADHD) in a clinically referred sample of youth with Down syndrome (DS). Methods: The medical records of children and adolescents with DS who received guanfacine for the treatment of ADHD from a multidisciplinary neurodevelopmental disorder clinic between September 1, 2011, and September 10, 2021, were reviewed. Demographic and clinical characteristics, guanfacine dose and treatment duration, and adverse effects were recorded. Clinical Global Impression Scale (CGI) scores for ADHD symptom severity (S) and improvement (I) were retrospectively assigned by a child and adolescent psychiatrist based on review of the clinic notes. Response to guanfacine was defined as completion of at least 12 weeks of treatment and a Clinical Global Impression Improvement subscale rating ≤2 (1 = "very much improved" or 2 = "much improved"). Results: Twenty-one patients were eligible for inclusion, of whom 17 (81%) completed at least 12 weeks of guanfacine. Ten of the 21 patients (48%; 95% confidence interval [CI]: 28%-68%) responded to treatment. The median time on guanfacine treatment covered by the clinic notes was 50.4 weeks, with a range of 0.3 weeks to 7.5 years. Thirteen patients (62%) remained on guanfacine at the time of their most recent clinic note. Nine patients had adverse events documented in their clinic notes (43%; 95% CI: 24%-63%), most commonly sleepiness (n = 7) and constipation (n = 2). Conclusion: About half of patients with DS responded to guanfacine for the treatment of ADHD and many tolerated long-term use. Study limitations primarily relate to the retrospective nature of the study and small sample size.

Multimodal imaging of type 2 acute macular neuroretinopathy in a young woman.

We report a case of type 2 acute macular neuroretinopathy (AMN) that occurred in an otherwise healthy 22-year-old white woman taking oral contraceptives and consuming large quantities of caffeinated coffee. The patient presented with a teardrop-shaped scotoma just inferior to her central vision in her left eye after a recent and significant increase in coffee consumption. A small extrafoveal retinal lesion was present superior to the fovea on pseudocolor fundus photography. Multimodal retinal imaging demonstrated focal disruption of the inner segment-outer segment junction of the photoreceptors with overlying hyperreflectivity at the level of the outer plexiform layer superior to the fovea, consistent with a diagnosis of type 2 AMN. Oral contraceptive use and high caffeine intake may be risk factors for the development of type 2 AMN.

Choroidal Structural Analysis in Alzheimer Disease, Mild Cognitive Impairment, and Cognitively Healthy Controls.

PURPOSE: To assess choroidal structural parameters in symptomatic Alzheimer disease (AD), mild cognitive impairment (MCI), and cognitively healthy control subjects. DESIGN: Prospective cross-sectional study. METHODS: This study took place in an outpatient neurological disorders clinic. Participants included 67 patients (112 eyes) with AD, 74 patients (143 eyes) with MCI, and 137 (248 eyes) control subjects. Subjects with diabetes, glaucoma, or retinal pathology were excluded. High-definition enhanced depth imaging foveal scans were obtained using Zeiss Cirrus HD-5000 AngioPlex. Subfoveal choroidal thickness (SFCT) was measured by 2 masked graders with a third adjudicator. Total choroidal area (TCA), luminal area (LA), and the choroidal vascularity index (CVI) were calculated after image binarization. Association of choroidal parameters with AD, MCI, or control subjects was assessed using multivariable generalized estimating equations, adjusted for age, sex, and visual acuity. RESULTS: After adjustment for age, sex, and visual acuity, TCA was significantly greater in patients with AD (ß = 2.73; p = .001) and MCI (ß = 4.38; p < .001) compared with control subjects. LA was significantly greater in patients with AD (ß = 1.68; p = .001) and MCI (ß = 2.69; p < .001) compared with control subjects, and CVI was significantly lower in patients with MCI (ß = -0.58; p = .002) compared with control subjects. SFCT was similar among patients with AD and MCI and control subjects on multivariable analysis (p > .05). CONCLUSIONS: TCA, LA, and CVI may differ between patients with AD, MCI, and healthy cognition, whereas SFCT may not differ among these groups. TCA, LA, and CVI deserve further study in subjects on the Alzheimer continuum.

Association of OCT Angiography Parameters With Age in Cognitively Healthy Older Adults.

BACKGROUND AND OBJECTIVE: To evaluate the association of changes in retinal anatomy and microvasculature with age and sex in cognitively healthy older adults. PATIENTS AND METHODS: Cross-sectional study of cognitively healthy subjects aged 50 years and older who underwent optical coherence tomography angiography (OCTA) to estimate the association between age and sex with ganglion cell layer-inner plexiform layer (GC-IPL); central subfield thickness (CST); subfoveal choroidal thickness (CT); foveal avascular zone (FAZ) size; and superficial (SCP), deep (DCP), and whole capillary plexus (WCP) vessel density (VD) and perfusion density (PD) measured in the ETDRS 3-mm and 6-mm circle and rings. RESULTS: Among 141 older adults (72.9% female; median age: 69 years), 282 eyes were imaged. Females had a greater CT, GC-IPL thickness, and FAZ size and a lower CST than males. After controlling for sex, both CT (P = .001) and GC-IPL thickness (P < .001) decreased with age, whereas FAZ size and CST did not. There was a reduction in VD and PD in SCP, DCP, and WCP with age in the 3-mm circle, 3-mm ring, and 6-mm circle (all P < .05). CONCLUSIONS: There is a significant reduction in both VD and PD, as well as decreased choroidal and GC-IPL thickness associated with aging, even beyond the fifth decade, in cognitively healthy adults. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:706-714.].

Prompt Improvement of an Enlarging Pigment Epithelial Detachment Following Intravitreal Dexamethasone in Neovascular Age-Related Macular Degeneration.

Purpose: This report discusses a case of neovascular age-related macular degeneration with mixed serous pigment epithelial detachment (PED) that was unresponsive to antivascular endothelial growth injections but responded to the addition of intravitreal steroid injection. Methods: We report a case. Results: A 75-year-old woman with neovascular age-related macular degeneration developed a mixed serous PED in her right eye. Three monthly aflibercept (Eylea, Regeneron) treatments led to resolution of associated intraretinal fluid; however, the mixed serous and fibrovascular PED significantly increased in size. The addition of intravitreal dexamethasone (Ozurdex, Allergan) to monthly aflibercept led to prompt flattening of the PED. Three months later, the PED recurred despite continued administration of monthly aflibercept. Conclusions: This case supports a growing body of research that PEDs unresponsive to antivascular endothelial growth injections may respond to intravitreal steroid injections.

Impact and Implication of Fovea-Involving Intraretinal Hemorrhage after Acute Branch Retinal Vein Occlusion.

PURPOSE: To compare clinical outcomes in patients with branch retinal vein occlusion (BRVO) with (group A) or without (group B) fovea-involving intraretinal hemorrhage (IRH). DESIGN: Retrospective cohort study. PARTICIPANTS: All patients diagnosed with acute, treatment-naive BRVO seen by the Duke Eye Center Retina Service from January 1, 2009, through June 30, 2017 who had treatment-naive BRVO with disease onset <3 months before presentation, macular involvement, spectral-domain OCT and color fundus photographs at presentation, and >12 months offollow-up. METHODS: Retrospective study using a database of patients diagnosed with BRVO over an 8-year period. The presence of fovea-involving IRH was determined from baseline fundus photographs by human graders and confirmed with multimodal imaging. Presenting features, treatment patterns, and clinical outcomes were compared. MAIN OUTCOME MEASURES: Visual acuity (VA), cystoid macular edema (CME), central subfield thickness (CST), and number of anti-vascular endothelial growth factor (VEGF) injections. RESULTS: Of 172 patients with BRVO, 33 (19.2%) presented with fovea-involving IRH. At presentation, group A had worse VA (0.54±0.06 logMAR [Snellen equivalent, 20/69] vs. 0.34±0.03 logMAR [Snellen equivalent, 20/44]; P = 0.001), greater CST (523.8±32 µm vs. 345.9±11.8 µm; P < 0.001), were more likely to have CME (93.9% vs. 48.2%; P < 0.001), and received more anti-VEGF injections in the first year (4.50±3.43 vs. 1.89±3.26; P < 0.001) than group B. Final VA was worse in group A (0.57±0.12 logMAR [Snellen equivalent, 20/74] vs. 0.35±0.05 logMAR [Snellen equivalent, 20/45]; P = 0.05). More patients in group A had loss of >2 lines of VA (36.4% vs. 18.7%; P = 0.04) or >3 lines (27.3% vs. 10.8%; P = 0.05) at final follow-up. Group A was more likely to have CME (63.6% vs. 27.3%; P < 0.001) at final follow-up with greater treatment burden, yet experienced a greater decrease in CST (-197.8±45.3 µm vs. -51.7±14.7 µm; P = 0.005). CONCLUSIONS: Acute BRVO presenting with fovea-involving IRH is associated with worse presenting features, greater treatment burden, and worse clinical outcomes despite current therapeutic interventions.