- [Impact of mydriatic eyedrops on distance visual acuity in patients with exudative age-related macular degeneration]. [Journal Article]
- JFJ Fr Ophtalmol 2019 Jun 13
- CONCLUSIONS: The absence of a clinically significant loss in post-dilation distance VA for exudative AMD could be explained by negligible glare coming from the ETDRS chart, milder photophobia, low pre-dilation VA's and a balance between higher order optical aberrations and diffraction. The opposite result for near VA could essentially be explained by greater glare induced by the light illuminating the Parinaud chart.Our primary goal was not achieved. A study presuming the absence of a clinically significant decrease in post-dilation distance VA would be necessary to consider directly measuring post-dilation VA in eyes with exudative AMD in our daily practice.
- Clinical and Molecular Characterization of PROM1-Related Retinal Degeneration. [Journal Article]
- JNJAMA Netw Open 2019 Jun 05; 2(6):e195752
- CONCLUSIONS: In this case-series study, PROM1 recessive variants were associated with early-onset, severe panretinal degeneration. The similar phenotypes observed in patients with homozygous missense variants and splice site variants compared with similarly aged patients with truncating variants suggests that all recessive variants have a null (or loss of function close to null) outcome on PROM1 function. In contrast, the dominant missense cases were associated with a milder, cone-driven phenotype, suggesting that the dominant disease is preferentially associated with cones. This has implications for the development of treatments for this severely blinding disease, and adeno-associated viral vector-based gene therapy and optogenetics could become successful treatment options.
- Efficacy of atropine 0.01% for the treatment of childhood myopia in European patients. [Journal Article]
- AOActa Ophthalmol 2019 Jun 13
- CONCLUSIONS: Low-dose atropine significantly slowed the rate of myopia progression in European paediatric patients with a favourable safety profile.
- GeneReviews®: Alström Syndrome [BOOK]
- BOOKUniversity of Washington, Seattle: Seattle (WA)
- Alström syndrome is characterized by cone-rod dystrophy, obesity, progressive bilateral sensorineural hearing impairment, acute infantile-onset cardiomyopathy and/or adolescent- or adult-onset restri…
Alström syndrome is characterized by cone-rod dystrophy, obesity, progressive bilateral sensorineural hearing impairment, acute infantile-onset cardiomyopathy and/or adolescent- or adult-onset restrictive cardiomyopathy, insulin resistance / type 2 diabetes mellitus (T2DM), non-alcoholic fatty liver disease (NAFLD), and chronic progressive kidney disease. Cone-rod dystrophy presents as progressive visual impairment, photophobia, and nystagmus usually starting between birth and age 15 months. Many individuals lose all perception of light by the end of the second decade, but a minority retain the ability to read large print into the third decade. Children usually have normal birth weight but develop truncal obesity during their first year. Sensorineural hearing loss presents in the first decade in as many as 70% of individuals and may progress to the severe or moderately severe range (40-70 db) by the end of the first to second decade. Insulin resistance is typically accompanied by the skin changes of acanthosis nigricans, and proceeds to T2DM in the majority by the third decade. Nearly all demonstrate hypertriglyceridemia. Other findings can include endocrine abnormalities (hypothyroidism, hypogonadotropic hypogonadism in males, and hyperandrogenism in females), urologic dysfunction / detrusor instability, progressive decrease in renal function, and hepatic disease (ranging from elevated transaminases to steatohepatitis/NAFLD). Approximately 20% of affected individuals have delay in early developmental milestones, most commonly in gross and fine motor skills. About 30% have a learning disability. Cognitive impairment (IQ <70) is very rare. Wide clinical variability is observed among affected individuals, even within the same family.
- Early administration of adalimumab for paediatric uveitis due to Behçet's disease. [Journal Article]
- PRPediatr Rheumatol Online J 2019 Jun 10; 17(1):29
- CONCLUSIONS: Adalimumab is effective for treating children with Behçet's disease-associated uveitis. Control of ocular inflammation was achieved without local and systemic corticosteroid, thus preventing further complications.
- Implication of Melanopsin and Trigeminal Neural Pathways in Blue Light Photosensitivity in vivo. [Journal Article]
- FNFront Neurosci 2019; 13:497
- Photophobia may arise from various causes and frequently accompanies numerous ocular diseases. In modern highly illuminated world, complaints about greater photosensitivity to blue light increasingly…
Photophobia may arise from various causes and frequently accompanies numerous ocular diseases. In modern highly illuminated world, complaints about greater photosensitivity to blue light increasingly appear. However, the pathophysiology of photophobia is still debated. In the present work, we investigated in vivo the role of various neural pathways potentially implicated in blue-light aversion. Moreover, we studied the light-induced neuroinflammatory processes on the ocular surface and in the trigeminal pathways. Adult male C57BL/6J mice were exposed either to blue (400-500 nm) or to yellow (530-710 nm) LED light (3 h, 6 mW/cm2). Photosensitivity was measured as the time spent in dark or illuminated parts of the cage. Pharmacological treatments were applied: topical instillation of atropine, pilocarpine or oxybuprocaine, intravitreal injection of lidocaine, norepinephrine or "blocker" of the visual photoreceptor transmission, and intraperitoneal injection of a melanopsin antagonist. Clinical evaluations (ocular surface state, corneal mechanical sensitivity and tear quantity) were performed directly after exposure to light and after 3 days of recovery in standard light conditions. Trigeminal ganglia (TGs), brainstems and retinas were dissected out and conditioned for analyses. Mice demonstrated strong aversion to blue but not to yellow light. The only drug that significantly decreased the blue-light aversion was the intraperitoneally injected melanopsin antagonist. After blue-light exposure, dry-eye-related inflammatory signs were observed, notably after 3 days of recovery. In the retina, we observed the increased immunoreactivity for GFAP, ATF3, and Iba1; these data were corroborated by RT-qPCR. Moreover, retinal visual and non-visual photopigments distribution was altered. In the trigeminal pathway, we detected the increased mRNA expression of cFOS and ATF3 as well as alterations in cytokines' levels. Thus, the wavelength-dependent light aversion was mainly mediated by melanopsin-containing cells, most likely in the retina. Other potential pathways of light reception were also discussed. The phototoxic message was transmitted to the trigeminal system, inducing both inflammation at the ocular surface and stress in the retina. Further investigations of retina-TG connections are needed.
- Increased pupillary constriction velocity in benign essential blepharospasm associated with photophobia. [Journal Article]
- PlosPLoS One 2019; 14(6):e0217924
- We evaluated whether the pupillary light reflex is altered in benign essential blepharospasm patients. Twenty-three patients with benign essential blepharospasm, 47 with reflex blepharospasm, and 29 …
We evaluated whether the pupillary light reflex is altered in benign essential blepharospasm patients. Twenty-three patients with benign essential blepharospasm, 47 with reflex blepharospasm, and 29 dry eye disease controls were included. Pupillary light reflex-related parameters were measured under mesopic (10 lux) and photopic illuminance (200 lux) using an infrared pupillometer. Additionally, we assessed photophobia grade, eyelid function, and dry eye disease-related parameters. There were no differences in age, sex predominance, or dry eye disease-related parameters among the three groups, or in photophobia grade and eyelid function between benign essential blepharospasm and reflex groups. Constriction velocity and maximum constriction velocity in the mesopic condition were significantly greater in the benign essential blepharospasm group (3.26 ± 0.56 and 5.27 ± 0.90 mm/s) than in reflex (2.86 ± 0.62 and 4.59 ± 1.00 mm/s) or dry eye disease groups (2.96 ± 0.46 and 4.72 ± 0.67 mm/s). Constriction velocity and maximum constriction velocity in the mesopic condition positively correlated with photophobia grade (r = 0.525 and 0.617, P = 0.025 and 0.006) in the benign essential blepharospasm group. Pupillary light reflex may be related to the pathophysiology of benign essential blepharospasm with photophobia. Further studies are required to reveal connections among pupillary light reflex, photophobia, and focal dystonia in benign essential blepharospasm patients with photophobia.
- Development of Acute Vogt-Koyanagi-Harada-like Syndrome during the Treatment Course with Vemurafenib for Metastatic Melanoma. [Journal Article]
- OIOcul Immunol Inflamm 2019 Jun 04; :1-4
- CONCLUSIONS: Vemurafenib, a potential adjunct treatment for metastatic melanoma, was complicated by the development of panuveitis, papillitis, and multiple serous detachments. These ocular symptoms were similar to the presentation of acute VKH syndrome.
- Aflibercept-Related Sterile Intraocular Inflammation Outcomes. [Journal Article]
- OROphthalmol Retina 2019 Apr 11
- CONCLUSIONS: This is the largest study of aflibercept-related sterile intraocular inflammation, and is the only large report to exclude eyes injected with intraocular antibiotics. Most patients presented early with decreased VA and intraocular inflammation, but without injection, hypopyon, fibrin, or severe pain. Final VA remained decreased in a significant minority of patients.
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- Salzmann nodular degeneration features in a case of Kabuki make-up syndrome. [Journal Article]
- BCBMJ Case Rep 2019 May 30; 12(5)
- Kabuki syndrome (KS) is a multiple congenital anomaly syndrome with diversified ophthalmological manifestations. We report a case of a boy with bilateral features of Salzmann nodular degeneration (SN…
Kabuki syndrome (KS) is a multiple congenital anomaly syndrome with diversified ophthalmological manifestations. We report a case of a boy with bilateral features of Salzmann nodular degeneration (SND) associated with KS. An 18-year-old Caucasian man with KS presented for a second opinion regarding incapacitating photophobia in his right eye, refractory to medical therapy. Biomicroscopy revealed bilateral subepithelial nodules in the midperiphery of the cornea, less extensive in the left eye, consistent with SND. Symptomatic improvement was achieved after superficial keratectomy, manually performed with a blade and adjuvant application of mitomycin C. We report a rare case of a KS patient with SND. Since KS manifestations may vary widely, it is important to perform an early ophthalmological examination for prompt detection and treatment of ocular abnormalities and thus improve life quality in these patients.