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To highlight the challenge of correct reproductive and therapeutic counseling in complex pedigrees with different inherited retinal dystrophies (IRD).

Hintergrund: Ziel der vorliegenden Studie war die Auswertung der Fokusbreite zweier “EDOF” Intraokularlinsen (Lara, Symfony) im Vergleich zu einer monofokalen Linse (Asphina). Methoden: Multizentrische (9 Kliniken, 5 Länder), prospektive, randomisierte klinische Studie mit 428 [zum vollständigen Text gelangen Sie über die oben angegebene URL], Wintertagung der Berlin-Brandenburgischen Augenärztlichen Gesellschaft 2019

Póster presentado al 17º Congreso Nacional de la Sociedad Española de Neurociencia, celebrado en Alicante del 27 al 30 de septiembre de 2017., The stress system coordinates the adaptive responses of the organism to stressors of any kind. Dysfunction in this circuit may induce disturbances in emotional and cognitive aspects of several stress-related pathologies including depression. Thus, comprehension of dynamics and communication between these structures may lead to better understand neurobiological mechanism underlying anxiety disorders and also the beneficial effects of some treatments, such as deep brain stimulation (DBS). The main purpose of this work is to delve into interactions between several of the structures related to stress control evaluating changes in their oscillatory activity after applying to 2 different models of acute stress. In Experiment 1 Local Field Potentials (LFP) from the infralimbic division of the medial prefrontal cortex (IL), the basolateral division of the amygdala and the dorsal hippocampus, were recorded after the injection of corticotropin releasing hormone (CRH) in the dorsal raphe nucleus (DR). In Experiment 2, we analysed the effects of the electrical activation of the central nucleus of the amygdala (CeA) over the oscillatory dynamics of the IL. In addition, in Experiment 3 the effects of high-frequency electrical stimulation (DBS) targeting the nucleus accumbens core in the oscillatory activity of the infralimbic cortex were examined. Injection of CRH in the DR induced a general activation of the amygdala and the hippocampus, so a significant increment in theta waves power, as well as theta LFP coherence between these structures, was observed. Electrical activation of the CeA caused a delayed but significant change in IL, with prominent slow waves accompanied by an increase in theta activity, coupled with faster oscillations, including gamma and spindles. Interestingly, DBS of nucleus accumbens core generated a similar but not identical slow wave in the IL since spindles were not detected. These results are discussed in terms of processing of the stress response and memory formation., This work was supported by the Instituto de Salud Carlos III, Subdirección General de Evaluación y Fomento de la Investigación (FIS Grant PI13-00038 and FIS Grant PI16- 00217) that were co-funded by the European Regional Development Fund ('A way to build Europe'), and by the Generalitat Valenciana (Emergent groups grant GV/2016/103).

Growing evidence suggests that inflammation is involved in the progression of retinitis pigmentosa (RP) both in patients and in animal models. The aim of this study was to investigate the effect of Adalimumab, a monoclonal anti-TNF alpha antibody, on retinal degeneration in a murine model of human autosomal recessive RP, the rd10 mice at postnatal day (P) 18. In our housing conditions, rd10 retinas were seriously damaged at P18. Adalimumab reduced photoreceptor cell death, as determined by scoring the number of TUNEL-positive cells. In addition, nuclear poly (ADP) ribose (PAR) content, an indirect measure of PAR polymerase (PARP) activity, was also reduced after treatment. The blockade of TNF alpha ameliorated reactive gliosis, as visualized by decreased GFAP and IBA1 immunolabelling (Muller cell and microglial markers, respectively) and decreased up-regulation of TNF alpha gene expression. Adalimumab also improved antioxidant response by restoring total antioxidant capacity and superoxide dismutase activity. Finally, we observed that Adalimumab normalized energetic and metabolic pattern in rd10 mouse retinas. Our study suggests that the TNF alpha blockade could be a successful therapeutic approach to increase photoreceptor survival during the progression of RP. Further studies are needed to characterize its effect along the progression of the disease.

Objective. The aim of this study was to assess the efficacy of anti-TNF-alpha therapy in refractory uveitis due to Behcet's disease (BD). Methods. We performed a multicentre study of 124 patients with BD uveitis refractory to conventional treatment including high-dose corticosteroids and at least one standard immunosuppressive agent. Patients were treated for at least 12 months with infliximab (IFX) (3-5 mg/kg at 0, 2 and 6 weeks and then every 4-8 weeks) or adalimumab (ADA) (usually 40 mg every 2 weeks). The main outcome measures were degree of anterior and posterior chamber inflammation, visual acuity, macular thickness and immunosuppression load. Results. Sixty-eight men and 56 women (221 affected eyes) were studied. The mean age was 38.6 years (S.D. 10.4). HLA-B51 was positive in 66.1% of patients and uveitis was bilateral in 78.2%. IFX was the first biologic agent in 77 cases (62%) and ADA was first in 47 (38%). In most cases anti-TNF-alpha drugs were used in combination with conventional immunosuppressive drugs. At the onset of anti-TNF-alpha therapy, anterior chamber and vitreous inflammation was observed in 57% and 64.4% of patients, respectively. In both conditions the damage decreased significantly after 1 year. At baseline, 50 patients (80 eyes) had macular thickening [optical coherence tomography (OCT) >250 mu m] and 35 (49 eyes) had cystoid macular oedema (OCT > 300 mu m) that improved from 420 mm (S.D. 119.5) at baseline to 271 mu m (S.D. 45.6) at month 12 (P < 0.01). The best-corrected visual acuity and the suppression load also showed significant improvement. After 1 year of follow-up, 67.7% of patients were inactive. Biologic therapy was well tolerated in most cases. Conclusion. Anti-TNF-alpha therapy is effective and relatively safe in refractory BD uveitis.

To assess the efficacy and safety of adalimumab in patients with juvenile idiopathic arthritis (JIA) and associated refractory uveitis.

Retinitis Pigmentosa is a common form of hereditary retinal degeneration constituting the largest Mendelian genetic cause of blindness in the developed world. It has been widely suggested that oxidative stress possibly contributes to its pathogenesis. We measured the levels of total antioxidant capacity, free nitrotyrosine, thiobarbituric acid reactive substances (TBARS) formation, extracellular superoxide dismutase (SOD3) activity, protein, metabolites of the nitric oxide/cyclic GMP pathway, heme oxygenase-I and inducible nitric oxide synthase expression in aqueous humor or/and peripheral blood from fifty-six patients with retinitis pigmentosa and sixty subjects without systemic or ocular oxidative stress-related disease. Multivariate analysis of covariance revealed that retinitis pigmentosa alters ocular antioxidant defence machinery and the redox status in blood. Patients with retinitis pigmentosa present low total antioxidant capacity including reduced SOD3 activity and protein concentration in aqueous humor. Patients also show reduced SOD3 activity, increased TBARS formation and upregulation of the nitric oxide/cyclic GMP pathway in peripheral blood. Together these findings confirmed the hypothesis that patients with retinitis pigmentosa present reduced ocular antioxidant status. Moreover, these patients show changes in some oxidative-nitrosative markers in the peripheral blood. Further studies are needed to clarify the relationship between these peripheral markers and retinitis pigmentosa.

Tissue adhesives have been used in ophthalmology instead of sutures to minimize the operating time. This case series investigated the effectiveness and safety of use of cyanoacrylate in Ahmed valve implantation through pars plana for refractory glaucoma.

To evaluate adalimumab therapy in refractory uveitis.

The idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome is a rare retinal vascular disorder characterized by multiple leaking aneurysmal dilations, retinal vasculitis, neuroretinitis and peripheral vascular ischemia. Visual loss mainly occurs due to the development of retinal neovascularization and/or exudative maculopathy. Although the treatment of choice has not yet been established, retinal photocoagulation seems to be the best option to control the disease and to prevent its progression. Herein, we report a case of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome with both retinal neovascularization and macular exudation successfully managed with intravitreal ranibizumab (Lucentis(®)) as adjunctive therapy to retinal photocoagulation.

The cilium in photoreceptors appears ultrastructurally very similar to the nasal ciliated epithelium. The purpose of this study was to evaluate the nasal ciliary beat frequency and beat pattern in patients with retinitis pigmentosa (RP) and Usher syndrome type II and compare it with that of healthy control subjects.

The purpose of this study was to evaluate the levels of neutrophil gelatinase-associated lipocalin (NGAL) in the aqueous humor in eyes with idiopathic acute anterior uveitis (AAU).

To determine the level of hepatocyte growth factor (HGF) in the aqueous humor of patients with retinitis pigmentosa (RP).

To evaluate the efficacy and safety of the pars plana clip (PPC) in the Ahmed valve tube inserted via the pars plana in patients with secondary refractory glaucomas.

Objetivo: Determinar si la inyección intravítrea de plasmina es efectiva en el tratamiento de las membranas epirretinianas maculares (MEM) y el síndrome de tracción vitreomacular (STVM), sin necesidad de asociar vitrectomía quirúrgica. Material y método Diseño: Estudio piloto prospectivo, de intervención, serie de casos. Pacientes: Siete pacientes, cuatro con membrana epirretiniana macular y tres con síndrome de tracción vitreomacular. Tratamiento: Inyección intravítrea de 0,2 ml de plasmina autóloga bajo anestesia tópica. La plasmina fue obtenida por un método simplificado modificado por adicción de uroquinasa. Principales medidas: Grado de separación de la membrana epirretiniana de la retina medido por tomografía de coherencia óptica (OCT) y la mejor agudeza visual corregida (escala Snellen), antes y al mes de la inyección de plasmina. Resultados: Todos los pacientes completaron el seguimiento de 1 mes. La membrana epirretiniana (MEM) permaneció oftalmoscópicamente adherida, sin cambios ni separación del plano de la retina. Sin embargo, en los casos con síndrome de tracción vitreomacular (STVM), la membrana se despegó del área foveal, desapareciendo los pliegues retinianos traccionales secundarios, y normalizando la mácula la estructura anatómica. La agudeza visual no se modificó en ninguno de los casos de membrana epirretiniana y mejoró en los casos de síndrome de tracción vitreomacular. No se observaron efectos secundarios. Conclusión: La inyección intravítrea de plasmina autóloga como tratamiento inicial previo a la vitrectomía quirúrgica, en la serie de casos estudiada, parece ineficaz en las membranas epirretinianas maculares, y más eficaz en el síndrome de tracción vitreomacular, mejorando en este último la agudeza visual y consiguiendo el despegamiento al menos parcial de la membrana. Estudios más amplios son necesarios para confirmar su eficacia y la posibilidad de incrementar la misma con reinyecciones periódicas de plasmina. Purpose: To determine the effectiveness of intravitreal plasmin injection in the treatment of macular epiretinal membranes (MEM) and vitreomacular traction syndrome (VMTS) without associated pars plana vitrectomy. Methods: Design: Interventional, prospective, case series pilot study. Patients: Seven patients were enrolled in the study, 4 with MEM and 3 with VMTS. Treatment: 0.2 ml of autologous plasmin intravitreally injected under topical anesthesia was administered to all patients. The plasmin was obtained by a simplified method with urokinase. Main outcome measures: Degree of detachment of the MEM and the VMTS measured by optical coherence tomography (OCT), and the best corrected visual acuity (Snellen scale) before and one month after the plasmin injection. Results: The follow-up period was completed by all the patients. The MEM remained attached to the retina in all cases, as measured either by biomicroscopy or OCT. The VMTS was completely detached from the foveal area in all cases, with the disappearance of secondary tractional retinal folds and recovery of the normal macular anatomic architecture as measured by OCT. Visual acuity was not modified in any of the MEM patients, and improved in all VMTS patients. No adverse effects were observed. Conclusion: In our case series, intravitreally injected autologous plasmin was not effective in the treatment of MEM, but resolved VMTS successfully, improving the visual acuity and releasing the retinal traction without the need for associated pars plana vitrectomy. Larger studies to confirm the efficacy of this technique and the possibility of success after repeated injections are warranted.

Objetivo: Determinar si la inyección intravítrea de plasmina es efectiva en el tratamiento del edema macular diabético difuso (EMDD). Diseño: Estudio piloto prospectivo, comparativo, de intervención, serie de casos. Pacientes: Dieciocho pacientes con edema macular diabético bilateral, recibieron como primera actuación terapéutica una inyección intravítrea de plasmina en un ojo, sirviendo el ojo contralateral como control. Tratamiento: Inyección intravítrea de 0,2 ml de plasmina autóloga bajo anestesia tópica. La plasmina fue obtenida por un metodo simplificado. Principales medidas: Engrosamiento macular central (EMC) medido por tomografía de coherencia óptica (OCT) y agudeza visual (escala LogMAR), al mes y los 3 meses. Resultados: Todos los pacientes completaron el seguimiento de 3 meses. Antes de la inyección el EMC 525,22 DE 80,12 µm [media ± desviación estandard (DE)] en los ojos inyectados, versus 515,44 DE 78,13 µm en los ojos control. Un mes tras la inyección el EMC era 323,72 DE 44,87 µm en los ojos inyectados y 518,44 DE 78,54 µm en los ojos control (P < 0,001, test bilateral de Wilcoxon para muestras pareadas). A los tres meses tras la inyección el EMC era 310,55 DE 35,38 µm en los ojos inyectados y 517,66 DE 80 µm en los ojos control (P < 0,001). El edema macular mejoró en todos los ojos inyectados (100%), con una reducción al menos del 50%, mientras permaneció sin cambios en el grupo control. Nueve de los 18 ojos tratados (50%) ganaron al menos 2 líneas de visión, manteniéndose dicha mejoría durante los tres meses de seguimiento. No se observó en ningún paciente efectos secundarios. Conclusión: La inyección intravítrea de plasmina autóloga, como tratamiento de primera elección, mejora el edema macular diabético difuso y la agudeza visual. Estudios futuros son necesarios para confirmar su eficacia y seguridad a largo plazo. Purpose: To determine whether intravitreal injection of plasmin is effective in treating diffuse diabetic macular edema (DDME). Design: A prospective, comparative, interventional case study. Patients: Eighteen patients with bilateral DDME received, as their primary therapeutic treatment, an intravitreal injection of plasmin in one eye, with their contralateral untreated eye serving as a control. Intervention: Intravitreal 0.2 ml of autologous plasmin injected under topical anesthesia. The plasmin was obtained by a simplified method. Main Outcome Measures: Central macular thickness (CMT), determined by optical coherence tomography (OCT), and Best Corrected Visual Acuity (LogMAR), assessed at one and three months of follow-up. Results: All patients completed the 3-month follow-up assessments. Before the injection, the CMT was 525.22 SD 80.12 µm [mean ± standard deviation (SD)] in the eyes to be injected, compared to 525.44 SD 78.13 µm in the control eyes. One month after the injection, the CMT was 323.72 SD 44.87 µm in the injected eyes and 518.44 SD 78.54 µm in the control eyes (P < 0.001, bilateral Wilcoxon test for paired samples). Three months after the injection, the CMT was 310.55 SD 35.38 µm in the injected eyes and 517.66 SD 80 µm in the control eyes (P < 0.001). Macular edema improved in all injected eyes (100%), with a reduction of at least 50% in every treated eye, but no changes occurred in the control group. Nine of the 18 treated eyes (50%) improved their BCVA by at least two vision lines. No adverse effects were observed in any of the patients. Conclusion: Intravitreal plasmin injection, as primary treatment, effectively reduces macular thickening due to DDME and improves visual acuity. Further studies are warranted to assess long-term efficacy and safety.

Caso clínico: Mujer de 22 años que refería la aparición brusca de escotomas centrales en ambos ojos tras cuadro catarral de vías respiratorias altas. En el fondo del ojo (FO) presentaba unas manchas redondeadas parafoveales de color marrón rojizo. Los campos visuales presentaban unos escotomas centrales que se correspondían con la localización de las lesiones retinianas. Discusión: La neurorretinopatía macular aguda (NMA) es una uveítis posterior idiopática, poco frecuente, sin tratamiento efectivo y de la que existen diversas teorías sobre su patogenia. Clinical case: A 22-year-old woman complained of symptoms consistent with the sudden onset of central scotomas in both eyes after a flu-like illness. The typical reddish brown macular lesions of acute macular neuroretinopathy were evident in the parafoveal area of both eyes. Visual field examination revealed central scotomas in both eyes that corresponded in location to the retinal injuries. Discussion: Acute macular neuroretinopathy is a rare idiopathic posterior uveitis for which no effective treatment is available. Several pathogenic theories are postulated.