Background: Rituximab is a monoclonal antibody inducing depletion of B lymphocytes and presently approved for the treatment of non-Hodgkin's lymphoma and rheumatoid arthritis. Here is the first report of the use of this drug in a case of Cogan's syndrome (CS). Case Presentation: a 25-year-old Italian woman was referred with conjunctival hyperaemia, interstitial keratitis, moderate bilateral sensorineural hearing loss accompanied by tinnitus, dizziness, nausea and vertigo, poorly responsive to oral and topical steroidal therapy. Diagnosis of typical CS was made. The administration of a combined immunosuppressive treatment resolved ocular inflammation, dizziness, nausea, and vertigo but gave little results in controlling progressive hearing loss. A noticeable improvement in hearing function was documented by pure tone audiometry after infusion of Rituximab. Discussion: in CS, hearing function is often the most difficult parameter to control with therapy. A positive effect of Rituximab on was observed in our case. The drug also allowed to significantly reduce the number of adjuvant immunosuppressive medications.
Purpose. To compare the functional and clinical outcomes of the iris-claw intraocular lens (IOL) placed on the anterior versus posterior surface of the iris.Patients and Methods. A multicenter, retrospective study. Data on eyes that underwent anterior or retropupillary iris-claw IOL implantation because of inadequate capsular support secondary to complicated cataract surgery, trauma, and dislocated/opacified IOLs since January 2015 were analyzed. For study inclusion, evaluation results had to be available in the medical records both preoperatively and at 1 and 12 months after implantation. The following parameters were compared between the groups: best-corrected distance visual acuity (BCDVA), spherical and cylindrical refractive error, endothelial cell density (ECD), central macular thickness (CMT), and percentage and type of postoperative complications.Results. In total, 60 eyes of 60 patients aged 73 ± 13 years were included: 28 eyes (47%) involved anterior, and 32 eyes (53%) retropupillary, iris-claw IOL fixations. Preoperatively, the groups were similar in all parameters except for a significantly higher proportion of retropupillary fixations in patients who had previously experienced a closed-globe trauma (). The groups showed comparable improvements in BCDVA after surgery (final BCDVA: 0.34 ± 0.45 vs. 0.37 ± 0.50 logMAR in the anterior and retropupillary placement groups, respectively). During follow-up, no group difference was observed in refractive error or CMT. Both groups experienced similarly marked ECD loss and showed similar incidence of postoperative complications, with cystoid macular edema being the most common complication. Multivariable linear regression showed that BCDVA at 1 month was the best predictor of the final BCDVA.Conclusions. Anterior chamber and posterior chamber iris-claw IOL fixations proved equally effective and safe for aphakic correction in eyes with inadequate capsular support.
The results of canaloplasty (CP) and Hydrus Microstent (HM) implantation were retrospectively compared at 24 months’ follow-up in a cohort of subjects referred to our Institution for uncontrolled IOP in primary or secondary (e.g., pseudoexfoliative and pigmentary) open-angle glaucoma. The outcome was labelled as “complete” success, “qualified” success, or “failure” if, two years after surgery, the eyes operated on needed “no” hypotensive medications, “some” hypotensive medications, or further glaucoma surgery to attain the target IOP, respectively. Both CP and HM implant allowed significant IOP reductions, with comparable rate of clinical success and safety profile. A slightly (albeit not significant) better trend for a “complete” clinical success was observed in the CP group.
1 January 2013 in Orphanet Journal of Rare Diseases
Neuro-Behcet's disease (NBD) involves the central nervous system; peripheral nervous system involvement is not often reported. NBD is quite common in adult patients and occurs rarely during childhood and adolescence. Young patients may share symptoms and signs of NBD with other neuro-ophthalmological disorders (e.g. idiopathic intracranial hypertension); thus, making the differential diagnosis difficult. Neuroimaging is mandatory and necessary for a correct NBD diagnosis but in children radiological examinations are often difficult to perform without sedation. From 1971 to 2011, 130 patients aged <=16 years have been reported with NBD, according to retrospective surveys, case series, and case reports. The origin of the reported cases met the well-known geographical distribution of Behcet's disease (BD); the mean age at presentation of neurological findings was 11.8 years, with male gender prevalence (ratio, 2.9:1). We considered in detail the neuro-ophthalmological features of the 53 cases whose neuroimaging alterations were described with an assigned radiological pattern of the disease (parenchymal: 14 cases, non-parechymal: 35 cases, and mixed: 4 cases). In 19/53 patients (36%), neuro-ophthalmological symptoms anticipated any pathognomonic sign for a BD diagnosis, or only occasional aphtae were recalled by the patients. Family history was positive in 17% of subjects. Headache was reported in 75% of the patients; in those presenting with cerebral vascular involvement, headache was combined to other symptoms of intracranial hypertension. Papilledema was the most frequently reported ophthalmological finding, followed by posterior uveitis. Treatment consisted of systemic steroids in 93% of patients, often combined with other immunosuppressive drugs (especially colchicine and azathioprine). Clinical recovery or improvement was documented in the large majority of patients. Nine subjects had definitive alterations, and one died. Based on our review and personal experience, a delayed diagnosis, and the consequently delayed immunosuppressive treatment, may favour permanent sequelae, in particular, optic atrophy.
Background/aims The study involved eyes affected by pigment dispersion syndrome (PDS) or pigmentary glaucoma (PG) investigated by ultrasound biomicroscopy (UBM). Different irido-corneal parameters were assessed and compared with those from healthy controls. The aim was to investigate the capacity of the UBM in differentiating the cases and, potentially, in confirming the pathogenic mechanisms. Methods Patients with a first diagnosis of PDS or PG were included. A cohort of healthy volunteers matched for sex, age and refractive errors was recruited. All underwent UBM examination: the following parameters were assessed in relaxed and stimulated accommodative state in one eye: iris–lens contact (ILC), irido-corneal angle (ICA) and iris concavity (IC). A receiver operating characteristic (ROC) analysis assessed the ability of UBM to discriminate between subjects with and without PDS/PG. Results There were 24 eyes in the case group: four diagnosed as PG and the remaining 20 as PDS. There were 25 eyes in the control group. The two groups were statistically superimposable except for baseline intraocular pressure, which was higher in the case group (p=0.0001). All UBM parameters were statistically different between the two groups. ICA in near vision was the best-performing parameter, reaching a sensitivity (=specificity) of 0.875 with a cut-off at 53.0°. The second most sensitive parameter was IC, still in near vision. Conclusion All UBM parameters examined were statistically different between the two groups. ROC analysis showed ICA and IC in near vision to be the most discriminatory parameters. This evidence confirms the importance of iris movements in inducing the particular features of PDS/PG.