Olsonnoble4823

The full-field electroretinography results were subnormal.

It was indicated that the novel

mutations identified may be strongly correlated with binocular ARB. This study provides significant information of the genotype-phenotype association in Japanese ARB patients. Further, the genetic analysis that we performed was very useful for the differential diagnosis and might have implications in the development of future treatment modalities.

It was indicated that the novel BEST1 mutations identified may be strongly correlated with binocular ARB. This study provides significant information of the genotype-phenotype association in Japanese ARB patients. Further, the genetic analysis that we performed was very useful for the differential diagnosis and might have implications in the development of future treatment modalities.

This prospective study evaluates whether rituximab is a safe and potentially effective treatment for nonparaneoplastic autoimmune retinopathy (npAIR).

Five npAIR patients were enrolled in a Phase I/II, prospective, nonrandomized, open-label, single-center study. All patients received a cycle of 1000 mg intravenous rituximab at weeks 0 and 2, with a second cycle of rituximab 6 to 9 months later. Clinical evaluation was performed at baseline, 6 and 12 weeks after each rituximab cycle, and then every 3 months for a total duration of 18 months. The primary outcome for this study was treatment success based on visual field and full-field electroretinography at 6 months. The secondary outcomes included treatment success at months 12 and 18, drug-related adverse events, changes in visual symptoms, and changes in quality of life.

Two patients met criteria for treatment success one based solely on electroretinography and the other based solely on visual field area, but treatment success was not sustained. Clinical response over the course of the 18-month study showed disease stabilization in three patients and treatment failure in two patients. There were no severe drug-related adverse events.

This is the first clinical trial prospectively evaluating the effect of rituximab in npAIR and, although rituximab was well tolerated, there was no clear-cut clinical improvement conferred by B cell depletion with rituximab.

This is the first clinical trial prospectively evaluating the effect of rituximab in npAIR and, although rituximab was well tolerated, there was no clear-cut clinical improvement conferred by B cell depletion with rituximab.

Reports of morning glory disc anomaly (MGDA) in India have mostly been case reports. The aim of this study was to describe the demographic and clinical profile of patients with MGDA in South India.

A retrospective review of the medical records of patients with MGDA seen at a tertiary eye hospital in South India over a period of 8 years was carried out. The patients' demographic and clinical data were extracted from the case files and were entered into Epi Info reporting software version 7.2.3.0 and then analyzed.

There were 51 eyes of 44 patients with MGDA comprised 25 (56.8%) males and 19 (43.2%) females. Seven (15.9%) patients had bilateral MGDA. The mean age for females was 5.8 years (standard deviation [SD] 5.8) and for males, 11.2 years (SD 12.1). This difference was not statistically significant with a

= 0.07. The most common ocular associations were strabismus, refractive error, and retinal detachment, whereas the most common systemic associations were cleft lip and cleft palate. Fifty-one percent of eyes were blind at presentation.

Patients with MGDA in India tend to present late with poor visual prognosis. Early diagnosis and prompt treatment of blinding complications are crucial in reducing the risk of irreversible visual loss. Associated systemic abnormalities highlight the importance of a multidisciplinary approach in the management of patients with this condition.

Patients with MGDA in India tend to present late with poor visual prognosis. Early diagnosis and prompt treatment of blinding complications are crucial in reducing the risk of irreversible visual loss. Associated systemic abnormalities highlight the importance of a multidisciplinary approach in the management of patients with this condition.

The purpose of this study was to evaluate whether papilledema severity is associated with specific demographic or clinical factors in patients with idiopathic intracranial hypertension (IIH).

A retrospective cohort study of consecutive IIH patients seen at one tertiary care institution between 1989 and March 31, 2017 was performed. IIH patients were classified as mild (Frisén Grade 1 or 2) or severe (Frisén Grade 4 or 5) based on grading of fundus photographs obtained at first presentation. Demographic and clinical variables including age, body mass index (BMI), gender, visual acuity, Humphrey visual field mean deviation, and cerebrospinal fluid (CSF) opening pressure were extracted from patient medical records for statistical analyses.

A total of 239 patients were included in the study 152 with mild papilledema and 87 with severe papilledema. There was no difference in age, race, BMI, or male gender between the mild and severe papilledema groups. CSF opening pressure was significantly higher in the severe papilledema group (41.89 cm of water vs. 33.69, 95% confidence interval [CI] -10.79--5.62,

< 0.0001). There was a significant difference in the Humphrey mean deviation (-6.38 dB compared to - 3.25 dB, 95% CI -4.82--1.44 dB,

< 0.001) and average logarithm of the minimum angle of resolution visual acuity at final follow-up (0.21 vs. 0.045, 95% CI -0.299--0.040 ,

= 0.01).

Age, race, sex, and BMI were similar in IIH patients with mild versus severe papilledema, emphasizing the importance of a dilated fundus examination to reliably stratify patients. Patients with severe papilledema are at higher risk of visual acuity and visual field loss at final follow-up.

Age, race, sex, and BMI were similar in IIH patients with mild versus severe papilledema, emphasizing the importance of a dilated fundus examination to reliably stratify patients. Patients with severe papilledema are at higher risk of visual acuity and visual field loss at final follow-up.The visual system has high metabolic requirements and is therefore particularly vulnerable to mitochondrial dysfunction. The most commonly affected tissues include the extraocular muscles, photoreceptors, retinal pigment epithelium, optic nerve and visual cortex. Hence, the most common manifestations of mitochondrial disorders are progressive external ophthalmoplegia, macular pattern dystrophy, pigmentary retinopathy, optic neuropathy and retrochiasmal visual field loss. With the exception of Leber hereditary optic neuropathy and stroke-like episodes seen in mitochondrial encephalopathy, lactic acidosis and stroke-like episodes, the majority of neuro-ophthalmic manifestations have an insidious onset. As such, some patients may not recognize subtle progressive visual symptoms. When mitochondrial disorders are highly suspected, meticulous examination performed by an ophthalmologist with targeted ancillary testing can help confirm the diagnosis. Similarly, neuro-ophthalmic symptoms and signs may be the first indication of mitochondrial disease and should prompt systemic investigations for potentially life-threatening associations, such as cardiac conduction defects. Finally, the ophthalmologist can offer symptomatic treatments for some of the most disabling manifestations of these disorders.Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.Acute central retinal arterial occlusion has a very poor visual prognosis. Unfortunately, there is a dearth of evidence to support the use of any of the so-called "conservative" treatment options for CRAO, and the use of thrombolytics remains controversial. In this review, we address a variety of these "conservative" pharmacologic treatments (pentoxifylline, isosorbide dinitrate, and acetazolamide) and nonpharmacologic approaches (carbogen, hyperbaric oxygen, ocular massage, anterior chamber paracentesis, laser embolectomy, and hemodilution) that have been proposed as potential treatments of this condition. We conclude that the available evidence for all treatments is insufficient to conclude that any treatment will influence the natural history of this disorder. Management of CRAO patients should instead focus on reducing the risk of subsequent ischemic events, including cerebral stroke. Certain patients may be considered for acute treatment with thrombolytics, although further research must clarify the efficacy, safety, and optimal use of these therapies.Optical coherence tomography (OCT) is a noninvasive imaging technique used to qualitatively and quantitatively analyze various layers of the retina. OCT of the retinal nerve fiber layer (RNFL) and ganglion cell-inner plexiform layer (GCIPL) is particularly useful in neuro-ophthalmology for the evaluation of patients with optic neuropathies and retrochiasmal visual pathway disorders. Fosbretabulin OCT allows for an objective quantification of edema and atrophy of the RNFL and GCIPL, which may be evident before obvious clinical signs and visual dysfunction develop. Enhanced depth imaging OCT allows for visualization of deep structures of the optic nerve and has emerged as the gold standard for the detection of optic disc drusen. In the evaluation of compressive optic neuropathies, OCT RNFL and GCIPL thicknesses have been established as the most important visual prognostic factor. There is increasing evidence that inclusion of OCT as part of the diagnostic criteria for multiple sclerosis (MS) increases its sensitivity. Moreover, OCT of the RNFL and GCIPL may be helpful in the early detection and monitoring the treatment of conditions such as MS and Alzheimer's disease. OCT is an important aspect of the neuro-ophthalmologic assessment and its use is likely to increase moving forward.It is estimated that 7.2% of community-dwelling older adults worldwide have major depression. Therefore, this study aimed to investigate the relationship between geriatric syndromes and depressive symptoms. Data were obtained from the Kaizuka Dementia Prevention Study 2018 and 2019, which was a community-based health check conducted in collaboration with the Osaka Kawasaki Rehabilitation University (Kaizuka City Office) and Cognitive Reserve Research Center in Osaka, Japan. The participants comprised 363 older adults (mean age 73.6 ± 6.6 years; women = 75.8%) who participated in a community-based health check. Depressive symptoms were assessed using the 15-item Geriatric Depression Scale (GDS-15). Depressive symptoms were defined as a GDS-15 score of ≥ 5. Furthermore, geriatric syndromes in participants-such as frailty, sarcopenia, and locomotive syndrome-were assessed. There was a 28.1% prevalence of depressive symptoms. In a logistic regression analysis with depressive symptoms as the dependent variable, both pre-frailty (odds ratio [OR] 1.