Eye and Brain最新文献

Septo-optic dysplasia (SOD), also known as de Morsier syndrome, is a rare congenital disorder belonging to the group of mid-line brain malformations. Despite the highly variable phenotypic penetration, its classical triad include a) optic nerve hypoplasia (ONH), b) agenesis of septum pellucidum and corpus callosum, and c) hypoplasia of the hypothalamo-pituitary axis. SOD has stringent diagnostic criteria requiring 2 or more features of the classic triad, therefore it represents a separate entity from other conditions such as ONH and achiasmia syndromes which share only some of these aspects, or SOD plus syndrome which is characterized by additional cortical abnormalities. Starting from its etiology and epidemiology, this narrative review focuses on the management of SOD patients, including their diagnosis, treatment and follow-up. To date, SOD is not curable; nonetheless, many of its symptoms can be improved through a tailored approach, consisting of hormonal replacement, corrective ophthalmological surgery and neuropsychological support.

Giant cell arteritis (GCA) is an inflammatory vasculitis typically affecting elderly that can potentially cause vision loss. Studies have demonstrated that early recognition and initiation of treatment can improve visual prognosis in patients with GCA. This review addresses the benefits of early diagnosis and treatment, and discusses the available treatment options to manage the disease.

Purpose: Changes to retina have been reported after a number of neurodegenerative conditions. The purpose of this study was to investigate retinal structures in Olympic boxers exposed to frequent head blows.

Methods: Retinal imaging offers potential as a non-invasive biomarker of neuropathology. Macula and retinal nerve fiber layer (RNFL) thickness was measured using optical coherence tomography (OCT) in UK Olympic boxers attending two mandatory eye screening programs, 18 months apart. Data from the two eye screenings provide longitudinal data of retinal change over time. Sedentary healthy subjects (controls) without past or present history of concussion were also screened at the time of the second boxer screening to provide comparison of cross-sectional data.

Results: Sixteen Olympic boxers aged 20-33 years and 20 sedentary healthy controls, aged 24-45 years, were recruited. Significant macula thickening was observed over time (18 months) in 75% of right and 50% of left eye sectors. For RNFL, left eye quadrants thickened. For right eye RNFL quadrants, thickening and thinning of this layer were observed. Cross-sectional results showed thinner macula sectors and RNFL quadrants in Olympic boxers compared to controls.

Conclusion: Significant change to macula and RNFL densities, occurring over an 18 month interval is an unexpected finding in otherwise heathy elite sportsmen. In addition, macula and RNFL were thinner than healthy sedentary controls. OCT may prove clinically useful as a candidate retinal biomarker of neuropathological change after mild traumatic brain injury and/or repeat head blows.

Optic nerve sheath meningiomas are rare benign neoplasms of the meninges surrounding the optic nerve. They are a significant cause of morbidity. While the mortality rate is practically zero, these tumors can blind or disfigure patients. Given that the clinical course can be variable, and treatment has the capacity to cause morbidity itself, the management of these patients can be difficult. We review the literature to discuss the prevalence of optic nerve sheath meningiomas, the association with neurofibromatosis type 2, natural history, and management options and strategies.

Introduction: The classic presentation of pigmentary dispersion syndrome (PDS) often consists of midperipheral iris transillumination defects, Krukenberg's spindle, and dense homogeneous trabecular pigmentation. Other subtle, sometimes overlooked features include pigment on the lens zonules, pigment on the anterior lens capsule and pigment along the equator of the posterior lens capsule.

Case: This unique presentation of PDS presented with bilateral, dense, oblique, and symmetrical pigment deposition along the posterior lens capsule that changed in shape, density, and extent over the span of 3 years.

Discussion: There have been few reports in the literature that describe a central accumulation of pigment along the posterior lens capsule associated with PDS. There are reported cases of pigment deposition along the central aspect of the posterior lens capsule, some changing over time, although none were bilateral and symmetrical. There are suggestions that perhaps this central pigment deposition is related to a break in the ligament of Weiger, allowing communication between the posterior chamber and posterior lens capsule. This is a case in which curvilinear, symmetrical, and changing pigment deposition on the posterior lens capsule is suggestive of perhaps another key features of PDS.

Purpose: Transcranial direct current stimulation (tDCS) has been studied in humans for its effects on enhancement of learning, amelioration of psychiatric disorders, and modification of other behaviors for over 50 years. Typical treatments involve injecting 2 mA current through scalp electrodes for 20 minutes, sometimes repeated weekly for two to five sessions. Little is known about the direct effects of tDCS at the neural circuit or the cellular level. This study assessed the effects of tDCS-like currents on the central nervous system by recording effects on retinal ganglion cell responsiveness using the rabbit retina eyecup preparation.

Materials and methods: We examined changes in firing to On and Off light stimuli during and after brief applications of a range of currents and polarity and in different classes of ganglion cells.

Results: The responses of Sustained cells were consistently suppressed during the first round of current application, but responses could be enhanced after subsequent rounds of stimulation. The observed first round suppression was independent of current polarity, amplitude, or number of trials. However, the light responses of Transient cells were more likely to be enhanced by negative currents and unaffected or suppressed by first round positive currents. Short-duration currents, that is, minutes, as low as 2.5 µA produced a remarkable persistency of firing changes, for up to 1.5 hours, after cessation of current.

Conclusion: The results are consistent with postulated tDCS alteration of central nervous system function, which outlast the tDCS session and provide evidence for the isolated retina as a useful model to understand tDCS actions at the neuronal level.

Multiple sclerosis (MS) is a progressive neurological disorder characterized by both inflammatory and degenerative components that affect genetically susceptible individuals. Currently, the cause of MS remains unclear, and there is no known cure. Commonly used therapies tend to target inflammatory aspects of MS, but may not halt disease progression, which may be governed by the slow, subclinical accumulation of injury to neuroaxonal structures in the central nervous system (CNS). A recognized challenge in the field of MS relates to the need for better methods of detecting, quantifying, and ameliorating the effects of subclinical disease. Simply stated, better biomarkers are required. To this end, optical coherence tomography (OCT) provides highly reliable, reproducible measures of axonal damage and neuronal loss in MS patients. OCT-detected decrements in retinal nerve fiber layer thickness and ganglion-cell layer-inner plexiform layer thickness, which represent markers of axonal damage and neuronal injury, respectively, have been shown to correlate with worse visual outcomes, increased clinical disability, and magnetic resonance imaging-measured burden of disease in MS patients. Recent reports have also suggested that OCT-measured microcystic macular edema and associated thickening of the retinal inner nuclear layer represent markers of active CNS inflammatory activity. Using the visual system as a putative clinical model in MS, OCT measures of neuroaxonal structure can be correlated with functional outcomes to help us elucidate mechanisms of CNS injury and repair. In this review, we evaluate evidence from the published literature and ongoing clinical trials that support the emerging role of OCT in diagnosing, staging, and determining response to therapy in MS patients.

Neurotrophic keratitis (NK) is a degenerative corneal disease caused by damage of trigeminal corneal innervation, which leads to spontaneous epithelial breakdown and corneal ulceration. The impairment of corneal sensory innervation causes the reduction of both protective reflexes and trophic neuromodulators that are essential for the vitality, metabolism, and wound healing of ocular surface tissues. A wide range of ocular and systemic conditions, including herpetic keratitis, ocular chemical burns, corneal surgery, diabetes, multiple sclerosis, and neurosurgical procedures, can cause NK by damaging trigeminal innervation. Diagnosis of NK requires careful investigation of any ocular and systemic condition associated with the disease, complete ocular surface examination, and quantitative measurement of corneal sensitivity. The clinical stages of NK range from corneal epithelial alterations (stage 1) to persistent epithelial defect (stage 2) and ulcer (stage 3), which may progress to corneal perforation. Management of NK is based on clinical severity, and the aim of the therapy is to halt the progression of corneal damage and promote epithelial healing. Although several medical and surgical treatments have been proposed, no therapies are currently available to restore corneal sensitivity, and thus, NK remains difficult and challenging to treat. The purpose of this review is to summarize available evidence on the pathogenesis, diagnosis, and treatment of NK. Novel medical and surgical therapies including the topical administration of nerve growth factor and corneal neurotization are also described.