Merabet Lotfi B, Mayer D Luisa, Bauer Corinna M, Wright Darick, Kran Barry S
From the *Department of Ophthalmology, Laboratory for Visual Neuroplasticity, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA.
†Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, MA; ‡New England College of Optometry, Boston, MA; §NECO Clinical Network, Perkins School for the Blind, Watertown, MA.
Semin Pediatr Neurol. 2017 May;24(2):83-91. doi: 10.1016/j.spen.2017.04.005. Epub 2017 Apr 10.
Cortical (cerebral) visual impairment (CVI) results from perinatal injury to visual processing structures and pathways of the brain and is the most common cause of severe visual impairment or blindness in children in developed countries. Children with CVI display a wide range of visual deficits including decreased visual acuity, impaired visual field function, as well as impairments in higher-order visual processing and attention. Together, these visual impairments can dramatically influence a child's development and well-being. Given the complex neurologic underpinnings of this condition, CVI is often undiagnosed by eye care practitioners. Furthermore, the neurophysiological basis of CVI in relation to observed visual processing deficits remains poorly understood. Here, we present some of the challenges associated with the clinical assessment and management of individuals with CVI. We discuss how advances in brain imaging are likely to help uncover the underlying neurophysiology of this condition. In particular, we demonstrate how structural and functional neuroimaging approaches can help gain insight into abnormalities of white matter connectivity and cortical activation patterns, respectively. Establishing a connection between how changes within the brain relate to visual impairments in CVI will be important for developing effective rehabilitative and education strategies for individuals living with this condition.
皮质(大脑)视觉障碍(CVI)是由围产期大脑视觉处理结构和通路受损引起的,是发达国家儿童严重视力障碍或失明的最常见原因。患有CVI的儿童表现出广泛的视觉缺陷,包括视力下降、视野功能受损,以及高阶视觉处理和注意力受损。这些视觉障碍共同作用,会极大地影响儿童的发育和幸福。鉴于这种疾病复杂的神经学基础,眼科保健从业者常常无法诊断出CVI。此外,与观察到的视觉处理缺陷相关的CVI神经生理学基础仍知之甚少。在此,我们介绍一些与CVI患者的临床评估和管理相关的挑战。我们讨论脑成像技术的进步如何可能有助于揭示这种疾病的潜在神经生理学。特别是,我们展示了结构和功能神经成像方法如何分别有助于深入了解白质连接和皮质激活模式的异常。建立大脑内部变化与CVI视觉障碍之间的联系,对于为患有这种疾病的个体制定有效的康复和教育策略至关重要。