Wheless James W, Ramsay R Eugene, Collins Stephen D
University of Tennessee Health Science Center, Le Bonheur Children's Medical Center, Memphis, Tennessee, USA.
Neurotherapeutics. 2007 Jan;4(1):163-72. doi: 10.1016/j.nurt.2006.11.008.
Refractory epilepsies such as infantile spasms (IS) and complex partial seizures (CPS) can have a severe negative impact on the neurological integrity and quality of life of affected patients, in addition to drastically increasing their risk of premature mortality. Early identification of potentially effective pharmacotherapy agents is important. Vigabatrin has been shown to be a generally well tolerated and effective antiepileptic drug (AED) in a wide variety of seizure types affecting both children and adults, particularly those with IS and CPS. A bilateral, concentric constriction of the peripheral visual field characterizes the visual field defect (VFD) associated with vigabatrin, well characterized by numerous studies. This peripheral VFD presents in 30-50% of patients with exposure of several years; however, most of these patients are asymptomatic. In well-controlled studies, the earliest onset in patients with CPS is 11 months and at 5 months in infants, with average onsets being more than 5 years and 1 year, respectively. Patients with a peripheral VFD retain an average 65 degrees of lateral vision (normal, 90 degrees). The fact that many patients never develop the vigabatrin-related peripheral VFD, despite long-term exposure at high doses, may support the hypothesis that the injury is an idiosyncratic adverse drug reaction (as opposed to a strict dose- or duration-dependent toxicity). Effective testing methods are available to aid in the early detection and management of the peripheral VFD. This article discusses issues of importance to clinical decision-making in the use of vigabatrin to assist the physician and patient in assessing the benefits of vigabatrin therapy and understanding the potential risks of the VFD and uncontrolled seizures.
难治性癫痫，如婴儿痉挛症（IS）和复杂部分性发作（CPS），除了会大幅增加患者过早死亡的风险外，还会对受影响患者的神经完整性和生活质量产生严重负面影响。尽早识别潜在有效的药物治疗药物很重要。已证明，氨己烯酸在影响儿童和成人的多种癫痫发作类型中，尤其是对患有婴儿痉挛症和复杂部分性发作的患者，是一种耐受性普遍良好且有效的抗癫痫药物（AED）。与氨己烯酸相关的视野缺损（VFD）表现为周边视野的双侧同心性收缩，众多研究已对其进行了充分描述。在暴露数年的患者中，有30%至50%会出现这种周边视野缺损；然而，这些患者大多没有症状。在严格控制的研究中，复杂部分性发作患者最早发病时间为11个月，婴儿为5个月，平均发病时间分别超过5年和1年。患有周边视野缺损的患者平均保留65度的周边视力（正常为90度）。尽管许多患者长期高剂量暴露，但从未出现与氨己烯酸相关的周边视野缺损，这一事实可能支持这样的假设，即这种损伤是一种特异质性药物不良反应（与严格的剂量或持续时间依赖性毒性相反）。有有效的检测方法可用于辅助早期检测和管理周边视野缺损。本文讨论了在使用氨己烯酸时对临床决策至关重要的问题，以帮助医生和患者评估氨己烯酸治疗的益处，并了解视野缺损和癫痫发作不受控制的潜在风险。
