作为一种新型无创且操作简单的主观检查手段,临界闪烁融合频率(critical flicker fusionfrequency,CFF)可动态反映人眼视功能变化情况。作为早期识别脱髓鞘病变和评估视功能恢复情况的敏感指标,上个世纪已被国外学者用于视网膜和视神经疾病研究中,包括氯喹中毒性视网膜病变、糖尿病视网膜病变、中心性浆液性视网膜病变、年龄相关的黄斑病变、乙胺丁醇中毒性视神经病变、视神经炎和非动脉炎性前部缺血性视神经病变。在视网膜和视神经疾病中,CFF均有不同程度下降,依据CFF改善程度以及主要损害的色光可能有助于视网膜和视神经疾病的鉴别,且CFF与其他视功能,视力、视野、视觉诱发电位的潜时具有较好的相关性。目前国内相关研究尚处于起步阶段,本文就CFF在视网膜和视神经疾病的应用情况做一总结。
As a new non-invasive and simple subjective examination method, critical flicker fusion frequency (CFF) can dynamically reflect the changes of visual function of human eyes. As a sensitive indicator for early identification of demyelinating diseases and assessment of visual function recovery, it has been used by foreign scholars in the last century in the field of retinal and optic nerve diseases, including chloroquine toxic retinopathy, diabetic retinopathy, central serous retinopathy, age-related macular degeneration, ethambutol-induced optic neuropathy, optic neuritis and non-arteritic anterior ischemic optic neuropathy. Though there was a different decrease of CFF in retina and optic nerve diseases, it may be helpful for the differentiation of retinal and optic nerve diseases according to the degree of CFF improvement and the main damaged color light. Moreover, CFF has a good correlation with other visual functions, visual acuity, visual field, and peak time of visual evoked potential. At present, and relevant domestic studies is still in its infancy. This article summarizes the application of CFF in retinal and optic nerve diseases.
目的:探讨小剂量利妥昔单抗(rituximab, RTX)预防视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder, NMOSD)复发的有效性和安全性。方法:采用前瞻性自身对照试验,选取2020年7月至2021年4月临床确诊为NMOSD的38例患者进行研究,给予小剂量RTX治疗。所有患者均进行病史采集、眼科检查和血清学指标检测,记录NMOSD年复发率(ARR)、最佳矫正视力(BCVA)、合并自身抗体情况和追加治疗的情况。视力检查采用Snellen视力表进行,并将结果转换为最小分辨角对数(logMAR)视力记录。随访至少12个月(17.29±2.2)个月,以末次随访为疗效判定时间点,比较治疗前后ARR、BCVA;分析复发与发病年龄、是否合并自身免疫抗体阳性和患自身免疫性疾病的关系。记录不良反应的发生率和追加治疗的时间。结果:共38例患者61眼纳入研究。其中男性4例,女性34例。发病年龄12~60岁,中位发病年龄23 (18~29.3)岁。病程10.0~265个月,中位病程65 (48.3~101.0)个月。治疗前logMAR矫正视力(1.15±0.13),治疗后logMAR矫正视力(1.54±0.39),比较差异无统计学意义(t=1.120,P=0.267)。治疗前ARR(1.50±0.86)次/年,治疗后ARR降低为(0.12±0.07)次/年,比较差异有统计学意义(t=8.304,P<0.001)。追加治疗时间为(6.4±2.3)月。随访期间3例患者复发,复发次数为 5次。 复发者与未复发者的发病年龄、合并免疫抗体阳性比例、合并自身免疫性疾病比例比较,差异均无统计学意义(均P>0.05)。38例患者中,出现输注不良反应7例,给予减慢RTX滴速及加用地塞米松5 mg治疗后均缓解,随访期间未见其他明显不良反应。结论:小剂量RTX可以有效清除B淋巴细胞,预防NMOSD复发,且安全性较好。
Objective: To evaluate the efficacy and safety of long-term treatment with low-dose rituximab for neuromyelitis optica spectrum disorders (NMOSD). Methods: A prospective self-control study. A total of 38 patients who were diagnosed with NMOSD from July 2020 to April 2021 were recruited for rituximab treatment. All patients collected medical history, ophthalmic examination and serological test. Recorded the annual recurrence rate (ARR), best corrected visual acuity (BCVA), combined autoantibodies and therapy times after the first treatment. The BCVA was examined using Snellen chart, and converted to logMAR. The patients were followed up at least 12(17.29±2.2) months, and the last follow-up was taken as the time point of efficacy evaluation. ARR and BCVA before and after treatment were compared. To analyze the relationship between relapse and age of onset, combination of autoimmune antibodies and autoimmune diseases. The incidence of side effects and duration of additional therapy were recorded. Results: A total of 38 NMOSD patients (4 male/34 female, 61 involved eyes) were included in this study. The ages of onset age were 12-60 years, the median onset age was 23 (18~29.3) years. Duration of disease was 10.0~265 months, the median duration was 65 (48.3~101.0) months. Before treatment, the mean BCVA was 1.15 ± 0.13 , the mean BCVA at last follow-up was 1.54 ± 0.39, which was no significant difference (t=1.120, P=0.267). The mean ARR before and after treatment were 1.50±0.86 and 0.12 ± 0.07, respectively, with significant difference (t=8.304, P<0.001). The mean reinfusion period was 6. 4±2.3 months. Five relapses in 3 patients were observed. There were no significant difference between relapsed patients and non-relapsed patients on onset age, with/without auto-immune antibody ratio, with/without auto-immune diseases ratio (all P>0.05). Of 38 patients, 7 patients had side effects, all patients who had side effects, slowing down the infusion speed of RTX or infusing 5 mg of dexamethasone could relieve the discomfort. Conclusions: Low-dose RTX can effectively clear B lymphocytes, prevent NMOSD recurrence and with good safety.
视神经炎(optic neuritis,ON)是指视神经的炎性脱髓鞘病变,是引起中青年人视力下降的主要原因。近年来,髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎(myelin oligodendrocyte glycoprotein antibody-positive ON,MOG-ON)成为神经眼科领域的研究热点,国内外报道不断增加。2021年3月,中华医学会眼科学分会神经眼科学组制定了《中国脱髓鞘性视神经炎诊断和治疗循证指南(2021年)》,将MOG-ON作为新的视神经炎亚型纳入脱髓鞘性视神经炎的诊疗体系,给广大眼科医生提供了新的参考依据。因此,临床医生需要充分认识MOG抗体相关疾病和MOG-ON的临床特征和治疗进展,努力提高其诊断和治疗水平,使此类患者能够得到更多的获益,造福于更多的视神经疾病患者。
Optic neuritis(ON)is an inflammatory demyelinating disease of the optic nerve, which is the main cause of vision loss in young and middle-aged people. In recent years, myelin oligodendrocyte glycoprotein antibody-positive ON(MOG-ON)has become a research hotspot in the field of neuro-ophthalmology, and reports at home and abroad are increasing.In March 2021, the Neuro-ophthalmology Group of Ophthalmology Branch of Chinese Medical Association formulated the Evidence-Based Guidelines for the Diagnosis and Treatment of Demyelinating Optic Neuritis in China(2021) , and included MOG-ON as a new optic neuritis subtype in the diagnosis and treatment system of myelinating optic neuritis providing a new reference for the majority of ophthalmologists. Therefore, clinicians need to fully understand the clinical features and treatment progress of MOG antibody-related diseases and MOG-ON, and strive to improve the level of diagnosis and treatment, so that such patients can get more benefits and benefit more patients with optic nerve diseases.
非器质性视力下降也称为心因性或功能性视力下降,除视力下降外,还可伴有视野缺损,多由于精神心理疾患导致的转换障碍引起,部分患者为诈病以获取利益。本文报道1例6岁的女性患者,主诉双眼反复视力下降1年余,早期被误诊为儿童视神经炎,给予糖皮质激素冲击治疗,治疗后稍有好转。通过本例患者误诊的教训,提醒我们在遇到儿童出现不明原因的视力下降时,在没有明确器质性疾病证据时要想到非器质性视力下降的可能,掌握识别非器质性视力下降的检查方法,不能忽略相对性传入性瞳孔障碍等基础的神经眼科检查。
Non-organic vision loss is also known as psychogenic or functional vision loss. In addition to vision loss, it can also be accompanied by visual field defect. It is mostly caused by conversion obstacles caused by mental and psychological diseases. Some patients cheat to obtain benefits. This paper reports a 6-year-old female patient who complained of repeated visual acuity decline for more than one year. She was misdiagnosed as pediatric optic neuritis in the early stage and was treated with glucocorticoid shock therapy, which her condition improved slightly after treatment. The misdiagnosis of this patient teaches us that when children have unexplained visual acuity decline, we should think of the possibility of non-organic visual acuity decline when there is no clear evidence of organic diseases, master the examination methods to identify non-organic visual acuity decline, and cannot ignore the basic neuro-ophthalmic examination such as relative afferent pupillary defect (RAPD).
非器质性视力下降也称为心因性或功能性视力下降,除视力下降外,还可伴有视野缺损,多由于精神心理疾患导致的转换障碍引起,部分患者为诈病以获取利益。本文报道1例6岁的女性患者,主诉双眼反复视力下降1年余,早期被误诊为儿童视神经炎,给予糖皮质激素冲击治疗,治疗后稍有好转。通过本例患者误诊的教训,提醒我们在遇到儿童出现不明原因的视力下降时,在没有明确器质性疾病证据时要想到非器质性视力下降的可能,掌握识别非器质性视力下降的检查方法,不能忽略相对性传入性瞳孔障碍(relative afferent pupillary defect,RAPD)等基础的神经眼科检查。
Non-organic vision loss is also known as psychogenic or functional vision loss. In addition to vision loss, it can also be accompanied by visual field defect. It is mostly caused by conversion obstacles caused by mental and psychological diseases. Some patients cheat to obtain benefits. This paper reports a 6-year-old female patient who complained of repeated visual acuity decline for more than one year. She was misdiagnosed as pediatric optic neuritis in the early stage and was treated with glucocorticoid shock therapy, which her condition improved slightly after treatment. The misdiagnosis of this patient teaches us that when children have unexplained visual acuity decline, we should think of the possibility of non-organic visual acuity decline when there is no clear evidence of organic diseases, master the examination methods to identify non-organic visual acuity decline, and cannot ignore the basic neuro-ophthalmic examination such as relative afferent pupillary defect (RAPD).