Wilhelm H, Dörr S, Paulsen F, Becker G, Classen J
Augenheilkunde, Universitätskliniken Tübingen, Schleichstrasse 12, Tübingen.
Klin Monbl Augenheilkd. 2009 Nov;226(11):869-74. doi: 10.1055/s-0028-1109872. Epub 2009 Nov 13.
While evaluating radiotherapy results in patients with primary and secondary optic nerve sheath meningiomas (ONSM) treated between 1993 and 2002, a large amount of data about early signs and symptoms has been collected which might be helpful for establishing an early diagnosis.
We have reviewed the charts of the patients available at the Centre of Ophthalmology, collecting especially pretreatment data.
112 patients, 4 with bilateral tumours, 83 % female were included. Mean age was 51.7 years. Visual acuity loss with relative afferent pupillary defect was the main symptom and sign in primary ONSM (38 patients). Median interval between first symptoms and diagnosis was 12 months. Optic discs were in approximately one half atrophic, the other half were swollen, and only rarely normal (3 cases only). Retinociliary shunt vessels were seen in 10 cases. Nerve fibre bundle defects were the major visual field finding (including constriction and central scotoma). Visual acuity was better than 0.5 (20 / 40) in 46 % and worse than 0.1 (20 / 200) in 30 %. In secondary ONSM, the interval to diagnosis was with a median of 6 months shorter than in primary ONSM. Approximately half of the optic discs were atrophic, only 6 % were swollen. Even here nerve fibre bundle defects were dominating, only 7 % had vertical hemianopic defect. Visual acuity was better than 0.5 (20 / 40) in 30 % and in worse than 0.1 (20 / 200) 22 %. 45 % had ocular motility disorders.
Vertical hemianopic defects were surprisingly rare. The high rate of nerve fibre bundle defects and the relatively high number of patients with good visual acuity might explain why this disorder is occasionally mistaken for glaucoma. A typical clinical appearance can be outlined: mainly mid-aged women, slowly progressing visual loss, frequently motility disorders, relative afferent pupillary defect, nerve fibre bundle defects and atrophic or--mainly in primary ONSM--swollen optic disc form the characteristic picture.
在评估1993年至2002年间接受治疗的原发性和继发性视神经鞘膜瘤（ONSM）患者的放疗结果时，收集了大量关于早期体征和症状的数据，这可能有助于早期诊断。
我们回顾了眼科中心现有患者的病历，特别收集了治疗前的数据。
纳入112例患者，其中4例为双侧肿瘤，女性占83%。平均年龄为51.7岁。视力丧失伴相对传入性瞳孔障碍是原发性ONSM的主要症状和体征（38例患者）。首发症状与诊断之间的中位间隔时间为12个月。约一半的视盘萎缩，另一半肿胀，仅有3例正常。10例可见视网膜睫状分流血管。神经纤维束缺损是主要的视野表现（包括视野缩小和中心暗点）。46%的患者视力优于0.5（20/40），30%的患者视力差于0.1（20/200）。在继发性ONSM中，诊断间隔时间的中位数比原发性ONSM短6个月。约一半的视盘萎缩，仅有6%肿胀。即便如此，神经纤维束缺损仍占主导，仅有7%有垂直偏盲缺损。30%的患者视力优于0.5（20/40），22%的患者视力差于0.1（20/200）。45%的患者有眼球运动障碍。
垂直偏盲缺损出奇地少见。神经纤维束缺损的高发生率以及视力良好患者的相对高比例，或许可以解释为何这种疾病偶尔会被误诊为青光眼。可以勾勒出一种典型的临床表现：主要为中年女性，视力缓慢下降，常伴有眼球运动障碍、相对传入性瞳孔障碍、神经纤维束缺损以及萎缩性或——主要在原发性ONSM中——肿胀的视盘构成特征性表现。
