Elmalem Valerie I, Younge Brian R, Biousse Valérie, Leavitt Jacqueline A, Moster Mark L, Warner Judith, Kupersmith Mark J, Landau Klara, Brodsky Michael C, Frohman Larry P, May Eugene F, Tomsak Robert L, Newman Nancy J
Department of Ophthalmology, Emory University School of Medicine, Atlanta, GA 30322, USA.
Ophthalmology. 2009 Oct;116(10):2011-6. doi: 10.1016/j.ophtha.2009.03.054. Epub 2009 Aug 21.
To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas.
Nonrandomized retrospective case series.
Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers.
Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI.
Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia.
Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia.
Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful.
描述疑似为神经鞘瘤的动眼神经肿物患者的病程及预后。
非随机回顾性病例系列研究。
来自9家三级大学神经眼科中心的37例连续的疑似滑车神经鞘瘤病例。
收集病例并描述其就诊时及随访期间的临床特征。纳入标准为脑部磁共振成像（MRI）显示沿动眼神经走行有提示神经鞘瘤的病变。排除标准为动眼神经麻痹的其他病因，如先天性、外伤性或微血管性；初次脑部MRI正常（或无）；缺乏足够的临床信息；以及后续随访脑部MRI显示病变消失。
患者的人口统计学特征、神经纤维瘤病的存在情况、就诊时的症状、垂直斜视、病变大小（就诊时及随访时）、随访时间以及病变或复视的治疗结果。
7例患者被排除，本系列纳入的30例患者中，男性占主导（77%），平均年龄51岁（范围9 - 102岁）。与既往病例报告不同，几乎所有病例就诊时均有动眼神经麻痹（29/30），且常为孤立性。平均随访时间为3.1年（范围0.2个月至11.1年）。未接受病变治疗的患者，初始病变大小与随访时病变大小无显著差异（4.·4 vs. 5 mm）（P = 0.36）。仅3例患者接受了神经外科手术切除，另有1例患者接受了伽玛刀放疗。大多数患者（24/30）未因垂直复视而进行斜视手术。
孤立性动眼神经麻痹且动眼神经小病变的患者预后良好，应通过系列MRI扫描进行随访，无需神经外科干预，除非出现脑干受压体征。大多数有复视且具有滑车神经鞘瘤典型良性动眼神经病变的患者，可通过棱镜眼镜或根本不治疗来适应，尽管斜视手术也可能成功。
