【摘要】 报道1例罕见的临床上表现为眼眶炎性假瘤形式的视神经束膜炎，并且强调区分视神经束膜炎和脱髓鞘的球后视神经炎。方法：病例报道。结果：一个54岁的健康的马来西亚女性，主诉右侧持续性头痛3d伴有复视。疼痛与眼球活动相关联。脑部和眼眶MRI显示右侧视神经鞘和眶内脂肪条纹异常增强，未有异常的增强提示脱髓鞘疾病。这名患者诊断为右眼视神经束膜炎。从开始使用全身性的类固醇激素，维持并逐渐减量超过6mo。她的症状得到缓解并且直到最近的随访都没有复发。结论：所有的眼眶炎性假瘤病例都应该考虑存在视神经束膜炎的可能，在开始治疗前必须与球后视神经炎相鉴别。因为这两种疾病有不同的治疗原则和预后。
【关键词】 视神经束膜炎 眼眶炎性假瘤 球后视神经炎
INTRODUCTION
Idiopathic orbital inflammation or orbital pseudotumour is usually confined to orbital structures [1]. It is often sub classified according to anatomy of target structure within the orbit[1]. The inflammation may present as primary dacryoadenitis, myositis, sclerotenositis, and perioptic neuritis or diffuse anterior soft tissue inflammation [1]. It may restrict to superior orbital fissure or cavernous sinus [1]. The clinical presentation will depend on the involved target orbital structures [1]. The inflammations of the lacrimal gland and muscle cone are commonly involved in the case of orbital pseudotumor [2,3].
Optic perineuritis (OPN) is an uncommon variant of orbital inflammatory disease, targeting the optic nerve sheath [4]. It should be distinguished from demyelinating optic neuritis because it carries a different treatment and prognosis [4]. This case report described a patient who was diagnosed to have optic perineuritis and responded well to systemic steroid. The signs and symptoms dramatically improved.
CASE REPORT
A 54yearold Malay lady presented to our clinic in June 2007 with complaints of right throbbing headache for one week duration and double vision for 3 days. It was associated with painful blurring of vision in the right eye. There was also pain on eye movement. Patient was otherwise healthy and premorbid vision of both eyes was good. There was no history of similar episode before and no history of sinusitis or fever. There were no nervous system complaints and no history of neurological diseases running in her family.
On examination, visual acuity of the right eye was 6/90, corrected to 6/18 with pinhole. There was mild ptosis of the right eye. However, there was no proptosis. The relative afferent pupillary defect was positive in the right eye. There was limitation of abduction of the right eye (Figure 1). Anterior segment examination and intraocular pressure of both eyes were normal. Funduscopy showed swollen and hyperemic disc in the right eye. The left eye was normal with visual acuity of 6/6.
The red desaturation and brightness examination were markedly reduced in the right eye. Other cranial nerves (CN 1, 5, 712) were normal. Neurological examination was normal. Visual field of the right eye showed defect of inferior visual field extended to superior field (Figure 2A).
Patient was investigated thoroughly to rule out the possibility of any infection, infiltration, malignancy and collagen diseases. Full blood picture, erythrocyte sedimentation rate, renal function test and serology investigation for syphilis were normal. Collagen screening for ANA, dsDNA and rheumatoid factor were negative. Chest radiography was also normal.
Her condition progressed in the next few days with marked limitation of ocular mobility while waiting for the magnetic resonance imaging (MRI) scanning. She had minimal eye movement in upgaze and downgaze. Her visual acuity had reduced to finger counting. MRI of brain and orbit demonstrated abnormal enhancement of right optic nerve sheath with streakiness of intraorbital fat. There was no evidence of eye proptosis or enlargement of extraocular muscles or involvement of lacrimal gland (Figure 3). MRI of the brain and orbit revealed no space occupying lesion or any evidence of abnormal enhancement to suggest demyelinating process. The diagnosis of right optic perineuritis was made.
enhancement of right optic nerve sheath, streakiness of orbital fat, enlargement of right optic nerve [“tramtrack” on axial view (A) and “doughnut” on coronal view (B)] and streakiness of orbital fat Patient was started on intravenous methylprednisolone 250mg four times per day for 3 days. It was followed by oral prednisolone 1mg/kg in divided doses. The headache and eye pain improved within 3 days. Oral predinisolone 1mg/kg was continued after eleven days. Visual acuity was improved to 6/12 after 14 days of treatment. The symptoms of diplopia were gradually improved. Oral prednisolone was tapered slowly according to the symptoms. The ocular motility improved. After two months on tapering dose of oral prednisolone, her visual acuity improved to 6/7.5 and ocular motility had become almost normal. Visual field of the right eye improved with only small superior field scotoma persisted (Figure 2B).
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