Objectives: Idiopathic intracranial hypertension (IIH) is a syndrome that excludes secondary causes such as intracranial space-occupying lesion, hydrocephalus, cerebrovascular disease, and hypoxic ischemic encephalopathy. If not be treated promptly and effectively, IIH can cause severe, permanent vision disability and intractable, disabling headache. This study aims to explore the clinical and image features for IIH, to help clinicians to understand this disease, increase the diagnose rate, and improve the outcomes of patients.
Methods: We retrospectively analyzed 15 cases of IIH that were admitted to Xiangya Hospital, Central South University, during January 2015 to September 2020. The diagnosis of IIH was based on the updated modified Dandy criteria. We analyzed clinical data of patients and did statistical analysis, including age, gender, height, weight, medical history, physical examination, auxiliary examination, treatment and outcome.
Results: There were 10 females and 5 males. Female patients were 22 to 42 years old with median age of 39.5. Male patients were 27 to 52 years old with the median age of 44.0. The BMI was 24.14-34.17 (28.71±2.97) kg/m2. All patients had a BMI above the normal range (≥24 kg/m2), among them 10 cases (66.7%) were obese, and 5 cases (33.3%) were overweight. Eleven cases (73.3%) had headache, and 8 cases (53.3%) had persistent visual loss of different severity. Other symptoms included paroxysmal amaurosis (2 cases), tinnitus (3 cases), horizontal diplopia (2 cases), unilateral peripheral facial paralysis (2 cases), and unilateral blepharoptosis (1 case). Iron-deficiency anemia was found in 3 patients. One of them fully recovered from IIH after the correction of anemia. Other comorbidities included hypertension (8 cases) and polycystic ovarian syndrome (1 case). Fourteen patients assessed blood lipid profile, and all of them had abnormity. Nervous system signs included cervical rigidity (2 cases), limited abduction of eyeball (6 cases), peripheral facial paralysis (2 cases), and blepharoptosis (1 case). Cerebral spinal fluids of all patients had normal cell count, chemical component, Gram's stain, acid-fast stain, and India ink stain. Typical image signs suggesting that IIH could be seen in some patients, including empty sella (5 cases, 33.3%) or partially empty sella (4 cases, 26.7%), distension of perioptic subarachnoid space (3 cases, 20%), flattening of the posterior sclera (5 cases, 33.3%), intraocular protrusion of the optic papilla (7 cases, 46.7%), and enhancement of the optic papilla (2 cases, 13.3%). Ophthalmic exam showed all patients had bilateral papilledema. After diagnosed as IIH, all patients received individualized dehydration treatment to reduce the intracranial hypertension. Three patients received the ventriculo-peritoneal shunt operation. Most patients had good outcome after treatment. For 2 patients, visual impairment was poorly recovered.
Conclusions: IIH primarily affects women of childbearing age who are overweight. The major hazard of IIH is the severe and permanent visual loss. Typical image signs have high specificity in IIH diagnosis. Prompt diagnosis and effective treatment are significantly important to improve the outcomes of patients.
目的: 特发性颅内压增高(idiopathic intracranial hypertension，IIH)是排除颅内占位性病变、脑积水、脑血管病、缺血缺氧等继发性病因后的一种颅内压增高综合征。如不能得到及时、有效的诊治，IIH可能导致严重的永久性视力障碍，以及难以治疗的致残性头痛。本研究旨在探讨IIH的临床与影像学特征，以期提高临床医师对该病的认识，减少临床漏诊，改善患者的预后。方法: 回顾性分析中南大学湘雅医院于2015年1月至2020年9月收治的15例IIH患者。IIH的诊断依据修订版Dandy标准。整理年龄、性别、身高、体重、病史资料、体格检查、辅助检查、治疗方案、病情转归等临床资料，进行相应的统计分析。结果: 共纳入10例女性，5例男性。女性患者22~42(中位年龄39.5)岁，男性患者27~52(中位年龄44.0)岁。患者BMI 24.14~34.17(28.71±2.97) kg/m2。15例患者BMI均超过正常范围(≥24 kg/m2)，其中肥胖10例(66.7%)，超重5例(33.3%)。11例(73.3%)患者有头痛症状，8例(53.3%)出现不同程度的持续性视力下降或丧失。其他症状包括发作性黑矇(2例)、耳鸣(3例)、水平复视(2例)、单侧周围性面瘫(2例)、单侧眼睑下垂(1例)等。3例患者合并缺铁性贫血，其中1例经纠正贫血后IIH症状消失。其他合并症包括高血压(8例)、多囊卵巢综合征(1例)等。14例患者进行了血脂检测，均有1项或多项血脂异常。神经系统体征包括颈强直(2例)、眼球外展受限(6例)、周围性面瘫(2例)、眼睑下垂(1例)等。所有患者脑脊液常规检查、生化检查、革兰氏染色、抗酸染色、墨汁染色等均正常。部分患者具备提示IIH的典型影像学征象，包括5例(33.3%)空蝶鞍，4例(26.7%)部分空蝶鞍，3例(20.0%)视神经周围蛛网膜下隙增宽，5例(33.3%)眼球后部变平，7例(46.7%)视乳头突出，2例(13.3%)增强扫描后视乳头强化等。眼科检查提示所有患者均有双侧视乳头水肿。所有患者确诊后均接受个体化脱水降颅压治疗。3例患者行脑室腹腔分流术(ventriculo-peritoneal shunt，VPS)治疗。大部分患者经治疗后病情转归良好，2例患者视力恢复欠佳。结论: IIH好发于肥胖的育龄女性，主要危害是可能对患者造成不可逆的严重视力障碍，典型影像学征象对于识别IIH有较高的特异性。临床上早期诊断、有效治疗十分重要，有助于改善患者的预后。.
Keywords: idiopathic intracranial hypertension; iron-deficiency anemia; neuroimaging; obesity; ocular fundus examination.