痛性眼肌麻痹综合征12例临床分析

目的探讨痛性眼肌麻痹综合征诊断、鉴别诊断与治疗。方法对12例痛性眼肌麻痹综合征发病情况、头痛性质及颅神经受累表现,辅助检查及治疗进行分析。结果本组12例患者中,眼肌麻痹治愈11例,好转1例;头痛多在激素应用后24~72 h缓解。结论眼肌麻痹综合征可根据头痛性质及眼肌麻痹症状,结合影像学检查确诊,糖皮质激素治疗有特效。     

痛性眼肌麻痹15例分析

痛性眼肌麻痹综合征又称Tolosa-Hunt综合征,临床较少见.多认为病因与海绵窦非特异性炎症肉芽肿有关,以头痛及颅神经麻痹为主要临床表现,需与伴头痛及眼肌麻痹的其他疾病相鉴别.我们回顾性分析近10 a来所遇15分析如下.     

76例眼肌麻痹的病因及其特点

目的：了解不同病因所致眼肌麻痹患者的临床特点,探讨其诊断及鉴别诊断方法。方法：回顾分析连续收治的76例以眼肌麻痹为主要症状患者的临床表现及辅助检查结果。结果：76例患者中54例（71.1%）诊断明确,其中病因以糖尿病最多见（20例,26.3%）;炎症性疾病次之（14例,18.4%）,包括Tolosa-Hunt综合征（THS）、Fisher综合征（FS）及眼肌麻痹性偏头痛（ophthalmoplegic migraine,OM）。糖尿病性眼肌麻痹患者最突出的临床特征为年龄大、最常累及动眼神经但无瞳孔改变。有22例（28.9%）眼肌麻痹患者未能明确诊断,包括不能排除动脉瘤及炎症性疾病者。在各种病因导致的眼动神经麻痹中,动眼神经最常受累,为49例（64.5%）,展神经次之,为38例（50.0%）。结论：眼肌麻痹的病因复杂,糖尿病性及炎症性疾病是眼肌麻痹患者最常见的病因。     

Tolosa-Hunt综合征临床分析

Tolosa-Hunt综合征是由海绵窦或眶上裂非特异性炎性肉芽肿引起的一种痛性眼肌麻痹。本文总结了22例符合国际头痛协会标准的痛性眼肌麻痹综合征患者的临床特点，诊断与鉴别诊断，并结合病理对病因进行了探讨。认为病因为免疫反应性非特异性炎性肉芽肿，而血管改变继发于炎性浸润。     

Tolosa-Hunt综合征10例临床诊治分析

目的研究Tolosa-Hunt综合征(THS)的病因、诊断及治疗。方法收集2010年至2014年住院诊断10例Tolosa-Hunt综合征患者的临床表现、神经影像学、实验室检查及激素治疗情况并进行回顾性分析。结果 10例患者的颅神经麻痹累及动眼神经、滑车神经、外展神经及三叉神经第1、2、3支,有1例为完全性动眼神经麻痹。10例患者神经影像学分类均为良性的,应用激素后疼痛快速减轻,仅有1例遗留轻度复视。对治疗反应无年龄及性别差异。应用激素后10例患者疼痛均较眼肌麻痹缓解快。结论 THS的诊断为排他性,神经影像学对于鉴别诊断THS非常重要,对于THS病人应全面完善检查除外其他疾病引起的疼痛性眼肌麻痹,包括血常规、红细胞沉降率、C反应蛋白、甲状腺、肝肾功能检查、胸片、头部及眼眶部CT及磁共振检查。     

眼肌麻痹病因及其临床特点分析

目的探讨眼肌麻痹的病因与临床特点。方法收集75例眼肌麻痹患者的临床资料,对其病因及临床特点进行分析。结果眼肌麻痹常见病因为脑血管病变、颅内动脉瘤、糖尿病眼肌麻痹、重症肌无力、鼻咽癌、痛性眼肌麻痹,其中老年患者以脑血管病变及糖尿病眼肌麻痹为主,其他年龄以动脉瘤及重症肌无力常见。结论眼肌麻痹犏因复杂,需根据临床特点仔细检查,以免误诊及漏诊,提高临床诊治水平。     

痛性眼肌麻痹综合征并多发性大动脉炎诊断分析

痛性眼肌麻痹综合征并多发性大动脉炎诊断分析李艳秋王凌燕肖海嗣作者单位：１３００５１长春市中心医院（李艳秋）；白求恩医科大学第三临床学院（王凌燕）；通化市东大区人民医院（肖海嗣）痛性眼肌麻痹综合征又称陶鲁沙－亨特（Ｔｏｌｏｓａ－Ｈｕｎｔ，ＴＳＨ）综合征...     

海绵窦区硬脑膜动静脉瘘致2型糖尿病患者眼肌麻痹一例的诊治及文献复习

[目的]结合文献复习,提出海绵窦区硬脑膜动静脉瘘致2型糖尿病患者眼肌麻痹的临床诊断和治疗策略.[方法]回顾分析本院1例由于海绵窦区硬脑膜动静脉瘘致眼肌麻痹的2型糖尿病患者的临床资料.[结果]脑血管数字减影造影（DSA）显示＂左侧海绵窦区硬脑膜动静脉瘘＂,行＂颅内动静脉瘘栓塞术＂后患者上睑下垂症状及眼球运动逐渐改善.[结论]眼肌麻痹的病因复杂.对于糖尿病患者突发的颅神经受损,除了考虑糖尿病神经系统并发症外,还需排除其他颅内占位性病变及海绵窦区硬脑膜动静脉瘘可能,必要时可行影像学检查以明确病因.     

眼肌麻痹致眩晕临床分析

目的探讨眼肌麻痹致眩晕患者的病因和临床特点。方法对2010年1月-2013年12月收治的45例眼肌麻痹致眼源性眩晕患者的临床资料进行回顾性分析,并就其致病病因、特点、治疗方法及效果进行总结。结果其病因以眼肌型重症肌无力最多,共20例（44.4%）,其次为Graves眼病,占9例（20.0%）,其他依次为糖尿病性眼肌麻痹5例（11.1%）、颅内炎性疾病4例（8.9%）、内直肌损伤3例（6.7%）、眼眶肿瘤2例（4.4%）和Lambert综合征2例（4.4%）。36例（80.0%）为神经肌肉接头病变或肌肉病变所致,由全身疾病导致的眼肌麻痹（88.9%）比例明显高于眼科局部疾病所致的眼肌麻痹（11.1%）。针对原发疾病进行治疗可获得较好疗效。结论眼源性眩晕病因复杂,非视力所致眼源性眩晕应高度怀疑全身疾病导致的眼肌麻痹。临床应仔细进行相关疾病筛查,并针对原发疾病进行积极治疗。     

眶内电针治疗Fisher综合征所致眼肌麻痹临床观察

目的:回顾分析眶内电针(IEA)治疗Fisher综合征(MFS)眼肌麻痹疗效及MFS眼肌麻痹患者的临床特点。方法:收集接受IEA治疗的MFS患者27例的资料,对一般资料、受累颅神经、感染情况、治疗前后最大复视角度、治疗及恢复情况等进行回顾分析。结果:患者从发病到开始接受IEA治疗的平均时间为(31.04±46.23)d,接受治疗后达到临床痊愈的平均天数为(33.67±21.96)d。临床痊愈(无复视致残)24例(88.9%)。治疗后,左眼和右眼的最大复视角度均较治疗前显著降低(P<0.01)。MFS眼肌麻痹特点:100%患者有外展神经病变,其中11.1%为单侧病变,88.9%为双侧受累;37.0%合并III、VI对颅神经麻痹,无孤立的III或IV对颅神经麻痹患者。70.3%有感染史;11.1%伴发高血压、7.4%伴发高脂血症、3.7%伴发2型糖尿病。22.2%伴有Bell征,双侧受累4例,单侧受累2例。25.9%眼睑下垂,双侧受累5例,单侧受累2例。3.7%伴有双侧瞳孔散大。结论:IEA治疗可加快MFS眼肌麻痹症状恢复,MFS眼肌麻痹的临床特点可提示临床诊断。     

Tolosa-Hunt syndrome due to actinomycosis of the cavernous sinus: the infectious hypothesis revisited

BACKGROUND: The Tolosa-Hunt syndrome is characterized by ophthalmoplegia with unilateral severe retro-orbital pain associated to a granulomatous inflammatory process occupying the cavernous sinus or the superior orbital fissure. The etiology is unknown and diagnosis is based upon a clinical response to steroid treatment and exclusion of neoplasm, trauma, aneurysms, infectious, and inflammatory diseases. CASE DESCRIPTION: A 43-year-old man was admitted because of a 1-week history of acute onset left-sided retro-orbital pain, followed by left sixth cranial nerve palsy. Magnetic resonance imaging was normal and Tolosa-Hunt syndrome was suspected. Steroid treatment controlled pain with recovery of ophthalmoplegia. Four months later, when a good response to treatment was still present, brain magnetic resonance imaging revealed a lesion enlarging the left cavernous sinus, isointense with the gray matter on T1-weighted sequences, hypointense on T2-weighted images, and with homogeneous enhancement after gadolinium injection. Two months later, ocular pain and sixth cranial nerve palsy recurred and new brain magnetic resonance imaging showed an extension of the tissue occupying the left cavernous sinus, over the sella, to the right cavernous sinus, making possible an endoscopic transphenoidal biopsy. RESULTS: Histopathological study revealed a granulomatous aspecific inflammation containing actinomycetes colonies. The patient was treated with intravenous penicillin G followed by amoxicillin per os, with improvement of pain and ophthalmoplegia. A control magnetic resonance imaging 1 month after therapy showed a consistent reduction of the enlarged cavernous sinus, and 3 months later neurological examination and brain magnetic resonance imaging were completely normal. CONCLUSIONS: The present case suggests that the International Classification of Headache Disorders (2nd edition) definition of Tolosa-Hunt syndrome does not reflect the complexity of the syndrome and that some cases of secondary painful ophthalmoplegias can fit the criteria for the primary form. Since the biopsy can only rarely be performed, we agree with other authors that clinical and radiological follow-up should be performed for at least 2 years. Moreover, we propose that in patients with painful ophthalmoplegia having transient response to steroid therapy, a trial with antibiotic therapy should be taken into account.     

Autres névralgies : névralgie occipitale,syndrome de la bouche brûlante,neuropathie ophtalmoplégique récurrente douloureuse,syndrome de Tolosa-Hunt

Occipital neuralgia is defined as an electricshock- type or stabbing pain in the area of the occipital nerves. There are several types of treatment available: medical (anticonvulsant or tricyclic agents), transitional (infiltrations) or surgical (neurolysis or stimulation). Burning mouth syndrome is a chronic and spontaneous burning-type pain to the mouth, which may affect the tongue and/or other oral mucosae, with no identifiable injuries. Recurrent ophthalmoplegic cranial neuropathy is a rare neurological syndrome characterised by the onset of recurrent headaches and ophthalmoplegia, affecting the third and/or sixth cranial nerves. Tolosa-Hunt syndrome occurs as a periorbital or hemicranial pain associated with ipsilateral paresis of the oculomotor nerves, oculosympathetic paralysis and a loss of sensitivity in the distribution of the trigeminal nerve branches.     

A new etiology for visual impairment and chronic headache: The Tolosa-Hunt syndrome may be only one manifestation of venous vasculitis

The Tolosa-Hunt syndrome is characterized by recurrent periods of painful ophthalmoplegia, dramatically ameliorated by steroid treatment. In half of the cases orbital phlebography shows characteristic changes. Ninety-six patients with orbital pain characteristic of the Tolosa-Hunt syndrome were submitted to orbital phlebography regardless of the existence of other symptoms. A pathologic phlebogram with changes typical of the Tolosa-Hunt syndrome was found in 50 patients. In 17 of these 50 patients the symptoms consisted of orbital pain only and no ophthalmoplegia. Twenty patients had pain and ipsilateral decrease of vision but no ophthalmoplegia, and 13 had painful ophthalmoplegia. Of 41 patients treated with steroid medication 39 responded dramatically with regard to the pain. Earlier findings indicate that the phlebographic changes are due to venous vasculitis, which thus may cause irreversible visual impairment but also chronic headache without ophthalmoplegia. It is concluded that the Tolosa-Hunt syndrome may be only one manifestation of a not uncommon disease.     

Painful Ophthalmoplegia: Oculomotor Nerve Palsy Without Mydriasis Due to Compression by Aneurysm

SYNOPSIS
A patient with a previously diagnosed left Horner's syndrome suddenly developed severe pain in the left eye and diplopia. Ipsilateral third cranial nerve palsy was present, but the pupil remained small and minimally reactive. A carotid angiogram showed a large aneurysm of the left internal carotid-posterior communicating junction. Pain was only relieved after successful clipping of the aneurysm and liberation of the third nerve. The effects of combined sympathetic and parasympathetic denervation of the pupil and the mechanism of their development in regard to the differential diagnosis of painful ophthalmoplegia are discussed.     

Tolosa-Hunt syndrome preceded by facial palsy

The Tolosa-Hunt syndrome consists of a painful ophthalmoplegia related to a granulomatous inflammatory process in the cavernous sinus, which may be documented by cerebral magnetic resonance imaging with gadolinium enhancement. Two cases of Tolosa-Hunt syndrome preceded by facial palsy observed in 1998 at the Department of Neurosurgery of the Second University of Naples are presented here. Both patients developed Tolosa-Hunt syndrome following an ipsilateral facial palsy that resolved in about 15 days with medical treatment. Cerebral magnetic resonance imaging with gadolinium enhancement showed, in both cases, inflammatory tissue in the cavernous sinus. The patients underwent corticosteroid therapy (prednisolone, 80 mg per day, intravenously) with pain regression. In the first case, the patient experienced recurrence of the syndrome that was definitively resolved with further corticosteroid treatment. The rare reports of facial palsy in patients with Tolosa-Hunt syndrome suggest the inclusion of this disease in the so-called multiple cranial nerve palsy syndrome. It is probable that Tolosa-Hunt syndrome has an inflammatory pathogenesis.     

表现为慢性痛性眼肌麻痹的抗GQ1b抗体综合征的诊断学特征并文献复习

目的探讨表现为慢性痛性眼肌麻痹的抗GQ1b抗体综合征的诊断学特征。 方法回顾性分析2022年2月7日北京大学第一医院神经内科收治的1例表现为慢性痛性眼肌麻痹的抗GQ1b抗体综合征患者的临床资料,总结其诊断学特征,并文献复习。 结果患者女性,66岁,2年前以眼痛、复视为首发症状,早期症状呈波动性,半年前眼痛及眼睑下垂症状急剧加重,伴眩晕。右眼上睑下垂,完全遮盖瞳孔,右眼外斜位,内收、上视、下视受限,右眼内收时角膜内缘距内眦2 mm,向左侧注视时复视最明显,双眼左右视时有眼痛。闭目难立征阳性,双侧膝反射及跟腱反射未引出。血GD3 IgG抗体、GT1a IgG抗体、GQ1b IgG抗体均强阳性。眼眶和头颅海绵窦核磁共振成像(MRI)未见明显异常;神经传导速度正常;脑脊液常规、生化正常;眼震电图提示扫视可见欠射,Ⅱ-Ⅲ型跟踪曲线。予小剂量口服糖皮质激素(30 mg/d)后,患者痛性眼肌麻痹及头晕症状明显好转。 结论抗GQ1b抗体综合征可以表现为复发性的慢性痛性眼肌麻痹,前庭神经功能障碍,结合临床表现及血清抗体谱检测有助于疾病的正确诊断,小剂量激素治疗有效。     

Painful Ophthalmoplegia of the Right Eye in a 20-Year-Old Man

Background

Painful ophthalmoplegia is an infrequent but very important presentation in the acute care setting and requires a thorough neurologic evaluation to determine its cause. This clinical sign has a large differential and generally indicates a serious cerebrovascular process.

Case Report

We describe a patient with a subacute presentation of painful ophthalmoplegia, ultimately diagnosed as Tolosa-Hunt syndrome, a rare inflammatory process of the cavernous sinus.

Conclusion

This case highlights the importance for the Emergency Physician to quickly recognize painful ophthalmoplegia and rapidly elucidate the cause to prevent further complications for the patient.