眼外肌麻痹患者257例的临床特征及病因分析

目的：探讨眼外肌麻痹的临床特征和病因。方法收集并回顾性分析257例眼外肌麻痹患者的临床资料,记录病史、临床表现以及相应的神经内科、眼科、内分泌科和影像学检查结果。结果单纯上眼睑下垂80例,单纯双眼复视131例,上眼睑下垂和双眼复视39例;单侧眼外肌麻痹223例,双侧眼外肌麻痹32例;外直肌麻痹84例,动眼神经支配的眼外肌麻痹178例,上斜肌麻痹24例;82例伴有眼眶局部疼痛,12例伴有瞳孔扩大;眼球运动神经病变163例,神经-肌肉接头病变69例,眼外肌病变25例。在163例眼球运动神经病变患者中,单个眼球运动神经麻痹148例,占90.8%;多个眼球运动神经麻痹15例,占9.2%。眼球运动神经周围性损害110例,占67.5%,核性损害16例,占9.8%,核间性损害13例,占8.0%,核上性损害24例占14.7%。确诊的病因有14种,包括重症肌无力,糖尿病性眼球运动神经损伤,动脉硬化缺血性眼球运动神经损伤,局部非特异性炎症,外伤性,局部占位性病变,甲状腺病变,颅内压增高,颅内后交通动脉瘤,线粒体脑肌病,颈内动脉海绵窦瘘,远隔部位肿瘤,多发性硬化,局部注射肉毒素,进行性肌营养不良。结论眼外肌麻痹的常见症状是双眼复视和上眼睑下垂,单侧眼外肌麻痹发生率高于双侧眼外肌麻痹,动眼神经支配的眼外肌麻痹发生率最高。眼眶部疼痛和瞳孔改变是常见的伴随症状。眼球运动神经性眼外肌麻痹患者并发高血压病、糖尿病的比例较高。眼外肌麻痹患者需要常规检查血糖,甲功、眼眶CT、头颅CT和（或）MRI、新斯的明试验,伴有瞳孔扩大时需要DSA排除颅内动脉瘤。     

Delayed complete bilateral ptosis associated with massive infarction of the right hemisphere

OBJECTIVE: To characterize the phenomenon of complete bilateral ptosis associated with massive infarction of the right cerebral hemisphere, particularly with respect to its time course and relationship to herniation syndromes. PATIENTS AND METHODS: In May through July 2002, consecutive patients admitted to the neurology/neurosurgery intensive care unit at the Mayo Clinic in Rochester, Minn, with massive infarction of the right cerebral hemisphere, and later, complete bilateral ptosis, underwent serial neurologic examinations and neuroimaging. RESULTS: Six patients with massive infarction of the right cerebral hemisphere developed complete bilateral ptosis. All had normal eyelid opening at initial examination and later developed ptosis, which clearly preceded signs of herniation. Three patients died after herniation syndromes developed. In the 3 survivors, improvement in ptosis closely followed resolution of midbrain distortion seen on computed tomographic scans. CONCLUSION: The precise anatomical explanation for complete bilateral ptosis is unclear, but upper brainstem involvement seems most likely. Complete bilateral ptosis is a valuable clinical sign in patients with a massive hemispheric infarction that precedes herniation and could provide an opportunity for early intervention.     

颧部整形的治疗进展

面部轮廓整形目前在整形外科手术中占有越来越大的比例,中面部作为全脸的中心位置,是面部轮廓整形的重点区域,而颧部整形对于面部轮廓的改善有着很重要的作用。颧骨形态特殊,四突、三面、五缘,位置表浅体表投影明显,是面颧部凸起的重要结构,根据解剖学的研究在术前对患者进行三维CT成像测量,分析颧骨形态及手术方案范围,根据手术目的选择相对应的标记点,从而决定手术达到的预期效果及左右对称性有着非常重要作用。颧骨手术包括颧骨后缩的注射填充和假体填充,颧骨肥大的不同截骨手术,口内入路L型截骨术、冠状切口入路双侧截骨术。颧骨手术并发症较少,常见的如血肿、神经损伤、面部不对称及面部软组织松弛下垂等。     

A Posttraumatic Dilated,Proptotic Eye Does Not Always Need a Lateral Canthotomy! A Review of Superior Orbital Fissure Syndrome for Emergency Physicians

BackgroundSuperior orbital fissure syndrome (SOFS) is a rare constellation of findings consisting of ophthalmoplegia, ptosis, a fixed dilated pupil, forehead anesthesia, and loss of the corneal reflex. This syndrome, though rare, is most often encountered in trauma with individuals sustaining a facial fracture.Case ReportWe present a case of a young woman who was diagnosed with SOFS after a fall in her house, hitting her face on a nightstand. Treatment consisted of high-dose i.v. steroids followed by a taper with close follow-up in the Ophthalmology clinic. We provide a brief review of SOFS, including treatment considerations and follow-up.Why Should an Emergency Physician Be Aware of This?SOFS can be easily overlooked in an individual presenting to the emergency department after facial trauma with proptosis. However, a thorough examination of the eye, visual acuity, and intraocular pressure will focus the physician on SOFS rather than the need for immediate decompression via lateral canthotomy. This report describes a traumatic cause of SOFS, the pathophysiology and treatment, and summarizes existing literature.     

Diabetic myonecrosis,an uncommon presentation of diabetes mellitus in tropical area: A case report

Diabetic myonecrosis is an uncommon complication related to long‐standing poorly controlled diabetes. A 33‐year‐old Sudanese male patient with type one diabetes presented with progressive, severe bilateral thigh pain with low‐grade fever. Laboratory results show hyperglycemia with ketonuria and elevated creatine kinase but normal white cell blood count. The patient was diagnosed initially with diabetic ketoacidosis with pyomyositis and received analgesic and insulin; the patient partially improved. After the second evaluation, bilateral thigh MRI was requested and shows diffuse edema involving the medial muscle group of the upper third of the right side with intramuscular facial edema, appearing as low signal in T1 and high signal in T2 and fat suppression images with no evidence of collection or abscess. Diagnosis of diabetic myonecrosis was made. The patient was managed conservatively and discharge on aspirin with full recovery.     

Dracula''s Teeth Syndrome

OBJECTIVE: To describe a patient with facial pain, ipsilateral facial dystonia, and phantom supernumerary teeth, beginning after resection of hypertrophic gums. She had familial sensorineural deafness. BACKGROUND: Atypical cranial dystonia subsequent to dental procedures is associated with facial pain, dysesthesias, or phantom phenomena, but not with phantom supernumerary teeth. Patients with migraine are susceptible to experience cephalic phantom phenomena after dental procedures. Wynne syndrome is a hereditary autosomal dominant disorder characterized by congenital sensorineural deafness and supernumerary teeth. METHODS: Clinical examination, computerized tomography of paranasal sinuses and facial bones, magnetic resonance imaging of the brain, cranial electrophysiological testing, and electroencephalogram. RESULTS: A 52-year-old woman with history of migraine without aura for 40 years exhibited focal right facial involuntary tonic contracture accompanying chronic severe pain over the same area after gum resection. She reported a daily sensation of having two extra upper canine teeth pressing on her tongue, simulating vampire's ("Dracula's") teeth. She had high-frequency bilateral sensorineural deafness. Her computerized tomography studies, brain magnetic resonance imaging, and cranial electrophysiological testing were normal. CONCLUSION: This patient with chronic migraine had atypical cranial dystonia beginning after a dental procedure. Her dystonia was complicated by the unusual phenomenon of phantom supernumerary teeth. This condition may be misdiagnosed as atypical or psychogenic facial pain when facial dystonia is localized or subtle and is, therefore, confused with an idiosyncratic gesture or habitual spasm. Her signs and symptoms are reminiscent of Wynne syndrome.     

Progressive disfiguring facial masses with pupillary axis obstruction from Morbihan syndrome: A case report

BACKGROUNDMorbihan syndrome is a rare illness, which presents with recurrent pitting edema on the upper two thirds of the face causing facial contour deformities, and Morbihan syndrome obstructing the pupillary axis with ptosis was seldomly reported.CASE SUMMARYA 59-year-old woman presented with a 15-year history of facial swelling that progressively invaded bilaterally from the inner canthus and eyelids outwards. Imaging examination indicated that the bilateral periorbital and the left temporal soft tissues had swelling without cranium invasion. Histopathological analysis showed a large amount of lymphocyte infiltration, and immunohistochemistry showed positive expression of CD68 in clear-cut granulomas and D2-40 in the lymphatic endothelium. Finally, the clinical diagnosis of Morbihan syndrome was confirmed.CONCLUSIONMorbihan syndrome is an infrequent and refractory disease, which is characteristic with recurrent woody facial edema on the upper two thirds of the face. Solid facial edema is persistent and non-pitting, causing facial contour deformities and even vision field impairment. The diagnosis of Morbihan syndrome depends on clinical features, imaging information, and pathology. Blepharoplasty is optional to improve the visual field for these patients with severe pupillary axis obstruction.     

The Tolosa–Hunt syndrome in children: a case report

Tolosa–Hunt syndrome (THS) is characterized by unilateral painful ophthalmoplegia with oculomotor paresis, associated with an idiopathic granulomatous inflammation involving the cavernous sinus, with a typical relapsing–remitting course. We report a case of an 8-year-old girl who was admitted because of an ophthalmoplegia with exotropia and ptosis of the left eyelid, accompanied by diplopia and left sovraorbital pain. The clinical data, neuroradiological findings and response to steroid treatment suggested THS, as defined by the 2004 International Classification of Headache Disorders (ICHD)-II criteria. THS must be considered a possible cause of painful ophthalmoplegia in childhood, as well as in adults, and confirmed with a focused neuroradiological investigation. The few paediatric cases described in the literature that meet the 2004 ICHD-II criteria are not sufficient to identify possible differences between the paediatric and the adult forms. Every new paediatric case should therefore be reported in order to gather and compare further information.     

Acute pseudotumor of the orbit

A case is presented of a 22-year-old man with a history of bilateral eye injection, lacrimation, and rhinorrhea. The right orbit was inflamed and its globe proptotic, with medial and lateral gaze deficits. Tomography revealed swelling about the right lacrimal gland. He was admitted and treated with high-dose steroids, which caused regression of his symptoms. This presentation and response to steroids is pathognomonic for acute pseudotumor of the orbit, a condition rarely described in the emergency medicine literature. If left untreated, treudotumor of the orbit may progress to blindness and ophthalmoplegia.     

Acute sphenoid sinusitis induced blindness: a case report

Background

Acute, isolated sphenoid sinusitis is a rare but potentially devastating clinical entity. Missing this diagnosis can lead to permanent vision loss due to injury of the optic nerve. Patients may present with preseptal inflammation, lid edema, chemosis, or ophthalmoplegia.

Objective

We report a case of acute sphenoid sinusitis in a 10-year-old child who presented to the Emergency Department with essentially painless vision loss.

Case Report

Previously healthy, the patient reported progressive decrease in vision in her right eye for the 5 days prior. Other than blurred vision in the right eye, she complained of a mild frontal headache and right eye irritation the past week, which had abated. On examination, she was reading a book with her head tilted to one side. She had no photophobia, or facial or eyelid swelling. Her pupils were 5 mm bilaterally, but the right was non-reactive to light. She was unable to see two fingers 6 inches in front of her face (right eye), whereas her visual acuity on the left was 20/25. She had bilateral elevated intraocular pressures and a Marcus Gunn pupil on the right. Ophthalmology was consulted and the diagnosis of acute sphenoid sinusitis causing compression and vascular compromise to the optic nerve was diagnosed ultimately by magnetic resonance imaging. The patient was transferred to the nearest pediatric specialty hospital, where an emergent endoscopic sphenoidotomy was performed. The patient’s vision subsequently returned.

Conclusion

Sphenoid sinusitis should be considered in patients presenting with acute vision loss. Awareness, early diagnosis, and intervention help prevent permanent complications.     

A case of cephalic tetanus with unilateral ptosis and facial palsy

Cephalic tetanus is defined as a combination of trismus and paralysis of one or more cranial nerves. Cranial nerves III, IV, VI, VII, and XII may be affected, but the facial nerve is most frequently implicated. A 64-year-old female visited hospital for left ptosis followed by facial palsy after a left forehead abrasion in a car accident. At nine days post injury, left ptosis developed, left facial palsy developed twelve days post injury, and at fifteen days post injury, trismus and dysphagia developed. The following day, there was progression of symptoms to generalized tetanus, such as dyspnea and generalized rigidity. Videofluoroscopic swallow study showed penetration and aspiration. We report a case of cephalic tetanus with ptosis, facial palsy, and dysphagia, which progressed to generalized tetanus.     

Bilateral painful idiopathic ophthalmoplegia: a case report

Around 3% of the individuals with painful ophthalmoplegia have bilateral complaints. In the vast majority of these cases, appropriate investigation demonstrates a secondary etiology, and we are not aware of idiopathic cases reported. Herein we report a case of bilateral ophthalmoplegia where extensive investigation did not suggest a secondary cause.     

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。     

Bilateral Sympathetic Dysfunction in Post-Traumatic Headaches

SYNOPSIS
Two patients developed generalized throbbing headaches, tenderness of the anterior neck and unilateral ptosis following whiplash injury. They were systematically evaluated for involvement of the central nervous system, carotid arteries, vagi and cervical sympathetic nerves. Biochemical study of pupils revealed bilateral postganglionic Horner's syndrome. Thermal-induced facial sweating showed supraorbital anhidrosis. Follow-up 18 to 54 months after injury demonstrated partial improvement in pupillary functions and headaches but persistent supraorbital anhidrosis. Pertinent literature on sympathetic dysfunction and post-traumatic headaches is reviewed, and the reasons and implications for sympathetic involvement in hyperextension-hyperflexion injury are discussed.     

Transcranial Color-Coded Sonography for the Detection of Cerebral Veins and Sinuses and Diagnosis of Cerebral Venous Sinus Thrombosis

This study aimed to determine the detection rate of transcranial color-coded sonography (TCCS) of cerebral veins and sinuses and to explore the diagnostic accuracy of TCCS for straight sinus (SS) and transverse sinus (TS) thromboses. The detection rates of cerebral veins and sinuses using TCCS and contrast-enhanced TCCS (CE-TCCS) were analyzed. The diagnostic accuracy of CE-TCCS was evaluated. Median time from symptoms to CE-TCCS was 10 (range, 1–150) d. The detection rate of bilateral basal veins of Rosenthal was 100% by CE-TCCS, followed by right TS (91.89%), SS (88.12%), left TS (74.59%) and vein of Galen (70.27%). Compared with magnetic resonance imaging/magnetic resonance venography, CE-TCCS showed 100% sensitivity and 96.3% specificity for SS thrombosis, 100% and 100% for right TS thrombosis and 100% and 94.4% for left TS thrombosis. In conclusion, CE-TCCS shows high identification rates of cerebral veins and sinuses and a high diagnostic accuracy for SS and TS thrombosis.     

颅内静脉系统窦汇区流向的血管造影分析

【目的】观察窦汇区的分流类型，为临床结扎或切除一侧横窦或乙状窦及预内静脉提供依据。材料与方法：对98例窦汇区造影片进行回顾性分析，所有病例均行双侧颈动脉及推动脉造影。结果：左右颈动脉造影主要右侧分流分别占55．1％、53．0％，椎动脉造影两侧对称分流及主要左侧分流分别占39．6％、33．7％，而单一左侧及单一右侧分流少见。结论：临床涉及结扎一侧静脉窦的患者，术前应判明窦汇区分流方向。     

Bilateral Episodic Mydriasis as a Migraine Equivalent in Childhood: a Case Report

We report a 14-year-old girl who presented with yearly attacks of bilateral internal ophthalmoplegia, nausea and headache, since the age of nine. The episodic isolated bilateral mydriasis in this child is believed to be a migraine equivalent.     

Chronic daily headache: When to suspect sinus disease

Migraine may be responsible for many headaches thought to be caused by the sinuses. Patients complaining of “sinus headache” must have a complete ear, nose, and throat examination. Occasionally, chronic headaches may arise from the sinuses; a thorough history is important to search for symptoms of facial pain and pressure along with other nasal sinus symptoms.     

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。     

Frontoethmoid sinus osteoma as a cause of subperiosteal orbital abscess

A 65-year-old woman presented with severe periorbital pain and swelling of the left eye, with complete ptosis, proptosis, and conjunctival chemosis. The eye was in a hypotropic position, and activity in the left superior rectus was inadequate. A firm, elastic, 2-cm mass was palpated near the superior orbital rim. Computed tomography revealed a subperiosteal abscess (SPA) at the superior portion of the orbit and a large frontoethmoidal sinus osteoma. After the SPA had been surgically drained and the osteoma completely removed, the patient recovered, with resolution of proptosis, ptosis, and motility limitations. Osteomas of the paranasal sinuses are usually asymptomatic and rarely cause SPA and orbital cellulitis. Therefore, osteoma cases must be closely followed to ensure that early diagnosis and treatment of SPA are successful in preventing severe visual loss and rescuing the patient’s vision.