medulla oblongata

  • 文章类型: Journal Article
    背景:Opalski综合征,延髓外侧综合征(LMS)的亚型,由于其不同的临床表现和潜在的非典型症状,提出了挑战。了解其流行病学,临床表现,结果对于优化患者护理至关重要。
    方法:系统综述,遵循PRISMA2020指导方针,进行了全面分析Opalski综合征。数据来自PubMed,Scopus,WebofScience,包括Embase,搜索于2023年5月进行。纳入病例报告的合格研究,案例系列,和编辑信。
    结果:该综述涵盖了1984年至2024年的78项研究,涉及94例Opalski综合征患者。分析显示男性占主导地位(76.60%),男女比例为3.1:1。常见的危险因素包括高血压(63.54%),糖尿病(32.29%),吸烟(32.39%),和酒精消费量(22.91%)。在5大洲的22个国家报告了Opalski综合征病例,亚洲是最普遍的地区(77.08%)。最初的表现通常包括共济失调或手指到鼻子和膝盖到脚跟测试阳性,头晕或眩晕,偏瘫,眼球震颤,霍纳的标志,第五或第七脑神经麻痹,都发生在50%以上的病例中。磁共振成像(MRI)和磁共振血管造影(MRA)等神经影像学技术对于诊断至关重要。尽管死亡率为4.16%,自2014年以来没有死亡报告,这表明临床管理取得了进步.
    结论:目标风险因素管理,早期识别症状,和利用先进的神经成像技术对于优化患者预后至关重要。临床医生必须保持对Opalski综合征的了解,以提高诊断准确性并调整治疗策略。
    BACKGROUND: Opalski syndrome, a subtype of lateral medullary syndrome (LMS), poses challenges due to its diverse clinical presentations and potential atypical symptoms. Understanding its epidemiology, clinical manifestations, and outcomes is crucial for optimizing patient care.
    METHODS: A systematic review, following PRISMA 2020 guidelines, was conducted to comprehensively analyze Opalski syndrome. Data from PubMed, Scopus, Web of Science, and Embase were included, with the search conducted in May 2023. Eligible studies spanned from included case reports, case series, and editorial letters.
    RESULTS: The review encompassed 78 studies from 1984 to 2024, involving 94 patients with Opalski syndrome. The analysis revealed a male predominance (76.60 %) with a male-to-female ratio of 3.1:1. Common risk factors included hypertension (63.54 %), diabetes mellitus (32.29 %), smoking (32.39 %), and alcohol consumption (22.91 %). Opalski syndrome cases were reported in 22 countries across 5 continents, with Asia being the most prevalent region (77.08 %). Initial presentations commonly included ataxia or positive finger-to-nose and knee-to-heel tests, dizziness or vertigo, hemiparesis, nystagmus, Horner\'s sign, and 5th or 7th cranial nerve palsy, all occurring in more than 50 % of cases. Neuroimaging techniques such as magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) were crucial for diagnosis. Despite a mortality rate of 4.16 %, no deaths have been reported since 2014, indicating advancements in clinical management.
    CONCLUSIONS: Targeted risk factor management, early recognition of symptoms, and utilization of advanced neuroimaging techniques are essential for optimizing patient outcomes. Clinicians must remain informed about Opalski syndrome to enhance diagnostic accuracy and tailor treatment strategies.
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  • 文章类型: Journal Article
    背景:室管膜囊肿(EC)通常存在于侧脑室附近,近室,或颞顶区域。以前的病例还发现了脑干的幕下EC,小脑,和蛛网膜下腔。他们大多是无症状的。在本文中,我们介绍了有史以来第一例手术治疗的有症状的髓质室管膜囊肿,以及关于脑干其他部位EC的文献的全面回顾。
    方法:这位51岁的女性出现听力损失,头晕,复视,和共济失调.射线照相成像表明髓质中存在非增强性病变,对脑干有质量影响。病理检查证实其表征为室管膜囊肿。该患者在神经导航下接受了枕下开颅手术,以开窗治疗髓质室管膜囊肿,术中超声和术中神经监测。组织病理学检查证实了室管膜囊肿的诊断。在一个月的随访中,KPS为90,ECOGPS为1,共济失调得到改善,复视完全消退.
    结论:由于它们的稀有性和与其他囊性结构的潜在相似性,EC可能被忽视或错误诊断,导致管理不善和手术灾难。因此,全面了解和认识其独特特征对于准确诊断和适当管理至关重要。
    BACKGROUND: Ependymal cysts (EC) typically present supra-tentorially near the lateral ventricle, juxta ventricular, or temporoparietal regions. Previous cases have also identified infratentorial EC of the brainstem, cerebellum, and subarachnoid spaces. They are mostly asymptomatic. In this paper, we present the first-ever case of a symptomatic medullary ependymal cyst treated with surgery, along with a comprehensive review of the literature on EC of other parts of the brain stem.
    METHODS: This 51-year-old female presented with hearing loss, dizziness, diplopia, and ataxia. Radiographic imaging indicated the presence of a non-enhancing lesion in the medulla with a mass effect on the brainstem. Pathological examination confirmed its characterization as an ependymal cyst. The patient underwent a suboccipital craniotomy for the fenestration of the medullary ependymal cyst under neuro-navigation, Intra-op ultrasound and intra-operative neuro-monitoring. Histopathological examination confirmed the diagnosis of an ependymal cyst. At one month follow-up, her KPS is 90, ECOG PS 1, and her ataxia has improved with complete resolution of diplopia.
    CONCLUSIONS: Due to their rarity and potential similarity to other cystic structures, EC may be overlooked or incorrectly diagnosed resulting in mismanagement and surgical disaster. Therefore, a comprehensive understanding and awareness of their distinct characteristics are essential for accurate diagnosis and appropriate management.
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  • 文章类型: Case Reports
    延髓是中枢神经系统生殖细胞肿瘤(GCT)最罕见的发生部位之一。由于关于流行病学的数据很少,临床表现,最佳干预,和长期预后,我们旨在通过介绍我们的代表性案例并对文献进行定量审查,来描述这一罕见实体的特征。一名24岁的妇女因眩晕和吞咽困难来到我们部门。磁共振成像显示,延髓引起的外生性病变均一增强,并延伸至第四脑室。进行了手术切除,并对纯生殖细胞瘤进行了组织学诊断。患者接受了化疗和全心室放疗。术后4个月无复发。根据文献,延髓GCT的预后似乎并不比典型部位差.引人注目的特征,包括在年龄较大时发生,女性优势,生殖细胞瘤的优势值得注意。局部复发的模式表明广泛的辐射覆盖不是先决条件。需要特别注意心脏和呼吸功能,这是导致死亡的主要因素。
    The medulla oblongata is one of the rarest sites of occurrence for germ cell tumors (GCTs) of the central nervous system. As there is scant data regarding epidemiology, clinical presentations, optimal intervention, and long-term prognosis, we aimed to delineate the features of this rare entity by presenting our representative case and performing a quantitative review of the literature. A 24-year-old woman presented to our department with vertigo and swallowing difficulties. Magnetic resonance imaging revealed a homogenously enhanced exophytic lesion arising from the medulla oblongata and extending to the fourth ventricle. Surgical resection was performed and a histological diagnosis of pure germinoma was made. The patient underwent chemotherapy and whole-ventricular irradiation. No recurrence has been experienced for 4 months after the surgery. According to the literature, the prognosis of GCTs at the medulla oblongata seems no worse than those at typical sites. Striking features including occurrence at an older age, female preponderance, and a predominance of germinoma were noteworthy. The pattern of local recurrence suggests extensive radiation coverage is not a prerequisite. Special attention is needed for cardiac and respiratory functions as the main factors eliciting mortality.
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  • 文章类型: Case Reports
    目的:视神经脊髓炎谱系障碍(NMOSD)是一种罕见的免疫介导的中枢神经系统(CNS)脱髓鞘疾病。缺乏与NMOSD相关的病态窦房结综合征(SSS)的报道;因此,现报告2例NMOSD患者发生SSS。
    方法:患者均为男性,表现为区域后综合征。脑部MRI显示延髓背侧有病变。当在他们的血清中发现水通道蛋白-4抗体时,他们被诊断为NMOSD。在住院期间,病例1也观察到心率缓慢和几次晕厥发作。而Holter监测显示窦房结停顿(10-11s),并诊断为SSS。安装了起搏器。病例2出现呼吸骤停,随后出现心脏骤停。他通过肾上腺素注射和心肺复苏成功复苏。通过免疫疗法,他们的神经功能变得稳定,心率和血压恢复到基线.
    结论:病窦综合征是一种危及生命的并发症,严重的心律失常应被视为与NMOSD相关的区域后综合征的潜在结果。
    OBJECTIVE: Neuromyelitis optica spectrum disorder (NMOSD) is a rare immune-mediated demyelinating disease of the central nervous system (CNS). There is a lack of reports of sick sinus syndrome (SSS) associated with NMOSD; thus, we hereby report two cases of patients with NMOSD who developed SSS.
    METHODS: The patients were both male and presented with area postrema syndrome. Brain MRI showed lesions in the dorsal part of their medulla oblongata. They were diagnosed with NMOSD when aquaporin-4 antibodies were found in their serum. Slow heart rates and several episodes of syncope were also observed in case 1 during hospitalization, while Holter monitoring showed sinus pauses (10-11 s) and SSS was diagnosed. A pacemaker was fitted. Case 2 had a respiratory arrest followed by a subsequent cardiac arrest. He was successfully resuscitated with epinephrine injection and cardiopulmonary resuscitation. Through immunotherapy, their neurological functions became stable and heart rate and blood pressure returned to the baseline.
    CONCLUSIONS: Since sick sinus syndrome is a life-threatening complication, serious heart arrhythmias should be considered as a potential result of area postrema syndrome associated with NMOSD.
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  • 文章类型: Case Reports
    颅内生殖细胞瘤是罕见的肿瘤,占原发性颅内肿瘤的0.5-2%。虽然它们通常发生在松果体中,鞍上区,基底神经节,还有丘脑,在延髓中出现的生殖细胞瘤非常罕见。诊断延髓生殖细胞瘤具有挑战性,可能导致误诊和预后不良。
    我们介绍了一例29岁男子左腿麻木的病例。放射学发现发现延髓有对比增强的病变。病人接受了肿瘤活检,术中病理诊断(IOD)怀疑诊断为延髓生殖细胞瘤。在确认诊断为生殖细胞瘤后,他接受了放化疗。延髓中出现的颅内生殖细胞瘤与其他位置的生殖细胞瘤不同,因为其在20多岁的个体中发病率较高,女性占主导地位。
    遇到延髓病变时,生殖细胞瘤应被视为鉴别诊断之一,应制定相应的手术策略,包括IOD.
    UNASSIGNED: Intracranial germinomas are rare tumors, accounting for 0.5-2% of primary intracranial neoplasms. While they typically occur in the pineal gland, suprasellar region, basal ganglia, and thalamus, germinomas arising in the medulla oblongata are exceptionally rare. Diagnosis of medulla oblongata germinoma is challenging, potentially leading to misdiagnosis and poor prognosis.
    UNASSIGNED: We present a case of a 29-year-old man complaining of left leg numbness. Radiological findings revealed a contrast-enhanced lesion in the medulla oblongata. The patient underwent tumor biopsy, and intraoperative pathological diagnosis (IOD) suspected the diagnosis of medulla oblongata germinoma. He underwent chemoradiotherapy after confirming the diagnosis of germinoma. Intracranial germinoma arising in the medulla oblongata differs from germinomas in other locations due to its higher incidence in individuals in their 20s and a slight female predominance.
    UNASSIGNED: When encountering lesions in the medulla oblongata, germinoma should be considered as one of the differential diagnoses, and surgical strategies including IOD should be planned accordingly.
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  • 文章类型: Review
    背景:延髓外侧综合征是脑干梗死的最常见类型。延髓外侧综合征导致相应的颅神经核和孤束核的损伤,眩晕,同侧共济失调,交叉感觉障碍,霍纳的标志,球麻痹,和其他潜在的症状或体征。然而,以心律失常和其他自主神经功能障碍为主要表现的病例较少见。临床上,猝死偶尔发生在延髓外侧综合征,这可能与严重的心律失常有关。这些患者可能遭受危及生命的心律失常甚至心脏骤停,应密切监测生命体征,以防止猝死。在年轻患者中,椎动脉夹层是最常见的原因。
    方法:这里,我们介绍一例由椎动脉夹层引起的延髓外侧综合征,并伴有严重的心动过缓。患者是一名49岁的男子,他因“左侧四肢和右侧面部突然麻木1小时”而入院。心电图(ECG)监测显示反复心率下降到低至23次/分钟,2-3秒后心率逐渐增加至35-55次/分钟。头部磁共振成像(MRI)检查显示延髓右背外侧脑梗死。数字减影血管造影(DSA)显示右椎动脉夹层动脉瘤。我们紧急放置了临时起搏器,随后用血小板聚集抑制剂保守治疗,血管软化剂和改善侧支循环。选择性弹簧圈栓塞椎动脉夹层动脉瘤和支架植入。在门诊随访中,患者预后良好。
    结论:应优先考虑延髓外侧综合征患者的临床治疗,在观察的早期阶段进行密切的心脏监测,并根据需要放置起搏器和气管插管以防止不良事件。
    BACKGROUND: Lateral medullary syndrome is the most common type of brainstem infarction. Lateral medullary syndrome results in damage to the corresponding cranial nerve nuclei and the nucleus tractus solitarius, with vertigo, ipsilateral ataxia, crossed sensory disturbances, Horner\'s sign, bulbar palsy, and other underlying symptoms or signs. However, cases with cardiac arrhythmia and other autonomic dysfunctions as the primary manifestations are less common. Clinically, sudden death occasionally occurs in patients with lateral medullary syndrome, which may be associated with severe cardiac arrhythmia. These patients may suffer in life-threatening arrhythmia and even cardiac arrest, and vital signs should be closely monitored to prevent sudden death. In younger patients, vertebral artery dissection is the most common cause.
    METHODS: Here, we present a case of lateral medullary syndrome caused by vertebral artery dissection with severe bradycardia. The patient was a 49-year-old man who was admitted with \"sudden onset of numbness in the left limbs and right side of the face for 1 hour\". Electrocardiogram (ECG) monitoring showed a repeated heart rate decrease to as low as 23 beats/min, followed by a gradual increase in heart rate to 35-55 beats/min after 2-3 seconds. Head magnetic resonance imaging (MRI) examination revealed right dorsolateral cerebral infarction of the medulla oblongata. Digital subtraction angiography (DSA) revealed a right vertebral artery dissecting aneurysm. We performed an emergency placement of a temporary pacemaker, followed by conservative treatment with platelet aggregation inhibitors, vascular softening agents and improved collateral circulation. Elective spring coil embolization of the vertebral artery dissecting aneurysm and stent implantation were performed. At outpatient follow-up, the patient had a good prognosis.
    CONCLUSIONS: Clinical management of patients with lateral medullary syndrome should be prioritized, with close cardiac monitoring at the early stages of observation and pacemaker placement and tracheal intubation as required to prevent adverse events.
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  • 卵黄囊瘤(YST)是一种罕见的原发性脑肿瘤,几乎仅发生在30岁以下的患者中。颅内生殖细胞肿瘤最常见于松果体和鞍上区域。延髓YSTs特别罕见。性腺外YST可能由于其特征而难以诊断,如生长模式的稀有性和多样性。此外,众所周知,他们的预后很差。我们在此报告一例50岁女性延髓YST。随访18个月,无肿瘤复发。
    Yolk sac tumor (YST) is a rare primary brain tumor that occurs almost exclusively in patients under 30 years old. Intracranial germ cell tumors are most frequently located in the pineal and suprasellar region. Medulla oblongata YSTs are particularly rare. Extragonadal YSTs may be difficult to diagnose because of their characteristics, such as the rarity and variety of growth patterns. Furthermore, they are known to have a very poor prognosis. We herein report a case of YST of the medulla oblongata in a 50-year-old woman. She was followed up for 18 months without any tumor recurrence.
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  • 文章类型: Review
    背景:脊髓空洞症通常伴随脊髓血管母细胞瘤(SHB)。它通常显示进展到延髓,被称为“syringcobulbia”,出现严重的症状,如吞咽困难和呼吸受损。对脊髓空洞症按时间顺序向空洞症进展的适当管理并不确定。本研究旨在揭示丁香球虫的临床和时间行为及其管理。
    方法:进行了5例SHB合并空洞症手术患者的单机构病例系列研究。连续术前磁共振成像扫描进行了进一步的详细分析,特别是关注脊髓空洞症的时间进展。进行文献综述以描述临床/影像学特征。
    结果:时序成像分析显示,尽管脊髓空洞症多年来进展相对稳定,它在发展为syrangobulbia时加速。在随后发生脊髓空洞症的区域观察到了髓内信号变化(“脊髓前裂”)。文献复习产生了另外15例SHB伴干燥球,共20起案件。球功能障碍4例(20%)。除1例仅进行了注射器切开术外,所有病例均进行了全切除。所有病例术后症状迅速改善,以及脊髓空洞症的即时成像分辨率。
    结论:与空洞症相关的症状往往会危及生命。值得注意的是,其解决可能与脊柱病变的手术切除几乎同步。脊髓空洞症的进展速度通常是稳定的,但是延伸到syrangobulbia时可能会加速。因此,强烈建议定期进行影像学随访,以确定当延髓前脊髓和脊髓空洞症向延髓上升时的最佳干预时机。
    Syringomyelia often accompanies spinal hemangioblastoma (SHB). It often shows progression to the medulla oblongata, dubbed as \"syringobulbia\", which presents critical symptoms such as dysphagia and respiratory compromise. Appropriate management of chronological syringomyelia progression toward syringobulbia is not set in stone. This study aims to unravel the clinical and chronological behavior of syringobulbia and its management.
    A single-institution case series study of 5 patients operated for SHB with syringobulbia was conducted. Serial preoperative magnetic resonance imaging scans were analyzed in further details, especially focusing on the chronological progression of syringomyelia. A literature review was performed to describe clinical/imaging characteristics.
    Chronological imaging analyses revealed that despite the relatively steady progression of syringomyelia over years, it accelerated when developing syringobulbia. Intramedullary signal change (\"presyringomyelia\") was observed in the area where syringomyelia subsequently occurred. Literature review yielded another 15 cases of SHB with syringobulbia, totaling 20 cases. Bulbar dysfunction was seen in 4 cases (20%). Gross total resection was performed in all cases except 1, which underwent just syringotomy. Rapid postoperative symptom improvement was observed in all cases, as well as immediate imaging resolution of syringomyelia.
    The symptoms associated with syringobulbia often become life-threatening. Notably, its resolution may be near-synchronous to surgical resection of the spinal lesion. The speed of progression of syringomyelia is usually steady, but it may accelerate when extending to syringobulbia. Regular imaging follow-up is thus highly recommended to determine the best timing of intervention when presyringomyelia and syringomyelia are ascending toward the medulla oblongata.
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  • 文章类型: Case Reports
    延髓中的畸胎瘤极为罕见,很少有报道。这些花岗岩肿瘤导致的严重和持续的脑干压迫可能导致潜在的致命损害。这里,我们报道了一例发生在延髓的畸胎瘤。这个15岁的男孩患有进行性步态障碍和四肢无力近13年。磁共振成像(MRI)显示延髓和C1-2脊柱中异常的混合肿块,经组织病理学检查证实为成熟畸胎瘤。然后,进行了手术切除,术后持续康复。经过一段时间的康复,该患者目前能够用棍子动员。没有出现局部复发的迹象。最后,手术切除仍然是畸胎瘤的首选治疗方法。
    Teratoma in the medulla oblongata is extremely infrequent and has been rarely reported. Severe and sustained brain stem compression resulting from these granitic tumors may cause potentially fatal impairment. Here, we reported a novel case of teratoma that occurred in the medulla oblongata. This 15 year-old boy suffered from a progressive gait disturbance and weakness of limbs for nearly 13 years. Magnetic Resonance Imaging (MRI) revealed an unusual mixed mass in the medulla oblongata and C1-2 spine, which was confirmed as mature teratoma by histopathological examination. Then, surgical resection was performed, followed by postoperative continuous rehabilitation. After a period of rehabilitation, this patient is currently able to mobilize with sticks. No signs of local recurrence occurred. Conclusively, surgical removal is still the preferred treatment for teratoma.
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  • 文章类型: Journal Article
    OBJECTIVE: Vertebral artery compression of the medulla is a rare vascular finding that causes a variety of clinical presentations, from asymptomatic to neurological disability. This article presents the largest literature review to date on medullary compression of the vertebral arteries.
    METHODS: An English literature search was performed using the PubMed database and the keywords vertebral artery tortuosity, vertebral artery compression, and medullary compression.
    RESULTS: A comprehensive literature search yielded 68 patients (57% male) with medullary compression by an intracranial vertebral artery (ICVA). The left side of the medulla was compressed in 44, the right side in 19, and bilateral in 7. The most common clinical symptom was weakness - 26 patients (36%) - 6 had quadriparesis and 6 had hemiparesis. 21 patients reported imbalance; 12 various sensory symptoms; 4 patients were asymptomatic.
    CONCLUSIONS: Understanding the anatomy of the vasculature can help mitigate future debilitating stroke symptoms. Concrete guidelines for revascularization surgery in symptomatic patients may also be effective. Future studies are needed to further clarify the prevalence, natural history, vascular etiology, and treatment of this condition, including asymptomatic patients and the likelihood that they will develop further neurological signs and disability.
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