cranial nerve

颅神经
  • 文章类型: Journal Article
    背景:三叉神经鞘瘤(TS)是颅内肿瘤,可引起明显的脑干压迫。TS切除可能是具有挑战性的,因为新的神经系统和颅神经缺陷的风险,特别是大(≥3厘米)或巨大(≥4厘米)TSs。由于先前的手术系列包括各种尺寸的TS,我们在此介绍我们通过显微外科手术切除治疗大型和巨大TS的临床经验.
    方法:这是一个回顾性研究,2012-2023年接受显微外科手术治疗的大型或巨型TS成人患者的单外科医生病例系列。
    结果:7例患者接受了TSs的显微外科手术切除(1例,6个巨人;4个男性;平均年龄39±14岁)。肿瘤分类为M型(硬膜间隙中窝;1例,14%),ME型(中窝颅外延伸;3例,43%),MP型(中、后窝2例,29%),或MPE型(中/后颅窝和颅外间隙;1例,14%)。6例患者接受额颞入路治疗(一名患者在同一坐位中结合经乳突开颅术,另一名患者采用延迟的经上颌入路),1例患者采用眶额颞入路治疗。5例(2例几乎全部切除)获得了全部切除。5例患者术前面部麻木,术后立即出现面部麻木,包括两个有恶化或新症状的。在平均22个月的随访中,有两名患者(28%)出现了新的非三叉神经颅神经缺陷。总的来说,80%的术前面部麻木患者和83%的面部麻木患者在术后过程中出现改善或消退。所有术前或术后新出现的非三叉神经肿瘤相关颅神经缺陷(4/4)的患者在随访中都有改善或消退。一名患者经历了保守治疗的肿瘤复发。
    结论:大型或巨大TSs的显微手术切除可以降低发病率和良好的长期颅神经功能。
    BACKGROUND: Trigeminal schwannomas (TSs) are intracranial tumors that can cause significant brainstem compression. TS resection can be challenging because of the risk of new neurologic and cranial nerve deficits, especially with large (≥ 3 cm) or giant (≥ 4 cm) TSs. As prior surgical series include TSs of all sizes, we herein present our clinical experience treating large and giant TSs via microsurgical resection.
    METHODS: This was a retrospective, single-surgeon case series of adult patients with large or giant TSs treated with microsurgery in 2012-2023.
    RESULTS: Seven patients underwent microsurgical resection for TSs (1 large, 6 giant; 4 males; mean age 39 ± 14 years). Tumors were classified as type M (middle fossa in the interdural space; 1 case, 14%), type ME (middle fossa with extracranial extension; 3 cases, 43%), type MP (middle and posterior fossae; 2 cases, 29%), or type MPE (middle/posterior fossae and extracranial space; 1 case, 14%). Six patients were treated with a frontotemporal approach (combined with transmastoid craniotomy in the same sitting in one patient and a delayed transmaxillary approach in another), and one patient was treated using an orbitofrontotemporal approach. Gross total resection was achieved in 5 cases (2 near-total resections). Five patients had preoperative facial numbness, and 6 had immediate postoperative facial numbness, including two with worsened or new symptoms. Two patients (28%) demonstrated new non-trigeminal cranial nerve deficits over mean follow-up of 22 months. Overall, 80% of patients with preoperative facial numbness and 83% with facial numbness at any point experienced improvement or resolution during their postoperative course. All patients with preoperative or new postoperative non-trigeminal tumor-related cranial nerve deficits (4/4) experienced improvement or resolution on follow-up. One patient experienced tumor recurrence that has been managed conservatively.
    CONCLUSIONS: Microsurgical resection of large or giant TSs can be performed with low morbidity and excellent long-term cranial nerve function.
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  • 文章类型: Journal Article
    背景:神经结节病很少见,在其表现形式中,只有少数病例报道了神经根受累。因此,神经结节病的磁共振成像(MRI)发现,特别是那些涉及神经根的,在文献中很少见。
    方法:我们介绍了神经结节病累及颈神经根和颅神经,同时进行系统的文献综述。
    结果:一名28岁女性突然出现右侧面部麻木以及左上肢和左手疼痛。初始脑和脊柱MRI显示左Meckel的洞穴/三叉神经中T2等高信号强度的隆起块,以及右侧C6和C7神经根的弥漫性肿大。2个月时的随访MRI显示,初始病变的大小减小,对侧出现新的相似病变(右Meckel洞穴,左C3-C8神经根)。特别是,涉及神经根的病变表现为沿神经根的中央扩大,不涉及相邻的脊髓。所有这些病变都表现出增强,导致结节病和淋巴瘤之间的区别。结节病随后通过肺门淋巴结活检证实。
    结论:本报告提出了涉及脊神经根的神经结节病的独特MRI特征,代表了同类中的第一个,并描述了整个临床过程中MRI发现的演变。
    BACKGROUND: Neurosarcoidosis is rare, and among its manifestations, nerve root involvement has been reported in only a few cases. Therefore, magnetic resonance imaging (MRI) findings of neurosarcoidosis, particularly those involving nerve roots, are scarce in the literature.
    METHODS: We presented the case of neurosarcoidosis involving cervical nerve roots and cranial nerves, alongside a systematic literature review.
    RESULTS: A 28-year-old female suddenly developed right facial numbness as well as left upper extremity and left hand pain. Initial brain and spine MRI showed a bulging mass of T2 iso-to-high signal intensity in the left Meckel\'s cave/trigeminal nerve, as well as diffuse enlargement of the right C6 and C7 nerve roots. Follow-up MRI at 2 months revealed a reduction in the size of the initial lesion and the appearance of new similar lesions on the contralateral side (right Meckel\'s cave, left C3-C8 nerve roots). In particular, the lesions involving the nerve roots demonstrated central enlargement along the nerve roots, without involvement of the adjacent spinal cord. All these lesions exhibited enhancement, leading to the differentiation between sarcoidosis and lymphoma. Sarcoidosis was subsequently confirmed through biopsy of a hilar lymph node.
    CONCLUSIONS: This report presents a distinctive MRI feature of neurosarcoidosis involving spinal nerve roots, representing the first of its kind, and describes the evolution of MRI findings throughout the clinical course.
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  • 文章类型: Case Reports
    背景:海绵状畸形(CM)是没有明确定义的壁的薄壁正弦血管簇。虽然它们可以发生在神经轴的任何地方,颅神经(CN)CMs是罕见的。
    方法:我们报告了一名47岁男性,患有渐进性CNIII性麻痹。最初的成像显示没有明显的发现,但随访MRI显示沿CNIII有一个生长的病变。术中发现证实了CNIIICM。CNIIICM的诊断和治疗是复杂的。放射学发现缺乏特异性,对于孤立的CNIII麻痹和异常的放射学发现的患者,需要考虑各种诊断。
    结论:手术是金标准,旨在完全切除病灶,同时最大限度地减少神经系统并发症。
    BACKGROUND: Cavernous malformations (CMs) are clusters of thin-walled sinusoidal vessels without well-defined walls. Though they can occur anywhere in the neuroaxis, cranial nerve (CN) CMs are rare.
    METHODS: We report a 47-year-old male with gradual CN III palsy. Initial imaging showed no significant findings, but a follow-up MRI revealed a growing lesion along CN III. Intraoperative findings confirmed a CN III CM. Diagnosing and treating CN III CM are complex. Radiological findings lack specificity, requiring consideration of various diagnoses for patients with isolated CN III palsy and abnormal radiological findings.
    CONCLUSIONS: Surgery is the gold standard, aiming for complete lesion removal while minimizing neurological complications.
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  • 文章类型: Case Reports
    一名11岁的男性拉布拉多十字架出现单侧前庭体征,同侧面部麻痹,中度迟钝,ptyalism,和轻瘫.脑部MRI显示弥漫性,多灶性T2/FLAIR在包括髓质在内的大脑各个区域发生高强度变化,中脑,pons,丘脑及右侧大脑半球轻度多焦对比增强。患者进展为三联肌,并伴有广泛性伸肌张力增加和三联肌。诊断为全身性破伤风,并开始使用抗生素,骨骼肌松弛剂和破伤风抗毒素并完全康复。据作者所知,这是首例犬破伤风病例,其中出现的体征涉及颅神经功能障碍,以及首例描述犬破伤风在中枢神经系统内的MRI变化的报告。
    An 11 years old male Labrador cross presented with unilateral vestibular signs, ipsilateral facial paresis, moderate obtundation, ptyalism, and paraparesis. MRI of the brain revealed diffuse, multifocal T2/FLAIR hyperintense changes throughout various regions of the brain including the medulla, midbrain, pons, thalamus and right cerebral hemisphere with mild multifocal contrast enhancement. The patient progressed to trismus with generalized increased extensor tone and risus sardonicus. A diagnosis of generalized tetanus was made and the patient was started on antibiotics, skeletal muscle relaxants and tetanus antitoxin and made a full recovery. To the best of the authors\' knowledge, this is the first reported case of canine tetanus in which the presenting signs involved cranial nerve dysfunction as well as the first report describing MRI changes in canine tetanus within the central nervous system.
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  • 文章类型: Journal Article
    RamsayHunt综合征是急性感染后膝状神经节中潜伏水痘带状疱疹病毒再激活的临床表现。它通常与急性周围性面神经麻痹有关,耳囊泡疹和急性感觉神经性听力损失。然而,在一些罕见的情况下,其他颅神经受累,特别是舌咽部和迷走神经。我们介绍了一名56岁的男性患者,他被诊断患有急性咽炎。两天后,病人出现构音障碍,声音嘶哑,左侧面部无力.考虑到患者的心血管事件史,怀疑是急性中风。磁共振成像排除了急性脑血管意外。患者的耳痛加重,他报告听力损失。临床检查显示左侧面部面瘫,左耳廓红斑皮疹,患者左鼓膜上存在多个囊泡,软腭左侧溃疡性病变和同侧腭咽麻痹。间接喉镜检查显示会厌左侧的溃疡性病变和左声带麻痹。患者被诊断为RamsayHunt综合征伴颅多发性神经病。口服阿昔洛韦和口服泼尼松龙。出院后七个月,面瘫改善至低度,柔性喉镜检查显示左声带恢复正常运动。RamsayHunt综合征可伴有其他颅神经受累。既往高血压危象和心肌梗死史可能会影响RamsayHunt综合征的诊断,尤其是当它有非典型的表现时。然而,对于单侧多发性颅神经麻痹的患者,应考虑这种诊断。
    Ramsay Hunt syndrome is a clinical manifestation of the reactivation of latent varicella zoster virus in the geniculate ganglion after acute infection. It is commonly associated with an acute peripheral facial nerve paralysis, auricular vesicular eruption and acute sensorineural hearing loss. However, in some rare cases the involvement of other cranial nerves, especially the glossopharyngeal and vagal is described. We present a 56-year-old male patient who was diagnosed with acute pharyngitis. Two days later, the patient developed dysarthria, hoarseness, and left side facial weakness. Considering a patient\'s history of cardiovascular events, the acute stroke was suspected. Magnetic resonance imaging ruled out an acute cerebrovascular accident. The patient\'s otalgia aggravated and he reported hearing loss. Clinical examination revealed facial paralysis affecting the left side of the face, erythematous rash on the left auricle, multiple vesicles present on patient\'s left tympanic membrane, ulcerous lesion on the left side of the soft palate and ipsilateral velopharyngeal palsy. Indirect laryngoscopy revealed ulcerous lesions in the left side of the epiglottis and left vocal cord paralysis. The patient was diagnosed with Ramsay Hunt syndrome with cranial polyneuropathy. Oral acyclovir and oral prednisolone were administered. Seven months after discharge, facial paralysis improved to lower grade and flexible laryngoscopy showed that the left vocal cord had resumed normal movement. Ramsay Hunt syndrome can be accompanied by involvement of other cranial nerves. Previous history of hypertensive crises and myocardial infarction may influence a diagnosis of Ramsay Hunt syndrome, especially when it has atypical presentation. However, this diagnosis should be considered in patients with unilateral multiple cranial nerve palsies.
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  • 文章类型: Case Reports
    孤立的下直肌麻痹是一种罕见的实体,甚至很少由解剖冲突引起。我们在这里报告了一例临床病例,该患者表现为孤立的下直肌肌麻痹,其特发性短突突出导致第三颅神经(CNIII)压迫脑池段。
    我们报告了一个病例,该病例与CNIII之间发生了解剖学冲突,表现为突出,高度不对称接近,并且神经直径不对称变细,偏离了同侧的直脑池轨迹。临床描述,文献综述,并进行了图像分析,包括使用来自扩散张量成像图像的融合图像进行CNIII纤维重建,稳定状态下的建设性干涉,以及在专用软件(BrainLABAG)上的T2流体衰减反演恢复图像。
    该案例说明了在CN缺陷的情况下解剖-临床相关性的重要性,并支持使用新的基于神经放射学的询问方法,例如CN扩散束造影来支持解剖CN冲突。
    UNASSIGNED: Isolated inferior rectus muscle palsy is a rare entity and even more rarely induced by an anatomical conflict. We report here a clinical case of third cranial nerve (CN III) compression in its cisternal segment by an idiopathic uncal protrusion in a patient presenting an isolated inferior rectus muscle palsy.
    UNASSIGNED: We report a case of an anatomical conflict between the uncus and the CN III in the form of a protrusion and highly asymmetrical proximity of the uncus and asymmetrically thinned nerve diameter deviated from its straight cisternal trajectory on the ipsilateral side were supported by an altered diffusion tractography along the concerned CN III. Clinical description, review of the literature, and image analysis were done including CN III fiber reconstruction using a fused image from diffusion tensor imaging images, constructive interference in steady state, and T2-fluid-attenuated inversion recovery images on a dedicated software (BrainLAB AG).
    UNASSIGNED: This case illustrates the importance of anatomical-clinical correlation in cases of CN deficits and supports the use of new neuroradiologically based interrogation methods such as CN diffusion tractography to support anatomical CN conflicts.
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  • 文章类型: Case Reports
    在高级别淋巴瘤中很少遇到神经淋巴瘤病。在这个系列中,我们回顾性分析了6例神经淋巴瘤病例,以寻找可能的危险因素,常见和不常见的演示文稿,和吸取的教训。在该系列中,神经性疼痛是单发或多神经根病最常见的症状。然而,氟脱氧葡萄糖正电子发射断层扫描-计算机断层扫描(FDGPET/CT)诊断的所有淋巴瘤浸润神经均无症状.腰部,臂丛神经,和三叉神经是最常见的部位,在FDGPET/CT上表现良好。大脑的磁共振成像(MRI)可以更好地描绘颅神经和脑膜受累。脑脊液流式细胞术正常,直到累及脑膜。FDGPET/CT增量评估神经外疾病部位,从而有助于确定活检部位和进一步的管理。我们得出的结论是,全身FDGPET/CT包括四肢和MRI大脑是评估晚期弥漫性大B细胞淋巴瘤中可疑神经淋巴瘤病的适当研究。
    Neurolymphomatosis is rarely encountered in high-grade lymphomas. In this case series, we retrospectively analyzed six neurolymphomatosis cases to look for possible risk factors, common and uncommon presentations, and the lessons learned. Neuropathic pain was the most common symptom with mono or polyradiculopathy in this series. However, all lymphomatous infiltrated nerves diagnosed on fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET/CT) were not symptomatic. The lumbar, brachial plexus, and trigeminal nerve were the most common sites and were depicted well on FDG PET/CT. Magnetic resonance imaging (MRI) of the brain better delineates cranial nerves and meningeal involvement. Cerebrospinal fluid flow cytometry was normal until meninges were involved. FDG PET/CT incrementally evaluated extra-neural disease sites, thus helping in deciding biopsy sites and further management. We concluded that a whole-body FDG PET/CT including limbs with MRI brain was the appropriate investigation for evaluating suspected neurolymphomatosis in advanced-stage diffuse large B-cell lymphoma.
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  • 文章类型: Journal Article
    抗GQ1bIgG抗体常伴有其他抗神经节苷脂抗体,诱发各种神经症状.我们在此报告了一名患有抗神经节苷脂抗体的患者,包括抗GQ1bIgG和抗GT1aIgG抗体,显示双侧眼肌麻痹,面神经麻痹,构音障碍,吞咽困难,两只手的感觉障碍,双侧动眼增强,绑架者,a(Gd)增强的T1加权脑磁共振成像(MRI)和面神经。他首先接受了静脉注射免疫球蛋白治疗,改善了眼肌麻痹,球麻痹,和手的感觉障碍,但是面神经麻痹恶化了,脑神经的Gd增强持续存在。大剂量甲基强的松龙治疗随后改善了面神经麻痹和颅神经的Gd增强。这是首例抗神经节苷脂抗体出现多个颅神经麻痹的病例,随后在MRI上追踪颅神经Gd增强的变化。
    The anti-GQ1b IgG antibody is often accompanied by other anti-ganglioside antibodies, which induces various neurological symptoms. We herein report a patient with anti-ganglioside antibodies, including anti-GQ1b IgG and anti-GT1a IgG antibodies, showing bilateral ophthalmoplegia, facial nerve palsies, dysarthria, dysphagia, dysesthesia in both hands, and enhancement of the bilateral oculomotor, abducens, and facial nerves on gadolinium (Gd)-enhanced T1-weighted brain magnetic resonance imaging (MRI). He was first treated with intravenous immunoglobulin, which improved ophthalmoplegia, bulbar palsies, and dysesthesia of hands, but the facial nerve palsies worsened, and Gd enhancement of the brain nerves persisted. High-dose methylprednisolone therapy subsequently improved the facial nerve palsies and Gd enhancement of the cranial nerves. This is the first case with anti-ganglioside antibodies presenting with multiple cranial nerve palsies that was followed to track the changes in the Gd enhancement of cranial nerves on MRI.
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  • 文章类型: Journal Article
    (1)背景:本研究旨在评估质子放疗(PRT)治疗2型神经纤维瘤相关神经鞘瘤病(NF2)患者前庭神经鞘瘤(VS)的疗效和治疗相关毒性。(2)方法:回顾性分析2004年至2016年连续使用PRT治疗的NF2患者,关注肿瘤体积,面部和三叉神经功能,听力,耳鸣,前庭症状,以及PRT后需要抢救治疗。(3)结果:纳入8例患者(中位年龄36岁,50%女性)。中位随访时间为71个月。五名(63%)患者接受了分段式PRT,三名(38%)患者接受了VS的PRT放射外科治疗。六名患者(75%)接受了VS手术;三名还接受了贝伐单抗。6例患者(75%)在PRT后不需要抢救治疗。两名患者(25%)的残余听力在PRT后丧失,在PRT之前,有6人已经失去了同侧听力。可以在6例患者中评估肿瘤和治疗相关的发病率。PRT之后,发生或恶化的病症是:面部轻瘫中有5人(83%),两例三叉神经感觉减退(33%),二耳鸣(33%),4例患者出现前庭症状(67%)。(4)结论:VSPRT后,队列中大多数NF2患者不需要额外治疗.肿瘤和/或治疗相关的颅神经缺陷很常见。这至少部分解释为使用PRT作为手术后的挽救治疗或贝伐单抗,在大多数情况下。还有进一步的机会来阐明质子放射疗法作为主要治疗的功效和毒性。
    (1) Background: This study aimed to evaluate the efficacy and treatment-related toxicity of proton radiotherapy (PRT) for vestibular schwannoma (VS) in patients with neurofibromatosis type 2-related schwannomatosis (NF2). (2) Methods: Consecutive NF2 patients treated with PRT for VS between 2004 and 2016 were retrospectively evaluated, focusing on tumor volume, facial and trigeminal nerve function, hearing, tinnitus, vestibular symptoms, and the need for salvage therapy after PRT. (3) Results: Eight patients were included (median age 36 years, 50% female). Median follow-up was 71 months. Five (63%) patients received fractionated PRT and three (38%) received PRT radiosurgery for VS. Six patients (75%) received prior VS surgery; three also received bevacizumab. Six patients (75%) did not require salvage therapy after PRT. Two patients (25%) with residual hearing lost it after PRT, and six had already lost ipsilateral hearing prior to PRT. Tumor and treatment-related morbidity could be evaluated in six patients. Following PRT, conditions that occurred or worsened were: facial paresis in five (83%), trigeminal hypoesthesia in two (33%), tinnitus in two (33%), and vestibular symptoms in four patients (67%). (4) Conclusion: After PRT for VS, the majority of the NF2 patients in the cohort did not require additional therapy. Tumor and/or treatment-related cranial nerve deficits were common. This is at least partly explained by the use of PRT as a salvage treatment after surgery or bevacizumab, in the majority of cases. There remains the further opportunity to elucidate the efficacy and toxicity of proton radiotherapy as a primary treatment.
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  • 文章类型: Case Reports
    本研究报告1例以多组脑神经损害为主要临床表现的犀牛-眼眶-脑真菌病(ROCM)患者,通过组织病理学和脑脊液宏基因组下一代测序(mNGS)技术证实。依靠MRI3D-SPACE技术,我们观察了患者脑神经损伤的位置和程度。结果提示,由黏液引起的真菌性脑膜脑炎可能沿颅神经会阴周围逆行进入颅骨。患者在口腔偏斜1.5天后入院,初步诊断为毛霉菌病感染。我们立即用静脉注射两性霉素B脂质体治疗患者。住院21天后,患者的临床症状没有明显改善。病人因经济困难及在家抗真菌治疗而出院,他的病情在6个月的随访中稳定下来.
    This study reported a case of a Rhino-Orbital-Cerebral Mycosis (ROCM) patient with multiple groups of cranial nerve damage as the primary clinical manifestation, confirmed by histopathology and cerebrospinal fluid metagenomic next-generation sequencing (mNGS) technology. Relying on the MRI3D-SPACE technology, we observed the location and extent of the cranial nerve damage in the patient. The results suggested that fungal meningoencephalitis caused by mucor may enter the skull retrograde along the cranial nerve perineurium. The patient was admitted to the hospital with a preliminary diagnosis of mucormycosis infection after 1.5 days of mouth deviation. We treated the patient immediately with intravenous amphotericin B liposomes. After 21 days of hospitalization, the clinical symptoms of the patient did not improve significantly. The patient was discharged due to financial difficulties and antifungal treatment at home, and his disease had stabilized at the 6-month follow-up.
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