Cranial nerve

颅神经
  • 文章类型: Journal Article
    这项研究的主要目的是回顾与颅底骨髓炎(SBO)相关的临床参数,次要目的是在治疗开始后1个月和6个月研究它们与患者预后的关系。
    这是一项单中心再观察性研究。
    该研究于2018年1月至2022年12月在吉隆坡马来亚大学医学中心进行。
    诊断为SBO的年龄超过15岁的患者被纳入研究。临床参数,调查,并记录随访记录.使用多变量分析在治疗开始后1个月和6个月分析疾病结果。
    该研究确定了31名SBO患者,其中大多数是患有糖尿病和高血压等合并症的老年男性。耳痛和耳漏是最常见的症状,和计算机断层扫描用于诊断。铜绿假单胞菌是最常见的病原菌,和静脉内广谱抗微生物药物用于治疗所有患者。25%的患者需要手术干预,和潜在的缺血性心脏病,贫血,单神经麻痹与不良预后显著相关。体重指数较高且C反应蛋白升高的患者在治疗1个月和6个月后表现出较差的预后。分别。
    早期识别,及时治疗,更好地控制合并症,营养,和监测可以改善SBO结果并减少并发症。因此,随着SBO患病率的增加,应制定诊断标准或管理指南以指导最佳临床实践.
    UNASSIGNED: The primary objective of this study is to review the clinical parameters associated with skull base osteomyelitis (SBO), with a secondary aim of studying their association with patient outcomes 1 and 6 months after treatment initiation.
    UNASSIGNED: This is a single-center restrospective observational study.
    UNASSIGNED: The study was conducted from January 2018 to December 2022 at the University Malaya Medical Center in Kuala Lumpur.
    UNASSIGNED: Patients aged over 15 years with a diagnosis of SBO were included in the study. Clinical parameters, investigations, and follow-up records were recorded. The disease outcomes were analyzed at 1 and 6 months after treatment initiation using multivariable analyses.
    UNASSIGNED: The study identified 31 patients with SBO, the majority of whom were elderly males with comorbidities such as diabetes and hypertension. Otalgia and otorrhea were the most common symptoms, and computed tomography scans were used for diagnosis. Pseudomonas aeruginosa was the most commonly identified pathogen, and intravenous broad-spectrum antimicrobials were used to treat all patients. Surgical intervention was required for 25% of patients, and underlying ischemic heart disease, anemia, and single nerve palsy were significantly associated with an unfavorable prognosis. Patients with higher body mass index and elevated C-reactive protein showed poorer outcomes after 1 and 6 months of treatment, respectively.
    UNASSIGNED: Early recognition, prompt treatment, better control of comorbidities, nutrition, and monitoring can improve SBO outcomes and reduce complications. Therefore, as the prevalence of SBO increases, diagnostic criteria or management guidelines should be established to guide the best clinical practice.
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  • 文章类型: Journal Article
    一些研究报告了外展神经麻痹的手术结果,但有关影响治疗成功的因素的信息仍然缺乏。这些因素对于制定治疗计划和提供疾病咨询至关重要。本研究旨在探讨斜视手术治疗外展神经麻痹的效果,并确定影响其成功的因素。包括相关文献的回顾。
    这项回顾性分析包括外展神经麻痹病例,专注于手术干预和相关患者数据,2012年4月1日至2022年4月30日在Phramongkutklao医院门诊就诊。相关文献综述包括手术成功率和影响手术结果的因素。
    这项研究招募了32名患者,包括19例部分外展神经麻痹和13例完全外展神经麻痹。斜视手术的总体成功率为78.1%。创伤是该人群中外展神经麻痹的主要原因(28.13%)。Fisher的精确和Mann-WhitneyU检验显示,在两组中,较短的外展神经麻痹发作和较小的术前角度与斜视手术的成功手术结果显着相关。相比之下,亚组分析显示,在水平斜视手术中,只有术前较小的角度与良好的手术结局显著相关.然而,垂直直肌转位组无显著因素。文献综述显示,外展神经麻痹的手术成功率为水平直肌手术的25%-82.6%,直肌移位的手术成功率为46.2%-91%。
    外展神经麻痹手术成功率达78.1%,部分外展神经麻痹占78.95%,完全性外展神经麻痹占76.92%。值得注意的是,手术前起病时间较短,术前角度较小,与术后1年的成功手术结局显著相关.
    已经进行了许多研究来确定眼肌手术治疗外展神经麻痹的有效性。然而,仍然缺乏有关可能影响治疗成功率的各种因素的信息。了解这些因素对于为患有这种疾病的个体制定适当的治疗计划和指导至关重要。我们的研究旨在检查斜视手术治疗外展神经麻痹的有效性,并确定影响其成功的因素。这是通过回顾相关文献并分析2012年4月1日至2022年4月30日Phramongkutklao医院的病例来实现的。我们纳入了32例部分或完全外展神经麻痹患者,创伤是导致我们组疾病的主要原因。结果显示,78.1%的病例手术成功。我们的分析显示,在麻痹开始后更早进行手术,并且在手术前具有较小的错位角度与更好的结果相关。然而,这些因素只对水平眼肌手术很重要,不是垂直肌肉移位.以前的研究报告这种情况的成功率从25%到91%不等。我们的研究得出结论,早期手术和某些术前因素可以改善眼肌手术后外展神经麻痹患者的预后。
    UNASSIGNED: Several studies reported surgical outcomes for abducens nerve palsy, but information on factors that affect treatment success remains lacking. These factors are crucial for developing a treatment plan and providing disease counseling. This study aimed to investigate the outcomes of strabismus surgery for abducens nerve palsy and determine the factors that influence its success, including a review of relevant literature.
    UNASSIGNED: This retrospective analysis included abducens nerve palsy cases, focusing on surgical interventions and relevant patient data, at the outpatient clinics of Phramongkutklao Hospital from April 1, 2012, to April 30, 2022. A relevant literature review included the surgical success rate and factors that influence surgical outcomes.
    UNASSIGNED: This study enrolled 32 patients, including 19 with partial and 13 with complete abducens nerve palsy. The overall success rate of strabismus surgery was 78.1%. Trauma was the leading cause of abducens nerve palsy in this population (28.13%). Fisher\'s exact and Mann-Whitney U-tests revealed that shorter abducens nerve palsy onset and smaller preoperative angle were significantly associated with successful surgical outcomes of strabismus surgery among the groups. In contrast, subgroup analysis revealed that only preoperative smaller angles were significantly associated with good surgical outcomes in horizontal strabismus surgery. However, the vertical rectus muscle transposition group demonstrated no significant factors. The literature review revealed that the success rate of surgery in abducens nerve palsy was 25%-82.6% for horizontal rectus muscle surgery and 46.2%-91% for rectus muscle transposition.
    UNASSIGNED: The surgical success rate for abducens nerve palsy reached 78.1%, including 78.95% for partial and 76.92% for complete abducens nerve palsy. Notably, a shorter onset preceding surgery and a smaller preoperative angle significantly correlated with successful surgical outcomes one year postoperatively.
    Numerous studies have been conducted to determine the effectiveness of eye muscle surgery for abducens nerve palsy. However, there is still a lack of information on the various factors that can influence the success rate of the treatment. Understanding these factors is crucial for developing appropriate treatment plans and guidance for individuals with this condition. Our study aimed to examine the effectiveness of strabismus surgery for abducens nerve palsy and identify the factors that affect its success. This was achieved by reviewing relevant literature and analyzing cases from Phramongkutklao Hospital between April 1, 2012, and April 30, 2022. We included 32 patients with partial or complete abducens nerve palsy, with trauma being the leading cause of the condition in our group. The results showed that the surgery was successful in 78.1% of cases. Our analysis revealed that getting surgery sooner after the palsy started and having a smaller misalignment angle before surgery were associated with better outcomes. However, these factors only mattered for horizontal eye muscle surgery, not vertical muscle transposition. Previous studies have reported success rates for this condition ranging from 25% to 91%. Our study concludes that earlier surgery and certain preoperative factors can improve outcomes for individuals with abducens nerve palsy after eye muscle surgery.
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  • 文章类型: Journal Article
    目的虽然经髁入路在技术上具有挑战性,它提供了大量的腹部和尾部暴露于颅骨交界处。这种方法需要在包括下颅神经在内的多个雄辩的神经血管结构周围导航,椎动脉及其分支,还有脑干.浅层暴露,包括切口位置和肌肉解剖,可以显着影响深度的手术角度和可操作性。方法我们在福尔马林防腐中逐步演示了经髁入路,注射乳胶的尸体头.在枕下肌的每一层内进行解剖。本文还包括具有说明性病例的小组。结果胸锁乳突肌(SCM)向前缩回;脾头炎,半壁肌炎,长肌与颈线分离,并向下反射。枕下肌组完全暴露。上斜肌和下斜肌与C1的横突断开。然后将上斜肌和直肌炎后主要肌切开下颈线。枕下肌群整体向下缩回。耳大神经与SCM横向缩回,枕大神经随着枕下肌群向下缩回。结论该技术避免了由肌皮肤入路引起的阻塞性肌肉体积,同时最大程度地增加了深度暴露。了解详细的肌肉解剖与插入位置和枕下神经的关系是完成和安全的颅外解剖的关键。勤奋的解剖有助于减少术后疼痛和肌肉痉挛,同时优化闭合技术。
    Objective  While the transcondylar approach is technically challenging, it provides generous ventral and caudal exposure to the craniovertebral junction. This approach requires navigation around multiple eloquent neurovascular structures including the lower cranial nerves, vertebral artery and its branches, and the brainstem. Superficial exposure, including incision location and muscle dissection, can dramatically affect the surgical angle and maneuverability at depth. Methods  We demonstrate the transcondylar approach in a step-by-step fashion in a formalin-embalmed, latex-injected cadaver head. Dissection within each layer of the suboccipital muscles was performed. A small cohort with an illustrative case is also included herein. Results  The sternocleidomastoid (SCM) muscle was retracted anteriorly; the splenium capitis, semispinalis capitis, and longissimus capitis muscles were disconnected from the superior nuchal line and reflected inferomedially. The suboccipital muscle group was fully exposed. The superior and inferior oblique muscles were disconnected from the transverse process of C1. The superior oblique and the rectus capitis posterior major muscles were then dissected off the inferior nuchal line, and the suboccipital muscle group was retracted inferomedially en bloc . The greater auricular nerve was retracted laterally with the SCM, and the greater occipital nerve was retracted inferomedially with the suboccipital muscle group. Conclusion  This technique avoids the obstructive muscle bulk that results from a myocutaneous approach while maximizing deep exposure. Understanding the detailed muscular anatomical relationship with the insertion location and suboccipital nerves is key to complete and safe extracranial dissection. Diligent dissection helps minimize postoperative pain and muscle spasm while optimizing the closure technique.
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  • 文章类型: Case Reports
    原发性颅神经淋巴瘤病(PCNL)是原发性CNS淋巴瘤(PCNSL)的一种罕见亚型,其中浸润性淋巴瘤受累仅限于颅神经。这里,我们报告了一例成功进行基因组分析的PCNL病例.一名57岁的男性经历了大约30个月的漫长的诊断前阶段,以由类固醇管理的多次颅神经病发作为特征。在诊断的时候,患者患有右侧颅神经病变,涉及颅神经(CN)V,VI,和七。右侧海绵状病变活检病理结果与大B细胞淋巴瘤浸润神经纤维一致。临床过程是积极的和难治性的,其特征是随着颈脊髓神经淋巴瘤病的发展而不断发展,脑脊液受累,室管膜和脑实质内受累,尽管有多种治疗方法,包括化学免疫疗法,布鲁顿酪氨酸激酶抑制剂,辐射,自体干细胞移植,嵌合抗原受体T细胞疗法(CAR-T),和全脑辐射。患者从最初诊断时和第一次颅神经病变发作后52个月存活了22个月。下一代测序确定的突变(MYD88,CD79b,和PIM1)在PCNSL中经常观察到。不寻常的发现包括涉及PIM1的总共22个突变,表明高度活跃的异常体细胞超突变和两个错义CXCR4突变。CXCR4突变从未在PCNSL中描述过,可能对疾病生物学和治疗干预有影响。我们提供了文献综述以进一步阐明PCNL。
    Primary cranial neurolymphomatosis (PCNL) is a rare subtype of primary CNS lymphoma (PCNSL) in which infiltrative lymphomatous involvement is confined to cranial nerves. Here, we report a case of PCNL with successful genomic profiling. A 57-year-old male had a lengthy prediagnostic phase spanning approximately 30 months, characterized by multiple episodes of cranial neuropathies managed by steroids. At the time of diagnosis, the patient had right-sided cranial neuropathies involving cranial nerves (CN) V, VI, and VII. Pathological findings of the right cavernous lesion biopsy were consistent with large B-cell lymphoma-infiltrating nerve fibers. The clinical course was aggressive and refractory, characterized by relentless progression with the development of cervical spinal neurolymphomatosis, cerebrospinal fluid involvement, and ependymal and intraparenchymal cerebral involvement, despite multiple lines of therapy, including chemoimmunotherapy, Bruton\'s tyrosine kinase inhibitor, radiation, autologous stem cell transplant, chimeric antigen receptor T-cell therapy (CAR-T), and whole-brain radiation. The patient survived for 22 months from the time of the initial diagnosis and 52 months after the first episode of cranial neuropathy. Next-generation sequencing identified mutations (MYD88, CD79b, and PIM1) that are frequently observed in PCNSL. The unusual findings included a total of 22 mutations involving PIM1, indicating a highly active aberrant somatic hypermutation and two missense CXCR4 mutations. CXCR4 mutations have never been described in PCNSL and may have implications for disease biology and therapeutic interventions. We provide a literature review to further elucidate PCNL.
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  • 文章类型: Journal Article
    海绵窦(CS)是一个要求苛刻的手术区域,鉴于其位置较深和多个神经血管结构的参与。反复讨论最佳手术入路,最近有人提出内窥镜经眶入路作为选择的外侧CS病变的可行途径。尽管如此,为了使这种技术安全地发展和巩固,对受累颅神经的全面解剖描述,硬脑膜韧带,需要动脉关系。
    CS的详细解剖描述,第三课程,IV,VI,和V脑神经,和颈动脉的C3-C7段,全部从腹外侧内窥镜经眶角度进行描述。
    解剖了五个防腐的人类尸体头(10面)。内窥镜经眶入路切除眶外侧缘,前路临床切除术,并进行了岩石切除术。上颅神经的过程是从它们明显起源于脑干开始的,穿过中窝或海绵窦,直到他们进入轨道。神经导航用于跟踪神经的进程并测量其手术暴露的长度。
    经眶入路使我们能够可视化CS的侧壁,颅神经III,IV,V1-3和VI。前路临床切除术和额硬脑膜和动眼三角的开放显示了III神经的完整进程,平均长度为37(±2)mm。打开三叉神经孔并切割允许跟随IV神经从其围绕脑梗的过程一直到轨道的肌腱,平均54(±4)mm。打开滑车下三角形,在海绵内和Gruber韧带下显示VI神经,扩展的岩石切除术使我们看到了它的脑池部分(27±6毫米)。三叉神经根完全可见,其三个分支也是如此(46±2、34±3和31±1mm,分别)。
    解决CS时需要全面的解剖知识和广泛的外科专业知识。经眶走廊暴露了大部分脑池和受累的颅神经的完整海绵状过程。这篇解剖学文章有助于理解神经的关系,血管,和CS方法中涉及的硬脑膜结构,对于最终完成经眶手术的学习过程至关重要。
    UNASSIGNED: The cavernous sinus (CS) is a demanding surgical territory, given its deep location and the involvement of multiple neurovascular structures. Subjected to recurrent discussion on the optimal surgical access, the endoscopic transorbital approach has been recently proposed as a feasible route for selected lesions in the lateral CS. Still, for this technique to safely evolve and consolidate, a comprehensive anatomical description of involved cranial nerves, dural ligaments, and arterial relations is needed.
    UNASSIGNED: Detailed anatomical description of the CS, the course of III, IV, VI, and V cranial nerves, and C3-C7 segments of the carotid artery, all described from the ventrolateral endoscopic transorbital perspective.
    UNASSIGNED: Five embalmed human cadaveric heads (10 sides) were dissected. An endoscopic transorbital approach with lateral orbital rim removal, anterior clinoidectomy, and petrosectomy was performed. The course of the upper cranial nerves was followed from their apparent origin in the brainstem, through the middle fossa or cavernous sinus, and up to their entrance to the orbit. Neuronavigation was used to follow the course of the nerves and to measure their length of surgical exposure.
    UNASSIGNED: The transorbital approach allowed us to visualize the lateral wall of the CS, with cranial nerves III, IV, V1-3, and VI. Anterior clinoidectomy and opening of the frontal dura and the oculomotor triangle revealed the complete course of the III nerve, an average of 37 (±2) mm in length. Opening the trigeminal pore and cutting the tentorium permitted to follow the IV nerve from its course around the cerebral peduncle up to the orbit, an average of 54 (±4) mm. Opening the infratrochlear triangle revealed the VI nerve intracavernously and under Gruber\'s ligament, and the extended petrosectomy allowed us to see its cisternal portion (27 ± 6 mm). The trigeminal root was completely visible and so were its three branches (46 ± 2, 34 ± 3, and 31 ± 1 mm, respectively).
    UNASSIGNED: Comprehensive anatomic knowledge and extensive surgical expertise are required when addressing the CS. The transorbital corridor exposes most of the cisternal and the complete cavernous course of involved cranial nerves. This anatomical article helps understanding relations of neural, vascular, and dural structures involved in the CS approach, essential to culminating the learning process of transorbital surgery.
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  • 文章类型: Case Reports
    先前已经描述了颅神经或其分支之间的通信。其中一些神经通信的确切功能意义仍有待充分理解。本文报道了颞下窝内的耳颞神经和下牙槽神经之间的独特交流。组织学检查表明从下牙槽神经到耳颞部神经的顺行连接,这可能与从一根神经的解剖区域到另一根神经的转介疼痛有关。
    Communications between cranial nerves or their branches have been described previously. The exact functional significance of some of these neural communications remains to be fully understood. This paper reports a unique communication between the auriculotemporal and inferior alveolar nerves within the infratemporal fossa. The histological examination indicates an antegrade connection from the inferior alveolar nerve to the auriculotemporal nerve, which could potentially be implicated in referred pain from the anatomical territory of one nerve to the other.
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  • 文章类型: Journal Article
    目的:本研究的目的是探讨三维(3D)高分辨率反转恢复(IR)制备的快速破坏梯度回忆(SPGR)磁共振成像(MRI)在颅神经脑膜癌(MC)诊断中的临床应用。
    方法:纳入2015年1月至2020年3月的114例MC患者,对其MRI进行回顾性分析。所有患者在接受造影剂之前都进行了MRI检查。在施用造影剂后,测量了2D常规MRI序列和3DIR制备的快速SPGR高分辨率T1加权(BRAVO)扫描序列。然后检查MC的特征和受累的颅神经。
    结果:在114例MC患者中,81例(71.05%)在对比增强3D-BRAVO成像上有颅神经增强,只有41例(35.96%)在常规MRI上有图像增强。对比增强的3D-BRAVO显示出比常规MRI更强的颅神经图像对比度增强(P<0.001)。此外,面神经和听觉神经的检测率,三叉神经,动眼神经,舌下神经,视神经,舌咽/迷走神经/副神经,对比增强3D-BRAVO成像显示外展神经占58.77%,47.37%,9.65%,8.77%,5.26%,3.51%,和0.88%,分别。我们发现受影响的面部和听觉神经之间存在统计学上的显着差异,以及三叉神经,动眼神经,舌下神经,和视神经.
    结论:在MC中,对比增强3D-BRAVO成像比2D常规增强MRI更有效地显示颅神经。面部,听觉,三叉神经是涉及MC的主要神经,和改善这些神经的扫描将有助于早期发现和治疗MC。
    The purpose of this study was to investigate the clinical utility of three-dimension (3D) high-resolution inversion recovery (IR)-prepared fast spoiled gradient-recalled (SPGR) magnetic resonance imaging (MRI) in the diagnosis of cranial nerve meningeal carcinomatosis (MC).
    A total of 114 patients with MC from January 2015 to March 2020 were enrolled and their MRIs were analyzed retrospectively. All patients underwent MRIs before being administered a contrast agent. Both a 2D conventional MRI sequence and a 3D IR-prepared fast SPGR high-resolution T1-weighted (BRAVO) scan sequence were measured after contrast agent administration. The characteristics of MC and the involved cranial nerves were then examined.
    Among the 114 MC patients, 81 (71.05%) had cranial nerve enhancement on contrast-enhanced 3D-BRAVO imaging, while only 41 (35.96%) had image enhancement on conventional MRI. The contrast-enhanced 3D-BRAVO displayed stronger image contrast enhancement of the cranial nerves than the conventional MRI (P < 0.001). Furthermore, detection rates for the facial and auditory nerves, trigeminal nerve, oculomotor nerve, sublingual nerve, optic nerve, glossopharyngeal/vagal/accessory nerve, and abductor nerve on contrast-enhanced 3D-BRAVO imaging were 58.77%, 47.37%, 9.65%, 8.77%, 5.26%, 3.51%, and 0.88%, respectively. We found a statistically significant difference between the affected facial and auditory nerves, as well as the trigeminal nerve, oculomotor nerve, sublingual nerve, and optic nerve.
    In MC, contrast-enhanced 3D-BRAVO imaging displayed the cranial nerves more effectively than 2D conventional enhanced MRI. The facial, auditory, and trigeminal nerves are the primary nerves involved in MC, and improved scanning of these nerves would aid in the early detection and treatment of MC.
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  • 文章类型: Editorial
    暂无摘要。
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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
    最常见的神经变性蛋白质病包括在脑中具有错误折叠的tau或α-突触核蛋白沉积的疾病。PNS中的病理蛋白聚集体在α-突触核蛋白病中得到了广泛认可,并且最近作为诊断生物标志物引起了关注。然而,Tau蛋白病的观察很少。为了表征PNS在tau蛋白病变中的参与,我们调查了54例tau病变[进行性核上性麻痹(PSP),n=15;阿尔茨海默病(AD),n=18;慢性创伤性脑病(CTE),n=5;和皮质基底变性(CBD),n=6;皮克病,n=9;边缘占优势的神经元包涵体4-重复tau蛋白病(LNT),n=1]使用免疫组织化学,Gallyas银染,生物化学,和接种试验。大多数PSP病例显示PNS中磷酸化和4次重复tau免疫反应性tau沉积如下:(tau阳性病例数/可用病例数)颅神经III:7/8(88%);IX/X:10/11(91%);和XII:6/6(100%);脊髓前根:10/10(100%)。PSP中的tau阳性内含物通常在轴突中显示出具有原纤维(神经原纤维缠结样)形态的结构,这些结构也被Gallyas银染色识别。CBD病例很少显示细颗粒状非嗜银tau沉积物。相比之下,PNS中的tau病理在AD中不明显,CTE和Pick病病例。单个LNT病例在PNS中也显示tau病理。在PSP中,PNS-tau受累的严重程度与相应细胞核的严重程度相关,虽然,偶尔,p-tau沉积物存在于颅神经中,但不存在于相关的脑干核中。毫不奇怪,大多数PSP病例表现为眼球运动障碍和延髓症状,一些病例还显示了运动神经元的下位征。使用tau生物传感器细胞,我们首次证明了tau在PNS中的播种能力。总之,突出的PNS-tau将PSP与其他tau蛋白病区分开来。PSP和CBD之间PNS-tau的形态学差异表明PNS中的tau病理可以反映中枢神经系统中的tau病理。PSP中tau病变的高频率和早期存在表明PNS-tau可能具有临床和生物标志物相关性。
    The most frequent neurodegenerative proteinopathies include diseases with deposition of misfolded tau or α-synuclein in the brain. Pathological protein aggregates in the PNS are well-recognized in α-synucleinopathies and have recently attracted attention as a diagnostic biomarker. However, there is a paucity of observations in tauopathies. To characterize the involvement of the PNS in tauopathies, we investigated tau pathology in cranial and spinal nerves (PNS-tau) in 54 tauopathy cases [progressive supranuclear palsy (PSP), n = 15; Alzheimer\'s disease (AD), n = 18; chronic traumatic encephalopathy (CTE), n = 5; and corticobasal degeneration (CBD), n = 6; Pick\'s disease, n = 9; limbic-predominant neuronal inclusion body 4-repeat tauopathy (LNT), n = 1] using immunohistochemistry, Gallyas silver staining, biochemistry, and seeding assays. Most PSP cases revealed phosphorylated and 4-repeat tau immunoreactive tau deposits in the PNS as follows: (number of tau-positive cases/available cases) cranial nerves III: 7/8 (88%); IX/X: 10/11 (91%); and XII: 6/6 (100%); anterior spinal roots: 10/10 (100%). The tau-positive inclusions in PSP often showed structures with fibrillary (neurofibrillary tangle-like) morphology in the axon that were also recognized with Gallyas silver staining. CBD cases rarely showed fine granular non-argyrophilic tau deposits. In contrast, tau pathology in the PNS was not evident in AD, CTE and Pick\'s disease cases. The single LNT case also showed tau pathology in the PNS. In PSP, the severity of PNS-tau involvement correlated with that of the corresponding nuclei, although, occasionally, p-tau deposits were present in the cranial nerves but not in the related brainstem nuclei. Not surprisingly, most of the PSP cases presented with eye movement disorder and bulbar symptoms, and some cases also showed lower-motor neuron signs. Using tau biosensor cells, for the first time we demonstrated seeding capacity of tau in the PNS. In conclusion, prominent PNS-tau distinguishes PSP from other tauopathies. The morphological differences of PNS-tau between PSP and CBD suggest that the tau pathology in PNS could reflect that in the central nervous system. The high frequency and early presence of tau lesions in PSP suggest that PNS-tau may have clinical and biomarker relevance.
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