CNS = central nervous system

  • 文章类型: Case Reports
    背景:中枢神经系统(CNS)结核瘤是结核病(TB)感染的可怕并发症。这些病变可以以不同的方式出现,并与显著的发病率和死亡率相关。在这些患者的护理中,及时诊断和治疗病变和潜在的感染至关重要。作者介绍了一例45岁的也门移民,有3个月的严重右颞枕头痛伴畏光和盗汗的病史。影像学显示右小脑半球有边缘增强病变。
    方法:实验室检查无异常且在正常范围内。Quantiferon检测结果为阴性,排除潜在的结核病感染。病人接受了枕下开颅手术,切除小脑病变显示干酪样肉芽肿形成,抗酸杆菌和Fite染色呈阳性。
    结论:中枢神经系统结核瘤是有原发性结核病史的患者需要考虑的重要差异,无论活动性疾病或免疫能力状态。切除这些病变仍然是安全有效的可行治疗选择。
    BACKGROUND: Central nervous system (CNS) tuberculomas are a feared complication of tuberculosis (TB) infection. These lesions can present in varying manners and are associated with significant morbidity and mortality. Prompt diagnosis and treatment of the lesion and the underlying infection are critical in the care of these patients. The authors presented a case of a 45-year-old Yemeni immigrant presenting with a 3-month history of severe right temporo-occipital headaches with photophobia and night sweats. Imaging showed a rim-enhancing lesion in the right cerebellar hemisphere.
    METHODS: Laboratory tests were unremarkable and within normal limits. QuantiFERON testing was negative, ruling out latent TB infection. The patient received a suboccipital craniotomy, and resection of the cerebellar lesion showed caseating granuloma formation, which was positive for acid-fast bacilli and Fite stain.
    CONCLUSIONS: CNS tuberculomas are an important differential to consider in patients with a history of primary TB, regardless of active disease or immunocompetent status. Resection of these lesions remains a viable treatment option that is safe and effective.
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  • 文章类型: Case Reports
    背景:原发性脊柱黑色素瘤极为罕见,占所有原发性黑色素瘤的1%。通常阴险地出现在胸脊髓中,原发性脊髓黑素瘤可因出血倾向而急性表现.
    方法:尽管它很少,当在磁共振成像中看到T1和T2强度的出血模式时,应将原发性脊柱黑色素瘤包括在鉴别诊断中。此外,完整的诊断至关重要,因为原发性脊柱黑色素瘤的预后比具有转移性扩散的原发性皮肤黑色素瘤的预后更有利。
    结论:切除是首选治疗方法,一些作者主张术后化疗,免疫疗法,和/或辐射。我们描述了一例出血性原发性脊柱黑色素瘤引起的急性四肢瘫痪,需要切除。
    BACKGROUND: Primary spinal melanoma is extremely rare, accounting for ∼1% of all primary melanomas. Typically presenting insidiously in the thoracic spinal cord, primary spinal melanomas can have an acute presentation due to their propensity to hemorrhage.
    METHODS: Despite its rarity, primary spinal melanoma should be included in the differential diagnosis when a hemorrhagic pattern of T1 and T2 intensities is seen on magnetic resonance imaging. Furthermore, the complete diagnosis is crucial because the prognosis of a primary spinal melanoma is considerably more favorable than that of a primary cutaneous melanoma with metastatic spread.
    CONCLUSIONS: Resection is the treatment of choice, with some authors advocating for postoperative chemotherapy, immunotherapy, and/or radiation. We describe a case of acute quadriplegia from hemorrhagic primary spinal melanoma requiring resection.
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  • 文章类型: Case Reports
    背景:原发性脑膜黑素细胞肿瘤是非常罕见的肿瘤,仅占所有原发性脑肿瘤的0.06%至0.1%,范围从良性局部肿瘤到高度侵袭性恶性病变。这些肿瘤的诊断往往是具有挑战性的从临床,放射学,和病理观点。同样具有挑战性的是原发性脑膜黑素细胞肿瘤和转移性黑色素瘤之间的区别。
    方法:作者报道了一例41岁男性,其影像学表现诊断为2型神经纤维瘤病:双侧内耳道病变(最符合双侧前庭神经鞘瘤),两个基于硬脑膜的病变推测为脑膜瘤,与周围神经鞘瘤一致的多发性脊柱病变,和一个与室管膜瘤一致的脊髓髓内病变。这些病变的活检显示黑素细胞肿瘤具有轻度至中度的异型性和轻度升高的增殖指数。这使得区分良性和恶性具有挑战性。此外,这些肿瘤的播散性使得很难确定它们是由脑膜引起还是由隐匿性原发性黑色素瘤引起的转移。
    结论:该病例说明了脑膜黑素细胞肿瘤的诊断所面临的挑战,并强调了将临床和影像学发现与组织学外观和分子研究相结合的重要性。
    BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma.
    METHODS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma.
    CONCLUSIONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.
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  • 文章类型: Case Reports
    背景:原发性脊髓髓内肿瘤导致显著的发病率和死亡。在这些患者中,尚未对术中超声作为定位和监测切除程度的辅助手段进行系统评估;术中超声造影(CEUS)的有效性几乎尚未完全探索。
    方法:确定了一个回顾性病例系列患者,这些患者在单一机构中同意术中超声造影的超说明书使用。7例平均年龄为52.8±15.8岁的患者接受了由一名主治医生在CEUS辅助下进行的髓内肿瘤切除术。组织病理学评估显示3例血管母细胞瘤,毛细胞星形细胞瘤1例,室管膜瘤2例,室管膜下瘤1例。术前磁共振成像对比增强与钆增强相关。术中超声造影有助于精确的病灶定位和骨髓切开术计划。动态CEUS研究可用于证明具有优势血管蒂的病变的血液供应。不管造影剂摄取如何,脊髓组织和肿瘤之间的差异增强有助于确定界面边界。
    结论:术中超声造影是对比增强髓内肿瘤术中勾画和体内确认全切除的有用辅助手段。需要进行系统的研究以确定CEUS在切除各种病理的髓内脊柱肿瘤中的作用。
    BACKGROUND: Primary intramedullary spinal tumors cause significant morbidity and death. Intraoperative ultrasound as an adjunct for localization and monitoring the extent of resection has not been systematically evaluated in these patients; the effectiveness of intraoperative contrast-enhanced ultrasound (CEUS) remains almost completely unexplored.
    METHODS: A retrospective case series of patients at a single institution who had consented to the off-label use of intraoperative CEUS was identified. Seven patients with a mean age of 52.8 ± 15.8 years underwent resection of intramedullary tumors assisted by CEUS performed by a single attending neurosurgeon. Histopathological evaluation revealed 3 cases of hemangioblastoma, 1 case of pilocytic astrocytoma, 2 cases of ependymoma, and 1 case of subependymoma. Contrast enhancement correlated with gadolinium enhancement on preoperative magnetic resonance imaging. Intraoperative CEUS facilitated precise lesion localization and myelotomy planning. Dynamic CEUS studies were useful in demonstrating the blood supply to lesions with a dominant vascular pedicle. Regardless of contrast uptake, the differential enhancement between spinal cord tissue and neoplasm assisted in determining interface boundaries.
    CONCLUSIONS: Intraoperative CEUS constitutes a useful adjunct for the intraoperative delineation of contrast-enhancing intramedullary tumors and in vivo confirmation of gross-total resection. Systematic investigation is needed to establish the role of CEUS for resection of intramedullary spinal tumors of various pathologies.
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  • 文章类型: Case Reports
    背景:世界卫生组织中枢神经系统肿瘤分类修订版第四版于2016年出版。基于这种分类,不鼓励一种名为“少星形细胞瘤/间变性少星形细胞瘤”的浸润性神经胶质瘤实体。建议在分析这些混合性神经胶质瘤的遗传改变时,应将其分类为弥漫性星形细胞瘤/间变性星形细胞瘤或少突胶质细胞瘤/间变性少突胶质细胞瘤。
    方法:一名78岁的女性因交通事故接受了脑部计算机断层扫描(CT)检查。头颅CT显示左颞顶叶有脑肿瘤;因此,她住院了.她做了清醒开颅手术。手术后,她只接受局部放疗;作者不能开出替莫唑胺,因为她患有左乙拉西坦诱导的全血细胞减少症.剩下的肿瘤在神经放射学上消失了,手术后40个月,她还活着,没有肿瘤复发。
    结论:组织病理学,该肿瘤被诊断为间变性少突星形细胞瘤,具有明显的星形细胞瘤和少突胶质细胞瘤双重表型.基因上,这两种成分揭示了星形细胞瘤和少突胶质细胞瘤的基因型,分别。因此,作者认为颞部肿瘤的综合诊断为具有双基因型的真正的间变性少粒星形细胞瘤.有趣的是,该病例还包括一个由纺锤体至卵圆形肿瘤细胞组成的区域,该区域揭示了星形细胞瘤和少突胶质细胞瘤之间的中间遗传改变。
    BACKGROUND: The revised fourth edition of the World Health Organization classification of central nervous system tumors was published in 2016. Based on this classification, one of the infiltrating glioma entities named \"oligoastrocytoma/anaplastic oligoastrocytoma\" is discouraged. It is proposed that these mixed gliomas should be classified as diffuse astrocytoma/anaplastic astrocytoma or oligodendroglioma/anaplastic oligodendroglioma when analyzing their genetic alteration.
    METHODS: A 78-year-old female underwent brain computed tomography (CT) because of a traffic accident. Cranial CT revealed a brain tumor in the left temporoparietal lobe; therefore, she was hospitalized. She underwent awake craniotomy. After the operation, she was treated with only local radiotherapy; the authors could not prescribe temozolomide, because she had had levetiracetam-induced pancytopenia. The remaining tumor neuroradiologically disappeared, and she was alive 40 months after the operation without tumor recurrence.
    CONCLUSIONS: Histopathologically, this tumor was diagnosed as an anaplastic oligoastrocytoma with a distinct dual phenotype of astrocytoma and oligodendroglioma components. Genetically, these two components revealed astrocytoma and oligodendroglioma genotypes, respectively. Therefore, the authors considered the integrated diagnosis of the temporal tumor as a true anaplastic oligoastrocytoma with a dual genotype. Interestingly, this case also included an area composed of spindle to oval neoplastic cells that revealed intermediate genetic alterations between astrocytomas and oligodendrogliomas.
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  • 文章类型: Case Reports
    背景:疱疹是年轻人群病毒性脑炎的最常见原因。脑部手术后的疱疹性脑膜炎非常罕见,however.文献中只有少数病例报道,只有一个涉及前庭神经鞘瘤手术后的感染。
    方法:作者报告了一例44岁的患者,该患者在切除大型囊性前庭神经鞘瘤后几天出现了严重的疱疹性脑膜炎。
    结论:当患者在手术后几天出现不典型症状时,必须考虑后颅窝手术后的单纯疱疹病毒性脑膜炎。
    BACKGROUND: Herpes is the most common cause of viral encephalitis in the young population. Herpes meningitis following brain surgery is very rare, however. Only a few cases are reported in the literature, and only one concerned an infection after vestibular schwannoma surgery.
    METHODS: The authors report a case of a 44-year-old patient who developed severe herpes meningitis a few days after removal of a large cystic vestibular schwannoma.
    CONCLUSIONS: Herpes simplex virus meningitis following a posterior fossa surgery must be considered when patients develop atypical symptoms a few days after surgery.
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  • 文章类型: Case Reports
    背景:多发性骨髓瘤(MM)有扩散到椎体的倾向;然而,累及中枢神经系统的髓外浆细胞瘤极为罕见。
    方法:作者报告了一名先前存在MM的患者的腰椎区域的髓外浆细胞瘤。他们提出了一名50岁的女性,患有严重的背部和神经根疼痛以及进行性神经功能缺损。
    结论:MM通常采用化疗和放疗治疗。然而,在这个独特的案例中,切除被证明是治疗的关键部分。
    BACKGROUND: Multiple myeloma (MM) has the propensity to spread to vertebral bodies; however, extramedullary plasmacytomas involving the central nervous system are extremely rare.
    METHODS: The authors report the first intradural extramedullary plasmacytoma in the lumbar region of the spine in a patient with preexisting MM. They present a 50-year-old female with severe back and radicular pain and progressive neurological deficit.
    CONCLUSIONS: MM is typically treated with chemotherapy and radiation therapy. However, in this unique case, resection proved to be a key part of treatment.
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  • 文章类型: Case Reports
    背景:同时发生的颅内和睾丸生殖细胞肿瘤(GCT)极为罕见,导致他们缺乏足够的治疗经验。因此,作者报告了一例这种GCT。
    方法:一名5岁男孩因头痛和呕吐入院。计算机断层扫描和磁共振成像表明松果体区域存在GCT的可能性。血清肿瘤标志物甲胎蛋白(AFP)为5,396.1μg/L,β-人绒毛膜促性腺激素水平在正常范围内。随后,肿瘤被切除了,最终病理结果为混合GCT。因此,增加了化疗和放疗。然而,作者同时在超声检查中发现了一个睾丸肿瘤,手术后病理提示成熟的囊性畸胎瘤。治疗后,病人恢复得很好,AFP水平下降到正常值。
    结论:据作者所知,本报告是第4例同时发生颅内和睾丸GCT,也是第1例同时发生松果体区和睾丸成熟畸胎瘤的混合GCT.手术的组合,化疗,松果体区混合GCT的放射治疗和睾丸生殖细胞肿瘤的手术切除对这些患者是有效的,但需要长期监测。
    BACKGROUND: Simultaneous intracranial and testicular germ cell tumors (GCTs) are extremely rare, leading to a lack of adequate experience in their treatment. Therefore, the authors report a case of this kind of GCT.
    METHODS: A 5-year-old boy was admitted to the hospital with headache and vomiting. Computed tomography and magnetic resonance imaging suggested the possibility of a GCT in the pineal region. The value of the serum tumor marker alpha-fetoprotein (AFP) was 5,396.1 μg/L, and β-human chorionic gonadotropin levels were within the normal range. Subsequently, the tumor was removed, and the final pathological result was a mixed GCT. Therefore, chemotherapy and radiation were added. However, the authors found a testicular tumor on ultrasound at the same time, and pathology after surgery suggested a mature cystic teratoma. Following treatment, the patient recovered well, and AFP levels dropped to normal values.
    CONCLUSIONS: To the authors\' knowledge, this report is the fourth case of simultaneous intracranial and testicular GCTs and the first case of a simultaneous mixed GCT in the pineal region and mature teratoma of the testis. A combination of surgery, chemotherapy, and radiation therapy for mixed GCTs in the pineal region and surgical excision for testicular reproductive cell tumors are effective in these patients, but long-term monitoring is required.
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  • 文章类型: Case Reports
    背景:中枢神经系统的浅表铁质沉着症是一种罕见的综合征,由于慢性,反复出血进入蛛网膜下腔.
    方法:作者介绍了一例14岁女孩的浅表铁质沉着症。它是髓母细胞瘤切除术后的晚期术后并发症。尽管病人没有症状,监视成像显示,在梯度回波(GRE)序列上,小脑叶和颅神经VII和VIII的脑池段内弥漫性含铁血黄素沉积。正式的听力测试显示,双侧高频音调识别丧失与早期感觉神经性听力丧失一致。由多个硬脑膜缺损引起的假性脑膜膨出被确定为可能的原因。并进行了明确的手术修复。术中,血的脑脊液的存在证实了浅表铁质沉着症的诊断。
    结论:该病例强调了在小儿后颅窝肿瘤切除术后的术后监测成像中常规包括GRE或敏感性加权序列的潜在需要。
    BACKGROUND: Superficial siderosis of the central nervous system is a rare syndrome notable for the presence of hemosiderin deposition due to chronic, repetitive hemorrhages into the subarachnoid space.
    METHODS: The authors presented a case of superficial siderosis in a 14-year-old girl. It arose as a late postoperative complication after resection of a medulloblastoma. Despite the patient being asymptomatic, surveillance imaging demonstrated diffuse hemosiderin deposition within the cerebellar folia and cisternal segments of cranial nerves VII and VIII on gradient echo (GRE) sequences. Formal audiometric testing demonstrated bilateral loss of high-frequency tone recognition consistent with early sensorineural hearing loss. A pseudomeningocele due to multiple dural defects was identified as the likely cause, and definitive surgical repair was performed. Intraoperatively, the presence of blood-tinged cerebrospinal fluid confirmed a diagnosis of superficial siderosis.
    CONCLUSIONS: This case highlighted the potential need to routinely include GRE or susceptibility-weighted sequences in postoperative surveillance imaging after resection of pediatric posterior fossa tumors.
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  • 文章类型: Case Reports
    背景:肺泡包虫病是一种罕见的疾病,但是在农村环境中生活或工作是一个重大的风险因素。肝脏是主要受影响的器官,在大约25%的病例中有额外的肝外表现。原发性肝外疾病很少见,孤立的大脑受累是极不寻常的。
    方法:作者描述了一个有免疫能力的农民中孤立的脑肺泡包虫病的例证性病例。大脑的磁共振成像显示主要是囊性病变,伴有病灶周围水肿和左额叶出现“葡萄串”。组织学显示,纤维囊壁的明显分界碎片伴有明显的炎症和坏死。较高的放大倍数显示残留有钩状体和钙化小体。免疫组织化学和聚合酶链反应(PCR)分析证实了脑泡型包虫病的诊断。有趣的是,血清学和胸部和腹部计算机断层扫描结果均为阴性,指示孤立的原发性肝外表现。
    结论:孤立,原发性中枢神经系统包虫病极为罕见,只有孤立的病例报告。就像作者的情况一样,它可以发生在有免疫能力的患者中,尤其是有农村职业史的人.血清学结果阴性不排除脑包虫病,这需要组织学确认。免疫组织化学染色和PCR分析在没有经典形态学发现的情况下特别有用。
    BACKGROUND: Alveolar echinococcosis is a rare condition, but living or working in a rural environment is a substantial risk factor. The liver is the organ primarily affected, with additional extrahepatic manifestations in approximately 25% of cases. Primary extrahepatic disease is rare, and isolated cerebral involvement is extremely unusual.
    METHODS: The authors described an illustrative case of isolated cerebral alveolar echinococcosis in an immunocompetent farmer. Magnetic resonance imaging of the brain showed a predominantly cystic lesion with perifocal edema and a \"bunch of grapes\" appearance in the left frontal lobe. Histology revealed sharply demarcated fragments of a fibrous cyst wall accompanied by marked inflammation and necrosis. Higher magnification showed remnants of protoscolices with hooklets and calcified corpuscles. Immunohistochemistry and polymerase chain reaction (PCR) analysis confirmed the diagnosis of cerebral alveolar echinococcosis. Interestingly, serology and thoracic and abdominal computed tomography results were negative, indicative of an isolated primary extrahepatic manifestation.
    CONCLUSIONS: Isolated, primary central nervous system echinococcosis is extremely rare, with only isolated case reports. As in the authors\' case, it can occur in immunocompetent patients, especially persons with a rural vocational history. Negative serology results do not exclude cerebral echinococcosis, which requires histological confirmation. Immunohistochemical staining and PCR analysis are especially useful in cases without classic morphological findings.
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