central nervous system lesions

  • 文章类型: Journal Article
    目的:探讨在健康志愿者和中枢神经系统(CNS)病变患者中使用鸟笼2H射频(RF)线圈进行3T氘代谢成像(DMI)的潜力。
    方法:改进的梯度滤波器,自制2H体积射频线圈,和球形k空间采样用于三维化学位移成像采集,以获得高质量的2H标记的水和葡萄糖代谢产物的全脑代谢图像。这些图像是在健康志愿者和三名具有不同病理的CNS病变的受试者中获得的。还进行了硬件和脉冲序列实验,以提高3T时DMI的信噪比。
    结果:在中枢神经系统病变不同的患者中,能够根据解剖标志量化局部葡萄糖代谢,在一名最活跃的疾病患者中观察到乳酸增加。
    结论:DMI提供了在3T时检查患有CNS病变的人类受试者的代谢活性的潜力,对于这种代谢成像技术的未来临床转化的潜力很有希望。
    To explore the potential of 3T deuterium metabolic imaging (DMI) using a birdcage 2 H radiofrequency (RF) coil in both healthy volunteers and patients with central nervous system (CNS) lesions.
    A modified gradient filter, home-built 2 H volume RF coil, and spherical k-space sampling were employed in a three-dimensional chemical shift imaging acquisition to obtain high-quality whole-brain metabolic images of 2 H-labeled water and glucose metabolic products. These images were acquired in a healthy volunteer and three subjects with CNS lesions of varying pathologies. Hardware and pulse sequence experiments were also conducted to improve the signal-to-noise ratio of DMI at 3T.
    The ability to quantify local glucose metabolism in correspondence to anatomical landmarks across patients with varying CNS lesions is demonstrated, and increased lactate is observed in one patient with the most active disease.
    DMI offers the potential to examine metabolic activity in human subjects with CNS lesions with DMI at 3T, promising for the potential of the future clinical translation of this metabolic imaging technique.
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  • 文章类型: Case Reports
    神经肌强直是一种罕见的周围神经兴奋过度综合征,通常与针对contactin相关蛋白样2和富含亮氨酸的抗体有关。胶质瘤灭活1.四价人乳头瘤病毒疫苗Gardasil®,2006年首次获得批准,已知是对乳头瘤病毒6型、11型、16型和18型的高效预防。分子上,这种非感染性重组疫苗是基于从人乳头瘤病毒衣壳中纯化的L1蛋白。自从这种疫苗获得批准以来,几项研究已经调查了其在应用后发生自身免疫性疾病的安全性。这里,我们介绍了第一例Gardasil®疫苗接种后,神经肌强直伴活性钆增强中枢神经系统脱髓鞘病变的病例。
    Neuromyotonia is a rare peripheral nerve hyperexcitability syndrome often associated with antibodies directed against contactin-associated protein-like 2 and leucine-rich, glioma inactivated 1. The quadrivalent human papilloma virus vaccine Gardasil®, first approved in 2006, is known to be a highly effective prophylaxis against papillomavirus types 6, 11, 16, and 18. Molecularly, this non-infectious recombinant vaccine is based on purified L1 proteins from the human papilloma virus capsid. Since the approval of this vaccine, several studies have investigated its safety regarding the occurrence of autoimmune conditions following application. Here, we present the first case of neuromyotonia with active Gadolinium enhancing demyelinating central nervous system lesions following vaccination with Gardasil®.
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  • 文章类型: English Abstract
    一名61岁的男子出现了缓慢进行性的认知能力下降。脑MRI显示左额叶病变沿软膜钆增强。进行了脑活检,组织病理学发现与IgG4相关疾病(IgG4-RD)的诊断相符。血清IgG4水平在正常范围内,并且没有其他全身器官被提示涉及临床症状或实验室检查结果。静脉注射甲基强的松龙治疗,然后口服强的松诱导显着改善了认知功能,并在最初诊断时检测到MRI结果。我们的病例强调了将IgG4-RD作为鉴别诊断之一的重要性,即使在没有正常血清IgG4水平的情况下,当遇到患有孤立性颅骨病变的患者时。
    A 61-year-old man presented with slowly progressive cognitive decline. Brain MRI revealed left frontal lobe lesions with gadolinium enhancement along pia mater. Brain biopsy was performed and histopathological findings was compatible with the diagnosis of IgG4-related disease (IgG4-RD). Serum IgG4 level was within a normal range, and no other systemic organs were suggested to be involved by clinical symptoms or laboratory findings. Intravenous methylprednisolone therapy followed by oral prednisone induction markedly improved the cognitive functions and MRI findings detected at the initial diagnosis. Our case highlights the importance of including IgG4-RD as one of the differential diagnosis when encountering the patients suffering from isolated cranial lesions even in the absence of normal serum IgG4 level.
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  • 文章类型: Case Reports
    BACKGROUND: Juvenile xanthogranuloma (JXG) is a disorder of histiocytic proliferation that affects young children and usually presents as spontaneously regressing cutaneous lesions. JXG with systemic involvement is a rare entity associated with significant morbidity and mortality. Intracranial solitary lesions are uncommon, and when comorbid with multiple lesions of the central nervous system in young children, it has an extremely worse prognosis.
    METHODS: We have reported here an unusual case of a 6-year-old boy who initially presented with the complaints of headache, vomiting, seizure, and speech disorder without cutaneous and other organ involvement and a neurological tendency to sleep. Acute hydrocephalus was detected in his brain CT. As an emergency intervention, ventriculo-peritoneal shunt operation was performed on the patient. His postoperative MRI revealed a disseminated intracranial disease involving the extensive dural, sellar-suprasellar region, the orbit, and the brain parenchyma. The patient accordingly underwent a pterional approach for open biopsy and for the mass tissue diagnosis. Histopathology reports were consistent with JXG. Unfortunately, the patient succumbed to the disseminated disease within 2 months of the JXG diagnosis.
    CONCLUSIONS: JXG is a disorder that usually affects the skin. Intracranial lesion can be simple or have multiple involvement. This is a rare case of fatal disseminated multiple intracranial JXG without cutaneous and other organ manifestations. The presentation as a sellar-suprasellar, extensive dural, orbit, and parenchymal involvement at the time of diagnosis is unusual and rarely described in the literature.
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  • 文章类型: Case Reports
    The abscopal effect is a term that has been used to describe the phenomenon in which localized radiation therapy treatment of a tumor lesion triggers a spontaneous regression of metastatic lesion(s) at a non-irradiated distant site(s). Radiation therapy induced abscopal effects are believed to be mediated by activation and stimulation of the immune system. However, due to the brain\'s distinctive immune microenvironment, extracranial abscopal responses following cranial radiation therapy have rarely been reported. In this report, we describe the case of 42-year-old female patient with metastatic melanoma who experienced an abscopal response following her cranial radiation therapy for her brain metastasis. The patient initially presented with a stage III melanoma of the right upper skin of her back. Approximately 5 years after her diagnosis, the patient developed a large metastatic lesion in her upper right pectoral region of her chest wall and axilla. Since the patient\'s tumor was positive for BRAF and MEK, targeted therapy with dabrafenib and trametinib was initiated. However, the patient experienced central nervous system (CNS) symptoms such as headache and disequilibrium and developed brain metastases prior to the start of targeted therapy. The patient received radiation therapy to a dose of 30 Gy delivered in 15 fractions to her brain lesions while the patient was on dabrafenib and trametinib therapy. The patient\'s CNS metastases improved significantly within weeks of her therapy. The patient\'s non-irradiated large extracranial chest mass and axilla mass also shrank substantially demonstrating the abscopal effect during her CNS radiation therapy. Following radiation therapy of her residual chest lesions, the patient was disease free clinically and her CNS lesions had regressed. However, when the radiation therapy ended and the patient continued her targeted therapy alone, recurrence outside of her previously treated fields was noted. The disease recurrence could be due to the possibility of developing BRAF resistance clones to the BRAF targeted therapy. The patient died eventually due to wide spread systemic disease recurrence despite targeted therapy.
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  • 文章类型: Journal Article
    The present study aimed to characterize the specific pathology and magnetic resonance imaging (MRI) findings observed in patients with leukemia with central nervous system (CNS) lesions, and to determine their value in the management of such patients. Lesions of the CNS were observed during and following treatment of leukemia. The data from stereotactic biopsy-proven pathology (12 patients) and MRI examinations (14 patients) were retrospectively evaluated. Proton-magnetic resonance-spectroscopy was performed in three patients. Factors that predisposed to lesions of the CNS were reviewed from the patient medical records. Among the 14 patients, eight had CNS leukemia, four had a CNS infection and two had a neurodegenerative disorder (one leukoencephalopathy and one glial cell hyperplasia). The clinical diagnosis based on clinical symptoms, signs and MRI features was not consistent with the pathological diagnosis in two patients. In one patient, the clinical diagnosis was a CNS infection; however, the patient\'s pathological diagnosis was CNS leukemia. In the other patient, the clinical diagnosis was CNS leukemia, but the pathological diagnosis was glial cell hyperplasia. CNS lesions in leukemia have a wide range of causes. Apart from the relapse of leukemia in the CNS, there are treatment-associated neurotoxicities and infections that are caused by immunocompromised states. As numerous leukemia-associated CNS lesions are treatable, early diagnosis is essential.
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  • 文章类型: Journal Article
    OBJECTIVE: This review examines the utility of current active, powered, wearable lower limb exoskeletons as aids to rehabilitation in paraplegic patients with gait disorders resulting from central nervous system lesions.
    METHODS: The PRISMA guidelines were used to review literature on the use of powered and active lower limb exoskeletons for neurorehabilitative training in paraplegic subjects retrieved in a search of the electronic databases PubMed, EBSCO, Web of Science, Scopus, ProQuest, and Google Scholar.
    RESULTS: We reviewed 27 studies published between 2001 and 2014, involving a total of 144 participants from the USA, Japan, Germany, Sweden, Israel, Italy, and Spain. Seventy percent of the studies were experimental tests of safety or efficacy and 29% evaluated rehabilitative effectiveness through uncontrolled (22%) or controlled (7%) clinical trials.
    CONCLUSIONS: Exoskeletons provide a safe and practical method of neurorehabilitation which is not physically exhausting and makes minimal demands on working memory. It is easy to learn to use an exoskeleton and they increase mobility, improve functioning and reduce the risk of secondary injury by reinstating a more normal gait pattern. A limitation of the field is the lack of experimental methods for demonstrating the relative effectiveness of the exoskeleton in comparison with other rehabilitative techniques and technologies.
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  • 文章类型: Journal Article
    BACKGROUND: Neuropathology centers are expected to offer a prompt and accurate intraoperative diagnosis regarding tumor/lesion type and grade on fresh unfixed tissue. Level of diagnostic accuracy according to type and grade and also, the experience at a new center has not been reported before.
    OBJECTIVE: The aim of this study is to review the agreement patterns according to tumor/lesion type and grade between intraoperative and final histopathologic diagnosis in central nervous system (CNS) lesion samples received by a newly established neuropathology center at a tertiary care neuropsychiatric hospital.
    METHODS: AGREEMENT BETWEEN INTRAOPERATIVE AND FINAL HISTOPATHOLOGIC DIAGNOSIS WAS CLASSIFIED AS: (I) Grade in agreement but type not in agreement; (II) grade not in agreement but type in agreement; (III) grade and type both not in agreement; (IV) grade and type both in agreement.
    METHODS: Confidence interval (CI) of agreements was calculated for various categories of neoplastic as well as non-neoplastic lesions. CI was also calculated for groups where n × p and n × (1 - p) were more than 5, i.e., fulfilled the requirement of the central limit theorem.
    RESULTS: On retrospective analysis of 333 cases, 284 (85.3%) cases were categorized as neoplastic while 49 (14.7%) cases were categorized as non-neoplastic. Among the neoplastic lesions agreement was seen in 237 (83.5%) cases while 47 (16.5%) cases showed disagreement. Similarly in non-neoplastic category; 46 (93.9%) cases showed agreement while 3 (6.15%) cases showed disagreement. Of the non-neoplastic lesions, one case fell into the agreement category I, 2 in category III and 46 in IV. Among neoplastic lesions, there were 21 cases in agreement category I, 17 in II, 9 in III and 237 in IV. On analyzing the accuracy of intraoperative reporting according to tumor type, the break up was: - Astrocytic: 2 (I), 16 (II), 2 (III), 86 (IV); oligodendroglial: 8 (I), 1 (II); ependymal: 2 (III), 6 (IV); embryonal: 23 (IV); cranial and spinal nerve tumors: 2 (II), 21 (IV); choroid plexus tumors: 4 (IV); meningeal tumors: 3 (I), 1 (III), 49 (IV); metastatic tumors: 3 (I), 17 (IV); cysts (tumor-like conditions): 14 (IV); neuronal and mixed neuronal glial tumors: 1 (III); malignant lymphoma: 1 (III); sellar tumors: 17 (IV); and mixed gliomas: 5 (I).
    CONCLUSIONS: This study identifies problem areas of CNS intraoperative reporting, in a new center, with reference to tumor typing and grading. It may forewarn upcoming centers of neuropathology about the potential problem areas of intraoperative reporting.
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