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Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure. 两个儿童MOGAD的初始表现令人困惑:颅内高压和新发癫痫。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-07-25 DOI: 10.1177/19418744231192159
Daniel J Zhou, Andria M Powers, Caleb A Cave, Emily K Dickas, Mary C Rickard, Geetanjali Rathore, Rhonda R Wright, Rana K Zabad, Sookyong Koh

We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm H2O. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.

我们报告了髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的两种不同的具有挑战性的初始表现。病例1描述了一名12岁男孩,他出现了对止痛药难以治疗的头痛,随后出现了颅内神经病变和颅内高压,经腰椎穿刺证实,开口压力>36 cm H2O。病例2描述了一名3岁男孩,他出现了对抗癫痫药物难治的新发性癫痫发作,在MOG抗体相关的癫痫发作脑炎(FLAMES)中表现为FLAIR高信号病变。在重复磁共振成像中,发现两名患者都有皮质T2高信号、软脑膜造影增强和双侧视神经增强。在脑脊液中,两名患者都有以中性粒细胞为主的脑脊液白细胞增多症。患者接受静脉注射免疫球蛋白、血浆置换和高剂量皮质类固醇治疗。第一名患者病情缓解,而第二名患者需要添加利妥昔单抗来治疗癫痫。这两个病例突出了MOGAD的多形性临床表型。
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引用次数: 0
Subacute Brachial Plexopathy due to Intraneural Epithelioid Hemangioma: A Case Report. 神经上皮样血管瘤引起的亚急性臂丛病:一例报告。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-05-08 DOI: 10.1177/19418744231174688
Vihang Nakhate, Robert P McInnis, Albert Sy, Marcelo Matiello

Brachial plexopathy is a common consideration in the differential diagnosis of upper extremity sensory and motor deficits, and neoplasms signify one possible etiology of brachial plexopathy. Of the neoplastic brachial plexopathies, hemangiomas involving the brachial plexus are rare. Most reported cases describe extraneural brachial plexus hemangiomas that present as a palpable, tender neck mass associated with pain and sensory disturbance, with minimal motor deficits. Here we share the case of a 48 year-old man with intraneural epithelioid hemangioma of the brachial plexus who presented with prominent motor weakness and no palpable mass. The patient presented with subacute onset of left arm pain, numbness and progressive weakness. Neurologic exam revealed lower motor neuron signs and weakness spanning multiple nerve root and peripheral nerve distributions. Dedicated brachial plexus MRI showed two mass lesions involving the cords of the brachial plexus, with corresponding FDG-avidity on PET/CT. Biopsy revealed intraneural atypical epithelioid hemangioma. After nerve transfer surgery, he had moderate improvement in left arm strength. This case serves to: emphasize the importance of both clinical localization and dedicated brachial plexus imaging in the evaluation of brachial plexopathy; introduce to the literature a new clinical presentation of brachial plexus hemangiomas; encourage consideration of neoplastic brachial plexopathy even when faced with an illness script resembling Parsonage-Turner Syndrome, to avoid delays in diagnosis and treatment.

在上肢感觉和运动缺陷的鉴别诊断中,臂丛病是一种常见的考虑因素,而肿瘤是臂丛病的一种可能病因。在肿瘤性臂丛神经病变中,涉及臂丛神经的血管瘤是罕见的。大多数报告的病例描述了神经外臂丛血管瘤,表现为可触摸的、柔软的颈部肿块,伴有疼痛和感觉障碍,运动功能障碍很小。在这里,我们分享一个48岁的男性臂丛神经内上皮样血管瘤的病例,他表现出明显的运动无力,没有明显的肿块。该患者表现为亚急性发作的左臂疼痛、麻木和进行性无力。神经系统检查显示运动神经元体征较低,多个神经根和外周神经分布无力。专用臂丛神经MRI显示两个涉及臂丛神经索的肿块,PET/CT上有相应的FDG亲和力。活检显示神经内非典型上皮样血管瘤。神经移植手术后,他的左臂力量有了适度的改善。本病例旨在:强调临床定位和专用臂丛神经成像在评估臂丛神经病变中的重要性;向文献介绍臂丛血管瘤的一种新的临床表现;鼓励考虑肿瘤性臂丛神经病变,即使面临类似帕森斯-特纳综合征的疾病,以避免延误诊断和治疗。
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引用次数: 0
Subacute Sclerosing Panencephalitis in a 63-Year-Old Woman Presenting as Generalized Choreoathetosis. 63岁女性的亚急性硬化性全脑炎,表现为全身性舞蹈病。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-05-19 DOI: 10.1177/19418744231177105
Ritwick Mondal, Shramana Deb, Manoj Mahata, Somesh Saha, Durjoy Lahiri, Julián Benito-León

The persistence of measles virus infection in childhood and early adolescence can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE), characterized by behavioral disturbances and intellectual disability followed by myoclonic jerks and occasional negative myoclonus. Movement disorders are rarely presenting manifestations in SSPE. We herein report a 63-year-old woman with generalized choreoathetosis as the presenting manifestation of stage-I SSPE. Our case was atypical for the patient's age and clinical presentation with generalized choreoathetosis and bilateral putaminal and caudate nucleus signal hyperintensity. Though highly uncommon, neurologists should keep SSPE as a differential diagnosis among patients with movement disorders. Measles-endemic countries should be more vigilant to the atypical and rare presentations of SSPE, such as generalized choreoathetosis.

麻疹病毒在儿童和青少年早期的持续感染很少会导致一种致命的进行性神经退行性疾病,称为亚急性硬化性全脑炎(SSPE),其特征是行为障碍和智力残疾,随后是肌阵挛抽搐和偶尔的负性肌阵痛。运动障碍很少出现在SSPE中。我们在此报告一位63岁的女性,全身性舞蹈病是第一阶段SSPE的主要表现。就患者的年龄和临床表现而言,我们的病例是非典型的,伴有全身性舞蹈病和双侧壳核和尾状核信号高信号。虽然非常罕见,但神经学家应该将SSPE作为运动障碍患者的鉴别诊断。麻疹流行国家应更加警惕SSPE的非典型和罕见表现,如全身性舞蹈病。
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引用次数: 0
Intravascular Lymphoma as a Cause of Recurrent Strokes - Case Report and Review of the Literature. 血管内淋巴瘤是卒中复发的一个原因——病例报告及文献回顾。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-06-12 DOI: 10.1177/19418744231183483
Charlie Weige Zhao, Tracey H Fan, Thomas Denize, Alba Coraini, Andrew Kraft, Anusha M Kumar, Lucy G Gao, Mayra E Lorenzo, Lyn M Duncan, Erica C Camargo Faye, David J Lin

Background: Intravascular lymphoma is an uncommon cause of ischemic strokes. Because of its rarity and atypical pattern, most diagnoses are made post-mortem.

Case study: We present a case of a 68-year-old male with multiple cardiovascular risk factors and recent SARS-CoV-2 infection who presented with recurrent strokes. Because of his stroke risk factors, he was initially managed with a sequentially escalating antithrombotic regimen. A malignant process was low on the differential at this point given his lack of systemic symptoms. When he continued to have new strokes despite these measures, including a spinal cord infarct, a broad workup was sent including for hypercoagulable states, vasculitis, and intravascular lymphoma. Eventually, a skin biopsy of a cherry angioma returned positive for lymphoma cells. He was treated with methotrexate followed by chemotherapy and rituximab. Unfortunately, he did not improve and was made comfort measures only by his family.

Conclusion: This case illustrates the importance of considering intravascular lymphoma as a potential etiology of recurrent strokes, as early diagnosis and treatment are important for preventing irreversible neurological damage.

背景:血管内淋巴瘤是缺血性中风的一种罕见病因。由于其罕见和非典型模式,大多数诊断都是在死后进行的。病例研究:我们报告了一例68岁的男性,他有多种心血管风险因素,最近感染了严重急性呼吸系统综合征冠状病毒2型,并出现复发性中风。由于他的中风风险因素,他最初采用了一种不断升级的抗血栓方案。鉴于他没有全身症状,恶性过程在这一点上的差异很低。尽管采取了这些措施,包括脊髓梗死,但当他仍有新的中风时,进行了广泛的检查,包括高凝状态、血管炎和血管内淋巴瘤。最终,樱桃血管瘤的皮肤活检结果显示淋巴瘤细胞呈阳性。他接受了甲氨蝶呤治疗,随后接受了化疗和利妥昔单抗治疗。不幸的是,他并没有好转,只有家人给他提供了安慰措施。结论:该病例说明了将血管内淋巴瘤视为复发性中风的潜在病因的重要性,因为早期诊断和治疗对于预防不可逆的神经损伤很重要。
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引用次数: 0
Post COVID mRNA-Vaccine Associated Cerebral Ischemia: Comment. COVID - 19 mrna疫苗相关脑缺血:评论
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-05-15 DOI: 10.1177/19418744231176178
Amnuay Kleebayoon, Viroj Wiwanitkit
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引用次数: 0
Postpartum Sciatic Neuropathy After Uncomplicated Vaginal Delivery. 无并发症阴道分娩后的产后坐骨神经病变。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-07-10 DOI: 10.1177/19418744231180939
Nader Boutros, Dina Saba, Bhavesh Trikamji

Postpartum neuropathies are common, including femoral neuropathy, peroneal neuropathy, lumbosacral trunk plexopathy, and lateral femoral cutaneous neuropathy. Sciatic mononeuropathy in the peripartum period is rare. Postpartum sciatic neuropathy (PSN) in the setting of cesarean section has been reported before. We present a case series of 2 sciatic mononeuropathies after vaginal delivery. Case 1 is a 25-year-old woman who presented with a left foot drop after normal vaginal delivery after being in labor for 3 hours. Case 2 is a 24-year-old woman who presented with a right foot drop after normal vaginal delivery and being in labor for 31 hours. Both cases noted foot drops in the immediate postpartum period. Neurologic examinations revealed flail foot, 4/5 hamstring muscle strength on MRC scale and intact hip abduction. They had paresthesia on the posterolateral aspect of the leg, dorsal and plantar aspect of the foot with absent ankle reflex. MRI did not show evidence of spinal cord, nerve root or plexus involvement. Electrodiagnostic studies revealed evidence of sciatic mononeuropathy proximal to the short head of biceps femoris. They were discharged home with an ankle brace and therapy. At 3 months follow up, they had complete resolution of weakness. There have been a few reported cases of PSN secondary to cesarean section. Sciatic involvement after vaginal delivery is extremely rare. We report 2 cases of PSN after vaginal delivery to highlight that sciatic mononeuropathy can occur not only after cesarean section, but also after uncomplicated vaginal delivery and should raise awareness of this risk to clinicians.

产后神经病变很常见,包括股神经病变、腓神经病变、腰骶干丛病变和股外侧皮神经病变。围产期的Sciatic单神经病是罕见的。剖宫产术中的产后坐骨神经病变(PSN)已有报道。我们报告了一系列2例阴道分娩后的坐骨神经单神经病。病例1是一名25岁的女性,在分娩3小时后,在正常阴道分娩后出现左脚下垂。病例2是一名24岁的女性,在正常阴道分娩并分娩31小时后出现右脚下垂。这两个病例都在产后立即出现足部下垂。神经系统检查显示,连枷足、MRC评分的4/5腘绳肌力量和完整的髋关节外展。他们在腿的后外侧、脚的背侧和足底有感觉异常,脚踝反射缺失。MRI没有显示脊髓、神经根或神经丛受累的证据。电诊断研究显示,股二头肌短头附近存在坐骨神经单神经病。他们出院回家后戴上了踝关节支架并接受了治疗。在3个月的随访中,他们的弱点得到了完全解决。有一些报告的PSN继发于剖宫产。阴道分娩后发生Sciatic受累的情况极为罕见。我们报告了2例阴道分娩后的PSN病例,以强调坐骨神经单神经病不仅发生在剖宫产后,也可能发生在无并发症的阴道分娩后,应提高临床医生对这一风险的认识。
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引用次数: 0
Superficial Cerebellar Siderosis and Spontaneous Intracranial Hypotension Secondary to Dural Tear. 硬脑膜撕裂继发的小脑浅侧积和自发性颅内低血压。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-05-28 DOI: 10.1177/19418744231179346
Priscilla Nethala, Sandhya Gh, Sumanth Shivaram

Spinal dural tears are being increasingly recongnized to cause superficial siderosis and intracranial hypotension. We report a patient with chronic headache who was detected to have cerebellar superficial siderosis and subtle signs of intracranial hypotension on imaging. Spinal imaging showed an upper thoracic dural tear secondary to a paradiscal osteophyte. She improved significantly with surgical repair of the tear. We highlight the importance of recognizing superficial sideorsis in patients with chronic headache as it serves as a marker for dural tear and intracranial hypotension.

硬脊膜撕裂越来越多地被认为会引起浅表性含铁蛋白增多症和颅内低血压。我们报告了一名慢性头痛患者,在影像学检查中发现其患有小脑浅表含铁血黄素沉着症和轻微的颅内低血压症状。脊椎影像显示,椎间盘旁骨赘继发于上胸段硬膜撕裂。通过手术修复撕裂,她的病情明显好转。我们强调了在慢性头痛患者中识别浅表侧斜视的重要性,因为它是硬膜撕裂和颅内低血压的标志。
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引用次数: 0
Bilateral and Symmetrical Basal Ganglia Calcifications May Aid in Mitochondrial Disease Diagnosis in Resource-Limited Settings. 在资源有限的情况下,双侧和对称基底神经节钙化可能有助于线粒体疾病的诊断。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-06-01 DOI: 10.1177/19418744231173831
Amado Jiménez-Ruiz, Victor Aguilar-Fuentes, Fátima Gabriela Macías-Ortíz, José Luis Ruiz-Sandoval
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引用次数: 0
Multidisciplinary Approach to Sedation and Early Mobility of Intubated Critically Ill Neurologic Patients Improves Mobility at Discharge. 插管危重神经系统患者镇静和早期活动的多学科方法改善出院时的活动能力。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-06-21 DOI: 10.1177/19418744231182897
Megan E Barra, Christine Iracheta, Joseph Tolland, Johnathan Jehle, Ljubica Minova, Karen Li, Mary Amatangelo, Patricia Krause, Ayush Batra, Henrikas Vaitkevicius

Background and purpose: Over-sedation may confound neurologic assessment in critically ill neurologic patients and prolong duration of mechanical ventilation (MV). Decreased sedative use may facilitate early functional independence when combined with early mobility. The objective of this study was to evaluate the impact of a stepwise, multidisciplinary analgesia-first sedation pathway and early mobility protocol on medication use and mobility in the neuroscience intensive care unit (ICU).

Methods: We performed a single-center prospective cohort study with adult patients admitted to a neuroscience ICU between March and June 2016-2018 who required MV for greater than 48 hours. Patients were included from three separate phases of the study: Phase I - historical controls (2016); Phase II - analgesia-first pathway (2017); Phase III - early mobility protocol (2018). Primary outcomes included propofol requirements during MV, total rehabilitation therapy provided, and functional mobility during ICU admission.

Results: 156 patients were included in the analysis. Decreasing propofol exposure was observed during Phase I, II, and III (median 2243.7 mg/day vs 2065.6 mg/day vs 1360.8 mg/day, respectively; P = .04 between Phase I and III). Early mobility was provided in 59.7%, 40%, and 81.6% of patients while admitted to the ICU in Phase I, II, and III, respectively (P < .01). An increased proportion of patients in Phase III were walking or ambulating at ICU discharge (26.7%; 8/30) compared to Phase I (7.9%, 3/38, P = .05).

Conclusions: An interdisciplinary approach with an analgesia-first sedation pathway with early mobility protocol was associated with less sedative use, increased rehabilitation therapy, and improved functional mobility status at ICU discharge.

背景和目的:过度镇静可能会混淆危重神经系统患者的神经系统评估,并延长机械通气(MV)的持续时间。减少镇静剂的使用可能有助于早期的功能独立性,与早期的行动能力相结合。本研究的目的是评估逐步,神经科学重症监护室(ICU)药物使用和活动的多学科镇痛第一镇静途径和早期活动方案。方法:我们对2016-2018年3月至6月入住神经科学ICU的需要MV超过48小时的成年患者进行了一项单中心前瞻性队列研究。患者来自研究的三个独立阶段:第一阶段-历史对照(2016);第二阶段-镇痛第一途径(2017);第三阶段-早期移动协议(2018)。主要结果包括MV期间的丙泊酚需求、提供的全面康复治疗以及ICU入院期间的功能活动。结果:156例患者被纳入分析。在I、II和III期观察到丙泊酚暴露量减少(中位数分别为2243.7 mg/天、2065.6 mg/天和1360.8 mg/天;I期和III期之间P=0.04)。在I、II和III期入住ICU的患者中,59.7%、40%和81.6%的患者提供了早期活动能力,与I期(7.9%,3/38,P=0.05)相比,III期患者出院时行走或走动的比例增加(26.7%;8/30),以及改善ICU出院时的功能活动状态。
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引用次数: 0
Continuous EEG Characteristics in Critically ill Patients Presenting With Seizures Prior to Death From Cardiac Arrest. 心脏骤停死亡前出现癫痫发作的危重病人的连续脑电图特征
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-10-01 Epub Date: 2023-05-12 DOI: 10.1177/19418744231174950
Tian Wang, Venkatesh K Raman, Gholam K Motamedi

Background: There have been limited reports about brain activity during cardiac arrest. Here we report 4 patients presenting with seizure who had cardiac arrest leading to their deaths while being on continuous video-EEG (cVEEG) monitoring and one-lead cardiac telemetry. Purpose: We illustrate characteristic stepwise EEG and EKG changes in these critically ill patients prior to their death. Research Design/Study Sample: All patients showed progressive broad spectrum of cardiac arrhythmias at or before the beginning of EEG suppression while there were no such changes seen in a control group of 4 randomly selected patients without cardiac arrest who had seizure on presentation and underwent cVEEG monitoring. Data Collection and Results: There was a progressive decline in EEG potentials associated with decreasing heart rate starting from the posterior region, more pronounced on the left, progressing to complete unilateral deactivation of the left fronto-central head regions while the right-sided networks became hyperactive before bilateral deactivation by the time of asystole. Conclusions: This case series provides a rare opportunity to compare EEG and EKG changes in patients who died while being on continuous EEG and EKG monitoring from hours to minutes prior to cardiac arrest and death.

背景:关于心脏骤停期间大脑活动的报道有限。在这里,我们报告了4名癫痫发作患者,他们在接受连续视频脑电图(cVEEG)监测和单导联心脏遥测时出现心脏骤停,导致死亡。目的:我们展示了这些危重患者死亡前特征性的逐步脑电图和心电图变化。研究设计/研究样本:所有患者在EEG抑制开始时或之前都表现出进行性广谱心律失常,而在由4名随机选择的无心脏骤停患者组成的对照组中没有观察到这种变化,这些患者在出现癫痫发作时进行了cVEEG监测。数据收集和结果:从后部开始,EEG电位逐渐下降,与心率下降相关,在左侧更为明显,进展到左侧额中央头部区域的完全单侧失活,而右侧网络在心脏停搏时双侧失活之前变得过度活跃。结论:该病例系列提供了一个难得的机会来比较心脏骤停和死亡前数小时至数分钟连续监测脑电图和心电图时死亡患者的脑电图和心电图变化。
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引用次数: 0
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