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Glioblastoma Mimicking Autoimmune Encephalitis. 模拟自身免疫性脑炎的胶质母细胞瘤
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-23 DOI: 10.1177/19418744251343174
Connor M Tierney, Marinos Kontzialis

A 66-year-old female patient presented with progressive short-term memory loss over a period of three months and mild gait imbalance. MRI of the brain demonstrated symmetric expansile T2 FLAIR hyperintensities within the bilateral mesial temporal lobes, thalami, and cingulate gyri (Figure 1). Due to the symmetry of the signal changes in the bilateral cerebral hemispheres and especially the mesial temporal lobes, an autoimmune encephalitis was strongly favored on imaging. Glioblastoma was a consideration on the initial scan; however, it was thought to be much less likely, and the patient received immunosuppression with plasmapheresis and IV steroids. At that time, it was even presumed that the patient improved mildly with plasmapheresis. The patient was discharged on PO steroids; however, a few weeks later the patient presented in status epilepticus. On repeat MRI brain, findings were not significantly changed, and the diagnosis of an autoimmune process was again favored on imaging. The patient received plasmapheresis and IV steroids. However, on the second admission the patient's neurologic function was markedly below baseline per the family's report. The patient had received an extensive autoimmune, infectious and metabolic work up, including testing for Creutzfeldt-Jacob disease, with all the tests coming back negative. Therefore, a brain biopsy was performed to understand the underlying pathology, IDH wild-type glioblastoma. On MRI, the expansile signal changes were bilateral and multifocal, affecting more than three cerebral lobes. This is a case of gliomatosis cerebri, which was misdiagnosed as autoimmune encephalitis due to the symmetry of cerebral involvement.

66岁女性患者表现为进行性短期记忆丧失3个月及轻度步态不平衡。脑部MRI显示双侧内侧颞叶、丘脑和扣带回内对称的扩张性T2 FLAIR高信号(图1)。由于双侧大脑半球尤其是内侧颞叶信号变化的对称性,自身免疫性脑炎在影像学上非常有利。最初扫描时考虑的是胶质母细胞瘤;然而,人们认为这种可能性要小得多,患者接受了血浆置换和静脉注射类固醇的免疫抑制。当时,人们甚至认为患者在血浆置换后病情有轻微好转。患者在PO类固醇治疗下出院;然而,几周后患者出现癫痫持续状态。在重复的MRI脑部检查中,发现没有明显的变化,并且自身免疫过程的诊断再次倾向于影像学。患者接受血浆置换和静脉注射类固醇。然而,在第二次入院时,患者的神经功能明显低于家庭报告的基线。患者接受了广泛的自身免疫、感染和代谢检查,包括克雅氏病检测,所有检测结果均为阴性。因此,进行脑活检以了解潜在病理,IDH野生型胶质母细胞瘤。在MRI上,扩张性信号改变是双侧和多灶性的,影响三个以上的脑叶。这是一例脑胶质瘤病,由于大脑受累的对称性而被误诊为自身免疫性脑炎。
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引用次数: 0
Horner's Syndrome and Mediastinal Schwannoma. 霍纳综合征和纵隔神经鞘瘤。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-21 DOI: 10.1177/19418744251345025
Riya Sharma, Siddharth Chand, Manoj Kumar Goyal, Ritu Shree

A 13-year-old boy developed right-sided Horner's syndrome following resection of a benign mediastinal schwannoma extending from T1 to T3. Postoperatively, he exhibited ptosis, miosis, and anhidrosis, confirmed by starch iodine testing. The tumor likely involved the upper thoracic sympathetic ganglia-a rare site for schwannomas. This image highlights a rare iatrogenic cause of preganglionic Horner's syndrome. While Horner's is classically associated with apical lung or cervical lesions, this case emphasizes the importance of recognizing postoperative Horner's syndrome as a clinical clue to cervicothoracic sympathetic injury. It highlights the value of anatomical-clinical correlation in localizing lesions along the sympathetic pathway.

一个13岁的男孩在切除从T1到T3的良性纵隔神经鞘瘤后出现右侧霍纳综合征。术后表现为上睑下垂、瞳孔缩小、无汗,经淀粉碘试验证实。肿瘤可能累及胸上交感神经节——神经鞘瘤的罕见部位。这张图像突出了神经节前霍纳综合征的罕见医源性病因。虽然霍纳综合征通常与肺顶端或颈椎病变相关,但本病例强调了将术后霍纳综合征视为颈胸交感神经损伤的临床线索的重要性。它强调了沿交感神经通路定位病变的解剖-临床相关性的价值。
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引用次数: 0
Ataxia-Parkinsonism in a Patient With Double-Positive Anti-Hu and Anti-NMDAR Antibodies. 抗hu和抗nmdar抗体双阳性患者的共济失调性帕金森病。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-19 DOI: 10.1177/19418744251342295
Carolina Cunha, Pedro Faustino, Inês Carvalho, Ana Morgadinho, Rosário Cunha, Diogo Reis-Carneiro

Movement disorders may be a core feature of autoimmune brain disorders, including paraneoplastic neurological syndromes. A 73-year-old white male presents with memory impairment, gait instability, dysphagia, and severe dysarthria progressing over 1.5 years. He recently developed behavior and sleep disturbances. His cognitive assessment showed time disorientation, short-term memory impairment with preserved retrieval, and pseudobulbar affect. The remainder of the neurological exam showed seborrhea, facio-cervical dystonia, a right positive palmomental reflex, fragmented pursuit eye movements, dysarthria, minor right pyramidal signs, bilateral asymmetric limb ataxia and symmetric akinetic parkinsonism. He was wheelchair-bound, capable of only a few short steps with help. MRI depicted generalized cortico-subcortical atrophy with temporal predominance. He was positive for antibodies anti-Hu in serum (1:10 000) and CSF (1:100), as antibodies anti-NMDAR in serum (1:320) and CSF (1:10). The patient had no clinical improvement after a therapeutical trial with 1 g intravenous methylprednisolone. Additional workup for occult neoplasia was positive for prostatic adenocarcinoma. He remains stable 2.5 years after disease onset.

运动障碍可能是自身免疫性脑疾病的核心特征,包括副肿瘤神经综合征。一个73岁的白人男性表现为记忆障碍,步态不稳定,吞咽困难,严重构音障碍进展超过1.5年。他最近出现了行为和睡眠障碍。他的认知评估显示时间定向障碍,保留检索的短期记忆障碍和假球影响。其余的神经学检查显示:皮脂分泌、面颈肌张力障碍、右侧手掌反射阳性、碎片化的眼球运动、构音障碍、轻微的右侧锥体征、双侧不对称肢体共济失调和对称动力性帕金森病。他坐轮椅,在别人的帮助下只能走几步。MRI显示全身性皮质-皮质下萎缩,以颞部为主。血清抗hu抗体(1:10 000)和脑脊液抗体(1:100)阳性,血清抗nmdar抗体(1:20 20)和脑脊液抗体(1:10)阳性。患者在静脉注射1g甲基强的松龙治疗试验后无临床改善。对隐匿性肿瘤的进一步检查显示前列腺腺癌阳性。发病2.5年后病情稳定。
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引用次数: 0
Early Neurological Deterioration in Ischemic Stroke: Prevalence, Predictors, Causes and 90-day Outcome in a Retrospective Tunisian Cohort. 缺血性卒中的早期神经退化:突尼斯回顾性队列的患病率、预测因素、原因和90天结果
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-14 DOI: 10.1177/19418744251341922
Narjes Gouta, Nizar Daoussi, Rihab Ben Dhia, Yasmine Saad, Mariem Mhiri, Mahbouba Frih-Ayed

Introduction: Early neurological deterioration (END) in ischemic stroke (IS) patients is a common complication that significantly impacts functional and vital prognoses. We aimed to determine the prevalence, predictors, and 90-day outcomes of END in Tunisian stroke patients.

Materials and methods: This was a retrospective cohort study of consecutive stroke cases admitted at the Neurology Department of the University Hospital in Monastir over 5 years, from 2018 to 2022. We included patients with an increment of two or more points on the NIHSS score within the first 7 days following IS. Univariate analysis and binary logistic regression were performed to identify independent factors associated with END.

Results: We included 489 patients with a mean age of 64 years (24 to 90) and a male predominance (sex ratio M/F = 1.86). The prevalence of END was 12.06% (59/489 patients). An elevated initial NIHSS score (OR = 1.13; 95% CI = 1.05-1.21), anterior choroidal artery stroke (OR = 5.39; 95% CI = 1.99-14.55), and large artery atherosclerosis (OR = 2.85; 95% CI = 1.17-6.9), were found to be independent factors associated with END. The causes of END included IS recurrence (12%), hemorrhagic transformation (10%), brain edema (10%), and stroke progression (68%). At 90 days, 80.8% (P < 0.001) of patients who experienced END had a mRS score of 2 or more, with a mortality rate of 18.6% (P < 0.001).

Conclusion: Patients with elevated NIHSS scores, AChA strokes, or LAA, should be prioritized for close observation during the acute phase. Enhancing our understanding of the predictive factors of END following IS could help target patients at very high risk of END and facilitate the development of more effective and rigorous strategies for prevention and treatment.

缺血性脑卒中(IS)患者的早期神经功能恶化(END)是一种常见的并发症,显著影响功能和生命预后。我们的目的是确定突尼斯卒中患者中END的患病率、预测因素和90天预后。材料和方法:本研究是一项回顾性队列研究,研究对象为莫纳斯提尔大学医院神经内科2018年至2022年5年内连续收治的脑卒中病例。我们纳入了在IS后的前7天内NIHSS评分增加2分或更多的患者。采用单因素分析和二元逻辑回归来确定与END相关的独立因素。结果:纳入489例患者,平均年龄64岁(24 ~ 90岁),男性居多(性别比M/F = 1.86)。END患病率为12.06%(59/489例)。初始NIHSS评分升高(OR = 1.13;95% CI = 1.05-1.21),脉络膜前动脉卒中(OR = 5.39;95% CI = 1.99-14.55),大动脉粥样硬化(OR = 2.85;95% CI = 1.17-6.9),是与END相关的独立因素。END的病因包括IS复发(12%)、出血性转化(10%)、脑水肿(10%)和卒中进展(68%)。90天时,80.8% (P < 0.001)的END患者mRS评分为2分或以上,死亡率为18.6% (P < 0.001)。结论:急性期NIHSS评分升高、AChA卒中或LAA患者应优先进行密切观察。加强我们对IS后END的预测因素的理解,可以帮助针对END风险极高的患者,并促进制定更有效和严格的预防和治疗策略。
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引用次数: 0
Utilization of DWI-FLAIR Mismatch for Intravenous Thrombolysis in an Elderly Patient With Stroke. DWI-FLAIR不匹配在老年脑卒中患者静脉溶栓中的应用。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-13 DOI: 10.1177/19418744251343999
Dylan Ryan, Vincent Chang, Aya Ouf

Objectives: To discuss utility of using DWI-FLAIR mismatch in select patients not included in the original WAKE-UP trial for administration of IV thrombolytics.

Methods: We identified a female over 100 years old who presented with stroke symptoms upon waking up. This case is selected due to its unique management. Relevant clinical data was collected through a review of the patient's medical records. All data were anonymized to ensure confidentiality.

Results: A 102-year-old female with a complex past medical history of atrial fibrillation, not on anticoagulation presented with a National Institutes of Health Stroke Scale (NIHSS) of 23. Stroke symptoms were present upon awakening. Noncontrast computed tomography (CT) of the head was negative for hemorrhage or early ischemic changes. CT angiography (CTA) of the head and neck was notable for a distal right M2 occlusion. A hyperacute magnetic resonance imaging (MRI) of the brain was pursued to determine potential eligibility for intravenous thrombolysis (IVT). Patient consented to IVT. NIHSS improved to 13. She was eventually discharged to a skilled nursing facility.

Discussion: We aimed to highlight the oldest known case of IV thrombolysis in this patient presenting with a stroke upon awakening. This is to emphasize possible benefit in cases not included in the original WAKE-UP trial.

目的:探讨在未纳入原始唤醒试验的患者中使用DWI-FLAIR错配静脉溶栓药物的效用。方法:我们确定了一位100岁以上的女性,她在醒来时出现中风症状。本案例因其独特的管理而被选中。通过查阅患者的医疗记录收集了相关的临床资料。所有数据都经过匿名处理以确保机密性。结果:一名102岁女性,既往有复杂的房颤病史,未进行抗凝治疗,其美国国立卫生研究院卒中量表(NIHSS)为23。醒来时出现中风症状。头部非对比计算机断层扫描(CT)未见出血或早期缺血性改变。头颈部CT血管造影(CTA)显示右侧远端M2闭塞。采用脑超急性磁共振成像(MRI)来确定静脉溶栓(IVT)的潜在资格。患者同意静脉滴注。NIHSS提高到13。她最终被送到了一家专业护理机构。讨论:我们的目的是强调已知最古老的静脉溶栓病例,该患者在醒来时出现中风。这是为了强调在最初的WAKE-UP试验中未包括的病例中可能的益处。
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引用次数: 0
A 51-Year-Old Woman With Subarachnoid Hemorrhage and Secondary Central Nervous System Vasculitis With Progression to Diffuse, Serpiginous Dolichoectasia. 51岁女性蛛网膜下腔出血伴继发性中枢神经系统血管炎,进展为弥漫性蛇形血管扩张。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-12 DOI: 10.1177/19418744251336501
Janet A Montelongo, Carley A Ellis, Jennifer J Cheng, Timothy A Fields, Daffolyn Rachael Fels Elliott, Abid Y Qureshi

A 51-year-old woman presented with acute onset of a severe headache, and was found to have diffuse subarachnoid hemorrhage with prominent cisternal and left cortical convexity blood on head computed tomography. The first 2 conventional angiograms were negative for aneurysm, but a third angiogram revealed a mycotic aneurysm of a distal left middle cerebral artery branch. Brain biopsy, associated with clipping of the aneurysm, demonstrated pathology consistent with vasculitis. Over the course of a month, she developed diffuse, serpiginous dolichoectasia of the cerebral arteries. Further investigation into the cause of vasculitis supported a diagnosis of either eosinophilic granulomatosis with polyangiitis (EGPA) or IgG4-Related Disease (IgG4-RD). The following clinical pathologic conference discusses the diagnostic challenges in discriminating between these 2 diseases, particularly in the setting of secondary angiitis of the central nervous system.

51岁女性,急性发作,严重头痛,头部计算机断层扫描发现弥漫性蛛网膜下腔出血,池和左皮质凸面血明显。前两次常规血管造影均未发现动脉瘤,但第三次血管造影显示左侧远端大脑中动脉分支的真菌性动脉瘤。脑活检,与动脉瘤夹闭有关,病理表现与血管炎一致。在一个月的时间里,她出现了弥漫性的、蛇形的脑动脉扩张。血管炎病因的进一步调查支持嗜酸性肉芽肿病合并多血管炎(EGPA)或igg4相关疾病(IgG4-RD)的诊断。接下来的临床病理会议将讨论鉴别这两种疾病的诊断挑战,特别是继发性中枢神经系统脉管炎的诊断挑战。
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引用次数: 0
Axonal Neuropathy in Hepatic Porphyria Should Not be Confused With Guillain-Barre Syndrome. 肝性卟啉症的轴突神经病不应与格林-巴利综合征混淆。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-09 DOI: 10.1177/19418744251342108
Josef Finsterer
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引用次数: 0
Acute Hepatic Porphyria vs. Guillain-Barré Syndrome: Response to "Axonal Neuropathy in Hepatic Porphyria Should Not be Confused With Guillain-Barre Syndrome". 急性肝卟啉症与格林-巴利综合征:对“肝卟啉症轴突神经病变不应与格林-巴利综合征混淆”的反应。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-08 DOI: 10.1177/19418744251342109
Ashok Kumar Pannu
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引用次数: 0
Stroke Hospitalization Administration & Monitoring: Routine or COVID-19 Care (SHAMROCC). 卒中住院管理和监测:常规或COVID-19护理(SHAMROCC)。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-05-02 DOI: 10.1177/19418744251338601
Timothé Langlois-Thérien, Michel Shamy, Brian Dewar, Tim Ramsay, Ronda Lun, Dylan Blacquiere, Robert Fahed, Dar Dowlatshahi, Grant Stotts, Célina Ducroux

Background: Monitoring stroke patients in critical-care units for 24 h after thrombolysis or endovascular thrombectomy is considered standard of care in current guidelines but is not evidence-based. Due to the COVID-19 pandemic, our center adopted a targeted protocol in April 2021 with 24-h critical-care monitoring no longer being guaranteed for stroke patients receiving reperfusion treatment. We aim to compare the incidence and timing of complications during the year under the targeted approach compared to prior years when the standard of care was followed.

Methods: We conducted a single-center retrospective cohort study. We analyzed data from stroke patients treated with thrombolysis and/or endovascular thrombectomy in 2019 (pre-COVID-19, standard of care), 2020 (during COVID-19, standard of care) and 2021 (during COVID-19, targeted protocol). Data extracted included demographics, the nature and timing of complications within the first 24 h, and the unit at the time of complication.

Results: Three hundred forty-nine patients were included in our study: 78 patients in 2019, 115 patients in 2020, and 156 patients in 2021. In 2021, 32% of patients experienced at least 1 complication within the first 24 h compared to 34% in 2020 and 27% in 2019. In 2021, 33% of patients admitted to critical-care units had a complication compared to 29% in non-critical care units. In 2021, 70% of complications had occurred by hour 8 compared to 49% in 2020 and 29% in 2019.

Conclusions: The incidence and timing of complications did not significantly worsen under the targeted approach compared to prior years and were not associated with hospital location.

背景:在目前的指南中,对重症监护病房的脑卒中患者进行溶栓或血管内取栓后24小时的监测被认为是标准的护理,但没有证据支持。由于2019冠状病毒病大流行,我中心于2021年4月采取针对性方案,不再保证对脑卒中再灌注患者进行24小时重症监护监护。我们的目的是比较在有针对性的方法下的一年并发症的发生率和时间与前几年相比,当护理标准被遵循。方法:我们进行了一项单中心回顾性队列研究。我们分析了2019年(COVID-19前,标准护理)、2020年(COVID-19期间,标准护理)和2021年(COVID-19期间,靶向方案)接受溶栓和/或血管内取栓治疗的脑卒中患者的数据。提取的数据包括人口统计数据,前24小时内并发症的性质和时间,以及并发症发生时的单位。结果:349例患者纳入我们的研究:2019年78例,2020年115例,2021年156例。2021年,32%的患者在前24小时内至少出现1种并发症,而2020年为34%,2019年为27%。2021年,33%入住重症监护病房的患者出现并发症,而非重症监护病房的这一比例为29%。2021年,70%的并发症发生在8小时前,而2020年和2019年分别为49%和29%。结论:与前几年相比,靶向入路下并发症的发生率和时间没有明显恶化,与医院位置无关。
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引用次数: 0
Spontaneous Abnormal Vertical Eye Movements of Coma. 昏迷的自发性异常垂直眼动。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2025-04-29 DOI: 10.1177/19418744251331649
Claire Allen, Siena Duarte, Jaeho Hwang, Romergryko G Geocadin, Kemar E Green

Spontaneous vertical eye movements in the critical care setting are often a source of confusion and alarm; while their origin remains at least partly theoretical, understanding their classification and associated clinical implications can inform the diagnostic workup and further clinical management. This case describes a patient who demonstrated ocular dipping: slow conjugate downward eye movements with a quick return to primary gaze. Ocular dipping is a rare phenomenon that was initially described in patients with hypoxic brain injury and has since been described in only a handful of cases. Dipping resides in a spectrum of spontaneous vertical eye movements, with ocular bobbing being the first of these described eye movements. Ocular bobbing is characterized by a fast downward movement followed by a slow return to the mid gaze position which is classically associated with pontine injury. Other vertical eye movements that can be seen in patients with a disorder of consciousness include other variations of ocular bobbing and dipping, vertical myoclonus, and small-amplitude mainly vertical movements.

在重症监护环境中,自发的垂直眼动往往是混乱和惊慌的来源;虽然它们的起源至少部分停留在理论层面,但了解它们的分类和相关的临床意义可以为诊断检查和进一步的临床管理提供信息。这个病例描述了一个患者表现出眼球下垂:缓慢的向下共轭眼球运动与快速返回到原凝视。眼球下沉是一种罕见的现象,最初在缺氧脑损伤患者中被描述,此后只有少数病例被描述。倾斜存在于自发垂直眼球运动的光谱中,眼球上下跳动是这些描述的眼球运动中的第一种。眼球跳动的特征是快速向下运动,然后缓慢地回到凝视中位,这通常与脑桥损伤有关。在意识障碍患者中可看到的其他垂直眼动包括眼球上下摆动、垂直肌阵挛和主要为垂直的小幅度运动。
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引用次数: 0
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Neurohospitalist
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