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Inflammatory Optic Neuropathy as a Presenting Feature of Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy. 炎症性视神经病变是大脑常染色体显性动脉病伴有皮质下梗塞和白质脑病的一种表现特征。
IF 1 Q4 Medicine Pub Date : 2023-04-01 Epub Date: 2022-10-26 DOI: 10.1177/19418744221132677
João Moura, Catarina Mendes Pinto, Pedro Pinto, Ernestina Santos

Background: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is commonly associated with early-onset stroke, migraine and vascular dementia. However, optic nerve involvement has been previously recognised. Results: We report a case of a 21-year-old female presenting with right inferior temporal scotoma, dyschromatopsia, relative pupillary afferent defect and bilateral disk oedema in the fundoscopic examination. Visual evoked potential latencies were bilaterally increased, suggesting optic neuropathy. Cerebrospinal fluid (CSF) evaluation showed 11 leukocytes, .45 mg/dL proteins, elevated IgG (4.55 mg/dL) and 2 oligoclonal bands (OCB) restricted to the CSF. ESR was 17 mm/h and CRP 5 mg/dL. Anti-Aquaporin4 anti-MOG antibodies were negative. The MRI showed right optic nerve hyperintensity, enhancing after the administration of contrast product, and multiple FLAIR focal lesions present throughout the white matter, with a noticeable confluence in the anterior temporal horns. She improved after IV high-dose methylprednisolone. Because the lesions of the white matter were highly atypical for an inflammatory disease and highly suggestive of CADASIL genetic testing was requested. A heterozygous pathogenic variant c994C>T p (Arg332Cys) in the exon 6 of the NOTCH3 gene, compatible with the diagnosis of CADASIL was found. Conclusions: This case highlights isolated optic nerve involvement as a presenting feature of CADASIL, possibly reflecting an inflammatory process associated with this hereditary vasculopathy.

背景:大脑常染色体显性动脉病伴有皮层下梗死和白质脑病(CADASIL)通常与早发性中风、偏头痛和血管性痴呆有关。然而,视神经受累的情况以前也曾被发现过。结果:我们报告了一例 21 岁女性患者的病例,她在眼底镜检查中出现右颞下部视网膜瘤、色觉障碍、相对瞳孔传入缺损和双侧视盘水肿。双侧视觉诱发电位潜伏期增加,提示视神经病变。脑脊液(CSF)评估显示有11个白细胞、0.45毫克/分升的蛋白质、升高的IgG(4.55毫克/分升)和2条局限于脑脊液的寡克隆带(OCB)。血沉为 17 毫米/小时,CRP 为 5 毫克/分升。抗Aquaporin4抗MOG抗体呈阴性。核磁共振成像显示右侧视神经高密度,使用造影剂后增强,整个白质存在多个FLAIR灶性病变,颞前角有明显汇合。在静脉注射大剂量甲基强的松龙后,她的病情有所好转。由于白质病变在炎症性疾病中非常不典型,而且高度提示 CADASIL,因此要求进行基因检测。结果发现,NOTCH3 基因第 6 外显子中的 c994C>T p (Arg332Cys) 杂合子致病变异与 CADASIL 诊断相符。结论该病例突出了CADASIL的一个表现特征--孤立的视神经受累,这可能反映了与这种遗传性血管病变相关的炎症过程。
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引用次数: 0
Inpatient Mortality in Parkinson's Disease. 帕金森病住院病人死亡率。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2023-04-01 Epub Date: 2023-03-21 DOI: 10.1177/19418744231153477
Oliver W Phillips, Zachary Kunicki, Richard Jones, Emmanuelle Belanger, Theresa I Shireman, Joseph H Friedman, Duk Soo Kim, Benzi Kluger, Umer Akbar

Introduction: Although a majority of the American public prefer to die at home, a large percentage of Parkinson's disease patients die in acute care hospitals. We examine trends in the clinical and demographic characteristics of Parkinson's disease patients who die in a hospital to identify populations potentially vulnerable to unwanted inpatient mortality.

Methods: Patients with Parkinson's disease admitted to a hospital from 2002-2016 were identified from the National Inpatient Sample (n = 710,013) along with their associated clinical and demographic characteristics. The main outcome examined was mortality during inpatient admission. From these data, logistic regression models were estimated to obtain the odds ratios of inpatient mortality among clinical and demographic attributes, and their change over time.

Results: Characteristics significantly associated with increased odds of inpatient mortality included increased age (OR = 1.70 for 55-65, 2.52 for 66-75, 3.99 for 76-85, 5.72 for 86+, all P < 0.001), length of stay ≤5 days (reference; 6 + days OR = 0.37, P < 0.001), white race or ethnicity (reference; Black OR = .84 P < .001, Hispanic OR = 0.91 P = 0.01), male (reference; female OR = 0.93 P < 0.001), hospitalization in Northeast (reference; Midwest OR = 0.78, South 0.84, West OR = 0.82; all P < 0.001), higher severity of illness (moderate OR = 1.50, major OR = 2.32, extreme OR = 5.57; all P < 0.001), and mortality risk (moderate OR = 2.88, major OR = 10.92, extreme OR = 52.30; all P < 0.001). Fitted probabilities overall declined over time.

Conclusion: Differences exist among PD patient populations regarding likelihood of in-hospital mortality that are changing with time. Insight into which PD patients are most at risk for inpatient mortality may enable clinicians to better meet end-of-life care needs.

导言:尽管大多数美国人更愿意死在家里,但很大一部分帕金森病患者都死在了急症医院。我们研究了死于医院的帕金森病患者的临床和人口统计学特征的变化趋势,以确定可能易受不必要的住院死亡率影响的人群:我们从全国住院病人样本(n = 710,013 人)中确定了 2002-2016 年住院的帕金森病患者及其相关的临床和人口特征。研究的主要结果是住院期间的死亡率。根据这些数据,我们对逻辑回归模型进行了估计,以得出住院病人死亡率在不同临床和人口特征之间的几率比,以及这些几率比随时间的变化情况:01)、男性(参考;女性 OR = 0.93 P <0.001)、东北部住院(参考;中西部 OR = 0.78、南部 0.84、西部 OR = 0.82;所有 P <0.001)、疾病严重程度较高(中度 OR = 1.50、重度 OR = 2.32、极重度 OR = 5.57;所有 P <0.001)和死亡风险(中度 OR = 2.88、重度 OR = 10.92、极重度 OR = 52.30;所有 P <0.001)。随着时间的推移,拟合概率总体下降:结论:随着时间的推移,帕金森病患者群体在院内死亡概率方面存在差异。了解哪些帕金森病患者的住院死亡率风险最高,可帮助临床医生更好地满足临终关怀需求。
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引用次数: 0
Polyradiculitis Complicating SARS-CoV-2 Vaccinations is not Infrequent. 接种 SARS-CoV-2 疫苗后并发多发性神经炎的情况并不少见。
IF 1 Q4 Medicine Pub Date : 2023-04-01 Epub Date: 2022-12-05 DOI: 10.1177/19418744221138365
Josef Finsterer, Sounira Mehri
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引用次数: 0
Strongman - At Home. 强人-在家。
IF 1 Q4 Medicine Pub Date : 2023-04-01 DOI: 10.1177/19418744231153475
Steven R Peters

A neurologist reflects on the unintended heartlessness of keeping a patient with terminal cancer in hospital for further tests at a time when families are not permitted to visit. Peripherally involved in a patient's care, he witnesses the suffering brought about by pandemic-related visitation restrictions in the final weeks of a patient's life. In reviewing the course of events with the patient's widow, the physician has many of his assumptions overturned and more completely grasps the consequences of visitation restrictions on hospitalized patients.

一位神经科医生反思,在不允许家属探视的情况下,将癌症晚期患者留在医院接受进一步检查,这是一种无意的无情。他很少参与病人的护理,目睹了在病人生命的最后几周,与大流行有关的探视限制所带来的痛苦。在与病人的遗孀一起回顾事件的过程中,医生推翻了他的许多假设,并更全面地掌握了探视限制对住院病人的影响。
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引用次数: 0
Fatal Post COVID mRNA-Vaccine Associated Cerebral Ischemia. 致命的 COVID mRNA 疫苗接种后脑缺血。
IF 1 Q4 Medicine Pub Date : 2023-04-01 Epub Date: 2022-12-05 DOI: 10.1177/19418744221136898
Nadia McMillan, Harry J Rosenberg, Matthew P Anderson, Pankaj Pal, Kathryn Stephenson, Corey R Fehnel

Background: Venous thromboses have been linked to several COVID-19 vaccines, but there is limited information on the Moderna vaccine's effect on the risk of arterial thrombosis. Here we describe a case of post-Moderna COVID-19 vaccination arterial infarct with vaccine-associated diffuse cortical edema that was complicated by refractory intracranial hypertension.

Case summary: 24 hrs after receiving her first dose of the Moderna COVID-19 vaccine, a 30-year-old female developed severe headache. Three weeks later she was admitted with subacute headache and confusion. Imaging initially showed scattered cortical thrombosis with an elevated opening pressure on lumbar puncture. An external ventricular drain was placed, but she continued to have elevated intracranial pressure. Ultimately, she required a hemicraniectomy, but intractable cerebral edema resulted in her death. Pathology was consistent with thrombosis and associated inflammatory response.

Conclusion: Though correlational, her medical team surmised that the mRNA vaccine may have contributed to this presentation. The side effects of COVID-19 infection and vaccination are still incompletely understood. Though complications are rare, clinicians should be aware of presentations like this one.

背景:静脉血栓与多种 COVID-19 疫苗有关,但有关 Moderna 疫苗对动脉血栓风险影响的信息却很有限。在此,我们描述了一例接种 Moderna COVID-19 疫苗后动脉梗塞并伴有疫苗相关弥漫性皮质水肿的病例,该病例因难治性颅内高压而并发。病例摘要:一名 30 岁女性在接种第一剂 Moderna COVID-19 疫苗 24 小时后出现严重头痛。三周后,她因亚急性头痛和意识模糊入院。影像学检查初步显示有散在皮质血栓形成,腰椎穿刺开口压力升高。医生为她放置了脑室外引流管,但她的颅内压持续升高。最终,她需要进行半颅切除术,但顽固性脑水肿导致她死亡。病理结果与血栓形成和相关炎症反应一致:尽管存在相关性,但她的医疗团队推测 mRNA 疫苗可能是导致这一病症的原因之一。目前对 COVID-19 感染和疫苗接种的副作用还不完全清楚。虽然并发症很少见,但临床医生应注意类似这种情况。
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引用次数: 0
A 47-Year-Old Man Presenting With Seizures and Prior Stroke. 一名 47 岁的男性癫痫发作并曾中风。
IF 1 Q4 Medicine Pub Date : 2023-01-01 Epub Date: 2022-10-10 DOI: 10.1177/19418744221122877
Kiara Camacho-Caballero, Marco Malaga, Isabella Peixoto de Barcelos, Anibal F Prentice, Aaron L Berkowitz

A 47-year-old man presented to his local hospital in Peru after a generalized tonic-clonic seizure. His family reported a history of prior stroke of unclear etiology. This case report discusses the approach to a first seizure (including in tropical regions like Peru), the relationship between stroke and seizures, the approach to stroke in the young, and how to diagnose rare diseases in resource-limited settings.

一名 47 岁男子因全身强直-阵挛发作前往秘鲁当地医院就诊。据其家人报告,他曾有过病因不明的中风病史。本病例报告讨论了首次癫痫发作的处理方法(包括在秘鲁等热带地区)、中风与癫痫发作之间的关系、年轻人中风的处理方法以及如何在资源有限的环境中诊断罕见病。
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引用次数: 1
Hemichorea-Hemiballism as a Delayed Manifestation of Hyperglycemia: A Case Report. 作为高血糖症延迟表现的血球症:病例报告。
IF 1 Q4 Medicine Pub Date : 2023-01-01 Epub Date: 2022-08-08 DOI: 10.1177/19418744221111962
Vikram Shivkumar, Dipali Nemade

Hemichorea is characterized by involuntary, continuous, non-patterned movements on one side of the body. While it is most commonly caused by strokes, it can also be caused by metabolic derangements such as hyperglycemia. We present two patients who developed hemichorea in the setting of hyperglycemia. Our first patient had a history of uncontrolled diabetes mellitus and developed chorea 2 weeks following a hyperglycemic event. The second patient developed chorea while being on steroids for hip pain and was later diagnosed to have underlying diabetes mellitus. MRI showed hyperintensity in the contralateral lentiform nucleus in both cases. The chorea did not improve despite the correction of the hyperglycemia. Both patients were started on Tetrabenazine with significant improvement. Hyperglycemia-induced hemichorea might be underdiagnosed given the prevalence of diabetes mellitus in the population. Chorea can even be the presenting symptom of diabetes mellitus and in some cases is a delayed manifestation of hyperglycemia.

半身不遂的特征是身体一侧出现不自主、连续、无规律的运动。半身不遂最常见于中风,也可由高血糖等代谢紊乱引起。我们介绍了两名在高血糖情况下出现半身不遂的患者。第一位患者有未控制的糖尿病史,在高血糖事件发生两周后出现舞蹈症。第二名患者因髋部疼痛服用类固醇时出现舞蹈症,后被诊断为潜在糖尿病。核磁共振成像显示,这两名患者的对侧皮状核密度都很高。尽管纠正了高血糖,但舞蹈症并未改善。两名患者开始服用氯丙嗪(Tetrabenazine)后均有明显改善。鉴于糖尿病在人群中的流行率,高血糖诱发的舞蹈症可能被漏诊。舞蹈症甚至可能是糖尿病的首发症状,在某些病例中,舞蹈症是高血糖的延迟表现。
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引用次数: 1
Opioid-Associated Amnestic Syndrome Progressing to Diffuse Leukoencephalopathy. 阿片类相关遗忘综合征进展为弥漫性脑白质病。
IF 1 Q4 Medicine Pub Date : 2023-01-01 DOI: 10.1177/19418744211045862
Dylan Meng, Jason Randhawa
A 49-year-old female with a history of polysubstance use was found unconscious. Drug screen was positive for cocaine, opioids, and fentanyl. Upon awakening, she did not recall the 24 hours prior to her presentation. She had profound anterograde amnesia with no other focal deficits. Lumbar puncture and electroencephalography were unremarkable. Brain MRI revealed restricted diffusion in both hippocampi with associated T2-weighted-Fluid-Attenuated Inversion Recovery (FLAIR) hyperintensities (Figure 1). Three weeks into admission, she developed parkinsonism progressing to akinetic mutism. Repeat MRI showed resolution of the bilateral hippocampal findings with development of diffuse T2-FLAIR white matter hyperintensities consistent with delayed leukoencephalopathy (Figure 1). She had minimal response to medical management and is currently in long-term care. Over forty cases of an acute amnestic syndrome with bilateral hippocampal imaging changes in the context of opioid use have been reported, recently termed opioid-associated amnestic syndrome (OAS). Neuroimaging findings often resolve within days to weeks of the inciting event making diagnosis challenging in this vulnerable population.We report the second case of a diffuse leukoencephalopathy occurringwithin weeks of confirmedOAS as recently defined by Barash et al. In this case, as with many cases of OAS, one cannot rule out the confounding effect of hypoxia as a cause. It is currently unknown how commonly OAS progresses in this manner given the scarcity of reported cases. Prognosis should remain guarded until patients with OAS have been stable for at least one month following presentation based on two recent cases. Further study of OAS is warranted.
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引用次数: 1
Facial Diplegia Complicating Anti-SARS-CoV-2 Vaccinations. 抗SARS-CoV-2疫苗接种并发面瘫
IF 1 Q4 Medicine Pub Date : 2023-01-01 Epub Date: 2022-08-14 DOI: 10.1177/19418744221121430
Josef Finsterer
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引用次数: 0
Successful Treatment of Spinal Gnathostomiasis With Only Steroid Administration-2-Year Follow-Up. 仅使用类固醇成功治疗脊柱钩端螺旋体病--2 年随访。
IF 1 Q4 Medicine Pub Date : 2023-01-01 Epub Date: 2022-08-15 DOI: 10.1177/19418744221121428
Witoon Mitarnun, Praewa Tantisungvarakoon
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引用次数: 0
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