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Cavitating Osmotic Demyelination Syndrome Following Correction of Chronic Hyponatremia in Sheehan's Syndrome: A Novel Case Report. 希恩综合征慢性低钠血症纠正后的空洞渗透性脱髓鞘综合征:新颖的病例报告。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-05 DOI: 10.1177/19418744241279491
Alamgir Shaikh, Moisés León-Ruiz, Ritwik Ghosh, Manoj Soren, Bilwatosh Mukhopadhyay, Shyamal Kanti Pal, Julián Benito-León

Introduction: Rapid correction of hyponatremia can result in osmotic demyelination syndrome (ODS). Sheehan's syndrome, a rare pituitary disorder caused by severe postpartum hemorrhage, is a potential cause of chronic hyponatremia. This case report describes a rare progression of extrapontine myelinolysis to central pontine myelinolysis, ultimately leading to ODS, following the correction of chronic hyponatremia associated with Sheehan's syndrome. Notably, this event occurred a decade after the initial postpartum hemorrhage due to placenta previa.

Case report: A 40-year-old woman from rural West Bengal, India, presented in a comatose state after five years of progressively worsening symptoms, including fatigue, gastrointestinal disturbances, cold intolerance, hair loss, and severe apathy, which had been misdiagnosed as psychogenic and treated with selective serotonin reuptake inhibitors. Two days before her admission to our hospital, she was diagnosed with a lower respiratory tract infection, dehydration, and severe hyponatremia (118 mEq/L) at a local private healthcare facility. Despite treatment with 3% sodium chloride and intravenous antibiotics, her condition deteriorated, prompting her transfer. At the time of hospitalization, the patient was diagnosed with chronic hyponatremia and hypopituitarism consistent with Sheehan's syndrome. This condition was attributed to a severe postpartum hemorrhage that occurred a decade prior, resulting from placenta previa. Initial MRI revealed extrapontine myelinolysis, and the correction of her "compensated" hyponatremia was identified as the cause of her neurological decline. Follow-up MRIs at 7 and 14 weeks confirmed the development of cavitating ODS.

Discussion: This case highlights several key points: First, even a relatively gradual correction of hyponatremia can precipitate ODS, especially in patients with chronic conditions like Sheehan's syndrome. Second, it underscores the importance of meticulous management of chronic hyponatremia to prevent severe neurological outcomes. Third, it illustrates the diagnostic challenges of differentiating Sheehan's syndrome from primary psychiatric disorders, particularly in low-resource settings where the syndrome remains prevalent. The case also emphasizes the need for awareness among healthcare providers about the potential for severe complications arising from even minor corrections in serum sodium levels in such patients.

简介快速纠正低钠血症可导致渗透性脱髓鞘综合征(ODS)。希恩综合征是一种由严重产后出血引起的罕见垂体疾病,也是慢性低钠血症的潜在病因。本病例报告描述了在纠正与希恩综合征相关的慢性低钠血症后,脑外髓鞘溶解发展为中枢性脑桥髓鞘溶解,最终导致 ODS 的罕见病例。值得注意的是,这一事件发生在最初因前置胎盘导致产后出血的十年之后:一名来自印度西孟加拉邦农村地区的 40 岁女性,在出现疲劳、胃肠道紊乱、不耐寒、脱发和严重冷漠等症状五年后出现昏迷状态,这些症状曾被误诊为精神疾病,并接受了选择性 5-羟色胺再摄取抑制剂治疗。入院前两天,她在当地一家私人医疗机构被诊断为下呼吸道感染、脱水和严重低钠血症(118 mEq/L)。尽管她接受了 3% 氯化钠和静脉注射抗生素的治疗,但病情仍然恶化,因此转院。住院时,患者被诊断为慢性低钠血症和垂体功能减退症,与希恩综合征一致。这种情况是由于十年前发生的严重产后出血导致的前置胎盘引起的。最初的核磁共振成像显示她患有脊髓外肌纤维溶解症,而纠正她的 "代偿性 "低钠血症被认为是导致她神经功能衰退的原因。7周和14周的随访核磁共振检查证实她患上了空洞型ODS:本病例强调了几个要点:讨论:本病例强调了几个要点:首先,即使是相对渐进地纠正低钠血症也可能诱发 ODS,尤其是像希恩综合征这样的慢性病患者。其次,该病例强调了对慢性低钠血症进行精细管理以防止出现严重神经系统后果的重要性。第三,该病例说明了将希恩综合征与原发性精神疾病相鉴别的诊断难题,尤其是在该综合征仍然普遍存在的低资源环境中。该病例还强调,医疗服务提供者需要认识到,这类患者的血清钠水平即使是轻微的修正,也有可能导致严重的并发症。
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引用次数: 0
A 38-year-Old Woman With Flaccid Tetraparesis after Presenting With Abdominal Pain. 一名 38 岁女性因腹痛而患上弛缓性四肢瘫。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-02 DOI: 10.1177/19418744241281000
Carolina Rodrigues Dal Bo, Julia Miranda Menezes, Barbara Gabriela Gomes Silva, Sarah Ingrid Farias Dos Santos, Marisa Petrucelli Doher, René de Araújo Gleizer

A 38-year-old woman presented in the emergency department with acute abdominal pain. She underwent laparoscopic cholecystectomy. Postoperatively, she developed worsening abdominal pain, tachycardia, flaccid tetraparesis, urinary retention, constipation and SIADH. She also developed red-colored urine. Porphobilinogen was requested in an isolated urine sample, with the test result showing 256 mg/g of creatinine. This case didactically emphasizes the necessity of considering differential diagnosis in acute abdomen pain associated with neurological disorders.

一名 38 岁的女性因急性腹痛到急诊科就诊。她接受了腹腔镜胆囊切除术。术后,她出现腹痛加剧、心动过速、弛缓性四肢瘫痪、尿潴留、便秘和 SIADH。她还出现了红色尿液。在分离尿样中检测卟啉原,结果显示肌酐含量为 256 毫克/克。本病例从教学角度强调了在急性腹痛伴有神经系统疾病时考虑鉴别诊断的必要性。
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引用次数: 0
Scrub Typhus Meningoencephalitis Presenting as Generalized Convulsive Status Epilepticus with Basal Ganglia and Extra-limbic Cortical Involvement, Complicated by Cortical Multifocal Myoclonus. 恙虫病脑膜炎表现为全身抽搐性癫痫状态,伴有基底节和边缘皮质外受累,并发皮质多灶性肌阵挛。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-02 DOI: 10.1177/19418744241276903
Uttam Biswas, Moisés León-Ruiz, Ritwik Ghosh, Ritun Sarkar, Raghul Bheeman, Arpan Mukhopadhyay, Julián Benito-León

Background: Scrub typhus is an acute febrile infectious disease highly prevalent in the Asia Pacific region, often referred to as the "tsutsugamushi triangle." This mite-borne rickettsial zoonosis is caused by Orientia tsutsugamushi, an intracellular Gram-negative organism that primarily targets endothelial cells. The resulting vasculitis leads to multisystem involvement. In terms of neurological manifestations, meningoencephalitis is the most common presentation of scrub typhus. Other frequent neurological manifestations include cranial nerve paresis, transverse myelitis, and polyneuropathy. Status epilepticus, while reported, is a rare presenting feature of this infection. Although scrub typhus has been documented to present as limbic encephalitis, there have been no previous descriptions in the literature of neuroradiological patterns affecting the basal ganglia or extra-limbic cortices in this condition.

Case report: We report a case of a 23-year-old previously healthy woman who presented with scrub typhus meningoencephalitis. The condition manifested as encephalitis with involvement of the basal ganglia and extra-limbic cortices. She presented with generalized convulsive status epilepticus, which was complicated by cortical multifocal myoclonus.

Discussion: Scrub typhus can be a significant diagnostic challenge, potentially presenting with generalized convulsive status epilepticus and mimicking both clinical and radiological features of arboviral encephalitides, such as those caused by West Nile and Japanese encephalitis viruses. Furthermore, as demonstrated in this case, its radiological presentation can resemble that of autoimmune encephalitis. Given that scrub typhus is amenable to treatment with antibiotics, such as doxycycline and azithromycin, which do not increase seizure risk, it should be considered in the differential diagnosis for patients presenting with seizures or encephalitis, especially in endemic areas.

背景:恙虫病是亚太地区高发的一种急性发热传染病,通常被称为 "恙虫三角"。这种螨媒立克次体人畜共患疾病是由恙虫病菌引起的,恙虫病菌是一种细胞内革兰氏阴性菌,主要针对内皮细胞。由此引发的血管炎会导致多系统受累。就神经系统表现而言,脑膜脑炎是恙虫病最常见的表现。其他常见的神经系统表现包括颅神经麻痹、横贯性脊髓炎和多发性神经病。癫痫状态虽然也有报道,但却是这种感染的罕见表现特征。虽然恙虫病曾被记录为边缘脑炎,但以前的文献中还没有关于影响基底节或边缘外皮层的神经放射学模式的描述:我们报告了一例 23 岁的健康女性恙虫病脑膜脑炎病例。病情表现为脑炎,基底节和肢体外皮层受累。她出现全身抽搐性癫痫,并发皮质多灶性肌阵挛:讨论:恙虫病可能是诊断上的一大难题,它可能表现为全身抽搐性癫痫状态,并模仿虫媒病毒性脑炎的临床和影像学特征,如由西尼罗河病毒和日本脑炎病毒引起的脑炎。此外,正如本病例所示,其放射学表现可能与自身免疫性脑炎相似。鉴于恙虫病可通过抗生素(如强力霉素和阿奇霉素)治疗,且不会增加癫痫发作的风险,因此在对癫痫发作或脑炎患者进行鉴别诊断时应考虑到恙虫病,尤其是在恙虫病流行地区。
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引用次数: 0
Pitfalls in the Evaluation of Respiratory Failure in Myasthenia Gravis Patients: A Case Series. 肌无力患者呼吸衰竭评估中的误区:病例系列。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-31 DOI: 10.1177/19418744241280528
Sanem Pinar Uysal, Yuebing Li

Objectives: To highlight the importance of recognizing different presentations of respiratory failure due to myasthenic and non-myasthenic etiologies in myasthenia gravis (MG) patients.

Methods: We describe 3 patients with different presentations of respiratory failure in MG.

Cases: Patient 1 is a 49-year-old female with longstanding MG who presented with lethargy and neck weakness without notable respiratory distress. She was found to be in hypercarbic respiratory failure, which improved with plasmapheresis treatment. Patient 2 is a 58-year-old female who presented with ptosis, dysphagia, and dyspnea requiring intubation. Her hypophonia and dyspnea persisted despite escalation in MG treatment, and further workup revealed glottal stenosis secondary to granulomatosis with polyangiitis. Patient 3 is an 85-year-old female with MG presenting with refractory hypoxia, which was secondary to a large patent foramen ovale resulting in right-to-left shunting.

Discussion: All 3 cases emphasize the role of clinical reasoning and careful analysis based on thorough history taking, detailed neurologic exam and comprehensive laboratory findings to determine the etiologies for respiratory dysfunction in MG and provide appropriate treatment.

Conclusion: A lack of overt signs of respiratory distress in MG does not rule out the presence of respiratory failure due to the sedating effect of hypercapnia. There is a need to consider alternative etiologies of hypoxia in MG patients if typical symptoms or signs of MG exacerbations are absent.

目的强调识别重症肌无力(MG)患者因肌无力和非肌无力病因导致的呼吸衰竭不同表现的重要性:我们描述了 3 例不同表现的 MG 呼吸衰竭患者:患者 1 是一名 49 岁的女性,患有长期的 MG,表现为嗜睡和颈部无力,但没有明显的呼吸困难。她被发现处于高碳酸血症呼吸衰竭状态,经浆血疗法治疗后病情有所好转。患者 2 是一名 58 岁的女性,出现上睑下垂、吞咽困难和呼吸困难,需要插管治疗。尽管增加了 MG 的治疗,但她的发音减弱和呼吸困难仍持续存在,进一步检查发现她的声门狭窄继发于肉芽肿伴多血管炎。患者3是一名85岁的女性MG患者,表现为难治性缺氧,继发于巨大的卵圆孔导致的右向左分流:讨论:这三个病例都强调了临床推理的作用,以及基于全面的病史采集、详细的神经系统检查和全面的实验室检查结果进行仔细分析,以确定 MG 呼吸功能障碍的病因并提供适当的治疗:结论:MG 缺乏明显的呼吸窘迫症状并不能排除高碳酸血症的镇静作用导致的呼吸衰竭。如果没有 MG 病情加重的典型症状或体征,则需要考虑 MG 患者缺氧的其他病因。
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引用次数: 0
Cortical (Spastic) Isolated Unilateral Foot Drop: The Foot Knob Area. 皮质(痉挛)孤立性单侧足下垂:足钮区。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-31 DOI: 10.1177/19418744241279884
Jamir Pitton Rissardo, Ana Letícia Fornari Caprara

Foot drop is a condition characterized by impairment of the ability to dorsiflex the foot at the ankle joint. We aim to review the literature and report a case of isolated unilateral foot drop of central causes. A 59-year-old male previously healthy presenting with a right foot drop was admitted. Severe weakness of ankle dorsiflexion with intact plantar flexion was observed. Deep tendon reflexes were normal, no clonus was appreciated, and a plantar response resulted in flexion of all toes. Neuroimaging showed a lesion in the high left frontal lobe, centered along the medial aspect of the precentral gyrus. Levetiracetam and dexamethasone were started, and after four days, the patient reported a slight improvement in his ability to dorsiflex his ankle. Abdominal imaging showed a large right renal mass with invasion of the renal pelvis fat, suggestive of renal cell carcinoma, and cytology diagnosed clear cell renal cell carcinoma. There are 25 articles containing 33 individuals with unilateral foot drop secondary to non-traumatic central causes in the literature. The mean and median age were 50.26 (SD = 20.57) and 55.5 years old (12 - 79 years). Most of the patients were males, which accounted for 55.88% (19/34). The side of the foot drop was right at 58.82% (20/34).

足下垂是一种以足部踝关节外翻能力受损为特征的疾病。我们旨在回顾文献,并报告一例由中枢原因引起的孤立性单侧足下垂。患者为一名 59 岁男性,之前身体健康,入院时出现右足下垂。患者踝关节外翻严重无力,但跖屈功能完好。深部腱反射正常,无腱鞘炎,足底反应导致所有脚趾屈曲。神经影像学检查显示,病变位于左侧额叶高位,以中央前回内侧为中心。患者开始服用左乙拉西坦和地塞米松,四天后,他的踝关节外翻能力略有改善。腹部造影显示右肾肿块较大,侵犯肾盂脂肪,提示肾细胞癌,细胞学诊断为透明细胞肾细胞癌。文献中有 25 篇文章,包含 33 名因非外伤性中枢性原因继发的单侧足下垂患者。平均年龄和中位年龄分别为 50.26 岁(SD = 20.57)和 55.5 岁(12 - 79 岁)。大多数患者为男性,占 55.88%(19/34)。右侧足下垂占 58.82%(20/34)。
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引用次数: 0
Feasibility of Percutaneous Gastrostomy Tube Placement Using Only Local Anesthetic in Patients With Neuromuscular Dysfunction. 神经肌肉功能障碍患者仅使用局部麻醉剂经皮胃管置入术的可行性。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-22 DOI: 10.1177/19418744241274507
Srinidhi Shanmugasundaram, Nardine Mikhail, Tarek Jazmati, Abhishek Kumar, Pratik A Shukla

Background: Patients with neuromuscular disorders often require gastrostomy tube placement for feeding but routinely have contraindication to sedation due to poor airway control with intubation avoided at the risk of ventilator dependence.

Purpose: To assess the feasibility of percutaneous gastrostomy tube (G-tube) placement using only local anesthesia in patients with neuromuscular dysfunction.

Research design: A retrospective chart review was performed from 2013 to 2019 for all patients who underwent percutaneous G-tube placement under local anesthesia only.

Study sample: 12 patients (6 females, 6 males; mean age = 52.3 ± 21.8) with neuromuscular disorders underwent G-tube placement with only local anesthesia.

Data collection: Data collected included demographic data, medical history (source of neuromuscular dysfunction), procedural information, and complications.

Results: Technical success was achieved in 100% of patients with no major complications.

Conclusion: Placement of a percutaneous gastrostomy tube using only local anesthesia is safe and feasible in patients who have a contraindication to sedation due to poor airway control and for whom intubation is avoided due to risk of ventilator dependence.

背景:目的:评估神经肌肉功能障碍患者仅使用局部麻醉进行经皮胃造瘘管(G管)置管的可行性.研究设计:研究样本:12 名神经肌肉功能障碍患者(6 名女性,6 名男性;平均年龄 = 52.3 ± 21.8)仅在局部麻醉下接受了经皮胃管置入术:收集的数据包括人口统计学数据、病史(神经肌肉功能障碍的来源)、手术信息和并发症:结果:100% 的患者技术成功,无重大并发症:结论:对于因气道控制不佳而禁用镇静剂的患者,以及因呼吸机依赖风险而避免插管的患者,仅使用局部麻醉置入经皮胃造瘘管是安全可行的。
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引用次数: 0
A 22-Year-Old Man With Headache, Facial Numbness, Diplopia and Left Leg Numbness. 一名 22 岁男子,头痛、面部麻木、复视和左腿麻木。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-21 DOI: 10.1177/19418744241276711
Daniel Gabay Moreira, Paul E Sanmartin

We present a 22-year-old male with a subacute course of chin numbness, ophthalmoplegia, headache and left leg numbness in the setting of fever, chills and night sweats. Initial imaging investigations revealed cauda equina enhancement plus an infiltrative process at the basis of the skull associated with extensive bone marrow infiltration and lymphadenopathy elsewhere. We discuss the localization, differential diagnosis, and appropriate investigations for this unique clinical scenario. Important laboratory findings and treatment considerations are reviewed for this unusual final diagnosis.

我们要介绍的是一名 22 岁男性患者,他在发热、畏寒和盗汗的情况下出现了下巴麻木、眼肌麻痹、头痛和左腿麻木的亚急性病程。最初的影像学检查显示马尾增强,颅底有浸润过程,伴有广泛的骨髓浸润和其他部位的淋巴结病变。我们讨论了这种独特临床情况的定位、鉴别诊断和适当的检查。针对这一不寻常的最终诊断,我们还回顾了重要的实验室检查结果和治疗注意事项。
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引用次数: 0
Focal Status Epilepticus and Extreme Delta Brush Associated With Thrombotic Thrombocytopenic Purpura. 与血栓性血小板减少性紫癜相关的局灶性癫痫状态和极度德尔塔刷状反应
IF 1 Q4 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-04-01 DOI: 10.1177/19418744241245454
Lauren E Yap, Huanwen Chen, Sarah Ganji, Samuel E Calabria, Edwin J Serrano, Andrew B Stemer, Francis G Tirol, Noushin Jazebi

Acute focal neurological deficits demand immediate evaluation. In this report, we present the case of a woman 20-some years of age with a history of hemolytic anemia and thrombocytopenia who presented with altered mental status and focal neurological deficits including aphasia, acute left gaze preference, right homonymous hemianopsia, right lower facial weakness, and right arm and leg weakness. Extensive neurological and hematological workup revealed that the patient suffered from focal status epilepticus associated with an extreme delta brush patten on electroencephalogram, likely secondary to thrombotic thrombocytopenic purpura. This case underscores the connection between hematological disorders and the neurological axis, emphasizing the critical role of integrating the neurological examination and neuroimaging findings to formulate an effective management plan.

急性局灶性神经功能缺损需要立即进行评估。在本报告中,我们介绍了一例 20 多岁的女性病例,她有溶血性贫血和血小板减少病史,出现精神状态改变和局灶性神经功能缺损,包括失语、急性左侧凝视偏好、右侧同侧偏盲、右下面部无力、右臂和右腿无力。广泛的神经学和血液学检查显示,患者患有局灶性癫痫状态,并伴有脑电图上的极度三角刷斑,很可能继发于血栓性血小板减少性紫癜。本病例强调了血液病与神经轴之间的联系,强调了综合神经系统检查和神经影像学检查结果以制定有效治疗方案的关键作用。
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引用次数: 0
Anti-LGI-1 Limbic Encephalitis and Autoimmune Epilepsy Following a Third Dose of COVID-19 Vaccination: A Case Report. 接种第三剂 COVID-19 疫苗后出现抗 LGI-1 边缘脑炎和自身免疫性癫痫:病例报告。
IF 1 Q4 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-02-20 DOI: 10.1177/19418744241234100
Gerry Mullan, Michael Kinney

Anti-leucine rich glioma inactivated 1 (LGI-1) autoimmune encephalitis (AE) typically presents with cognitive impairment, faciobrachial dystonic seizures (FBDS) and hyponatraemia. Reports are growing of neurological complications following coronavirus disease 2019 (COVID-19) vaccination. Here we describe a 50 year old man who developed anti-LGI-1 limbic encephalitis and autoimmune epilepsy 4 days following a dose of the mRNA Pfizer COVID-19 vaccine (of note, his first two vaccinations were viral vector ChAdOX1-S). He presented with focal aware seizures characterised by short-lived episodes of confusion, emotional distress and déjà vu associated with palpitations. He also reported subacute progressive amnesia. He responded well to high-dose steroid and subsequent immunoglobulin therapy. To our knowledge, this is the first reported case of anti-LGI-1 AE following a mixed COVID-19 vaccination regimen. We aim to complement the early literature on this post-COVID-19 vaccination phenomenon.

抗富含亮氨酸胶质瘤灭活1(LGI-1)自身免疫性脑炎(AE)通常表现为认知障碍、面肌强直性发作(FBDS)和低钠血症。关于接种冠状病毒病 2019(COVID-19)疫苗后出现神经系统并发症的报道越来越多。在这里,我们描述了一名 50 岁的男性在接种 mRNA 辉瑞 COVID-19 疫苗 4 天后出现抗 LGI-1 边缘脑炎和自身免疫性癫痫(值得注意的是,他的前两次接种是病毒载体 ChAdOX1-S)。他出现了局灶性意识发作,其特征是短暂发作的意识模糊、情绪低落和似曾相识,并伴有心悸。他还报告了亚急性进行性健忘症。他对大剂量类固醇和随后的免疫球蛋白治疗反应良好。据我们所知,这是第一例报道的接种 COVID-19 混合疫苗后出现抗 LGI-1 AE 的病例。我们的目的是对早期有关 COVID-19 疫苗接种后这一现象的文献进行补充。
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引用次数: 0
Clinical Reasoning: A 38-Year-Old Woman Presenting With Acute Hyperkinetic Movements of Her Right Arm. 临床推理:一名 38 岁女性出现急性右臂过度运动。
IF 1 Q4 CLINICAL NEUROLOGY Pub Date : 2024-07-01 Epub Date: 2024-03-18 DOI: 10.1177/19418744241240524
Dylan Ryan, Tasnim Mushannen, Scott Le

We present a case report of a 38-year-old woman who presented to the hospital with acute onset high amplitude, non-rhythmic, hyperkinetic movements of the right upper extremity, abnormal sensation of the right upper extremity from the elbow to the hand, and the inability to recognize her hand without visual input. This case discusses the differential diagnoses of acute hyperkinetic movement disorders and concurrent alien-limb in a patient presenting within the time window for vascular intervention. Readers are led through the reasoning behind acute interventional decision-making in a patient with a rare presentation. Workup reveals the eventual diagnosis.

我们报告了一例 38 岁女性的病例,她因急性发作右上肢高振幅、非节律性、过度运动、右上肢从肘部到手部的感觉异常以及在没有视觉输入的情况下无法识别自己的手而就诊。本病例讨论了在血管介入治疗时间窗内出现的急性过度运动障碍和并发异肢的鉴别诊断。本病例将引导读者了解对这种罕见表现的患者进行急性介入治疗决策背后的原因。检查结果显示了最终诊断。
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引用次数: 0
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