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COVID-19-Related Spinal Subdural Hematoma Presented with Acute Compressive Myelopathy with a Review of the Literature.
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-11-29 eCollection Date: 2024-01-01 DOI: 10.1159/000528310
Hasanain A Al-Khalidi, Hayder K Hassoun, Zahra Aljid, Zuhair Allebban

Introduction: Neurological complication due to coronavirus disease 2019 (COVID-19) is accumulating and compressive myelopathy due to spinal subdural hematoma (SSDH) is rarely reported in association with COVID-19.

Case presentation: A 55-year-old male was presented with sudden onset of areflexic paraparesis, urinary retention, loss of all sensations below twelve spinal thoracic segments, and severe back pain. This condition necessitated an immediate order of a spinal cord MRI followed by an urgent surgery, which was crucial to save the spinal cord. COVID-19 was confirmed by a positive reverse-transcription-polymerase chain reaction and spinal MRI showed SSDH.

Conclusion: For a patient who presents with acute onset of severe back pain and myelopathy without a history of trauma, SSDH should be suspected. Additionally, coagulopathy associated with COVID-19 infection should increase the suspicion of SSDH which needs immediate surgical treatment to save the spinal cord.

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引用次数: 0
Virus-Induced Voracity: Uncovering Hyperphagia Post-Herpes Simplex Virus Type 1. 病毒引起的嗜睡:揭秘 1 型单纯疱疹病毒后食欲亢进。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-09 eCollection Date: 2024-01-01 DOI: 10.1159/000541698
Arpan Mitra, Nayana Bhuyan, Ankur Vivek, Akansha Jain, Vijaya Nath Mishra, Abhishek Pathak

Introduction: Herpes simplex virus type 1 (HSV-1) is the leading cause of sporadic fatal encephalitis, typically presenting with temporal lobe abnormalities. It usually manifests as fever, headache, seizure, altered sensorium, and focal neurological deficit. Hyperphagia as a sole complication of HSV-1 encephalitis is a rare presentation.

Case presentation: We report a 25-year-old woman with a 10-day history of fever, headache, and vomiting, progressing to confusion, visual hallucinations, and drowsiness. She had a history of meningoencephalitis at age 8 and well-controlled focal seizures. Upon admission, magnetic resonance imaging showed T2/fluid-attenuated inversion recovery hyperintensities in both temporal lobes with diffusion restriction. Electroencephalography indicated generalized slowing and cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis with elevated protein levels. Viral encephalitis was suspected, and intravenous acyclovir was initiated. CSF polymerase chain reaction (PCR) confirmed HSV-1. With treatment, she gradually improved but developed hyperphagia during hospital stay. Hyperphagia, a rare complication of herpes simplex virus (HSV) encephalitis, is a part of Kluver-Bucy syndrome typically associated with other cognitive dysfunctions. Despite early treatment, voracious appetite remained partially, emphasizing the need for rapid diagnosis and treatment to prevent severe outcomes.

Conclusion: The case highlights that acute onset hyperphagia can be an isolated complication of HSV encephalitis, requiring tailored therapeutic strategies. Follow-up showed significant weight gain with partial improvement in hyperphagia, underscoring the challenges in managing this condition.

简介单纯疱疹病毒 1 型(HSV-1)是散发性致命脑炎的主要病因,通常表现为颞叶异常。它通常表现为发热、头痛、抽搐、感觉改变和局灶性神经功能缺损。吞咽功能亢进作为 HSV-1 脑炎的唯一并发症是一种罕见表现:我们报告了一名 25 岁女性的病例,她发热、头痛和呕吐已有 10 天病史,随后出现意识模糊、视幻觉和嗜睡。她 8 岁时曾患脑膜脑炎,局灶性癫痫发作控制良好。入院时,磁共振成像显示她的两个颞叶均出现T2/流体增强反转恢复高密度,弥散受限。脑电图显示全身运动减慢,脑脊液(CSF)分析显示淋巴细胞增多,蛋白水平升高。怀疑是病毒性脑炎,于是开始静脉注射阿昔洛韦。脑脊液聚合酶链反应(PCR)证实了 HSV-1。经过治疗,她的病情逐渐好转,但在住院期间出现了吞咽功能亢进。食欲亢进是单纯疱疹病毒(HSV)脑炎的一种罕见并发症,是克鲁瓦-布西综合征的一部分,通常伴有其他认知功能障碍。尽管患者得到了早期治疗,但部分患者仍然食欲旺盛,这强调了快速诊断和治疗的必要性,以防止出现严重后果:本病例强调,急性发作性多食可能是 HSV 脑炎的一种独立并发症,需要采取有针对性的治疗策略。随访结果显示,患者体重明显增加,多食情况得到部分改善,这凸显了治疗这种病症所面临的挑战。
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引用次数: 0
A Rare Case of Capnocytophaga canimorsus Meningitis in an Immunocompetent Patient: A Successful Outcome with a Short Course of Antibiotics. 免疫功能正常患者患卡氏嗜血杆菌脑膜炎的罕见病例:短程抗生素治疗的成功结果。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-04 eCollection Date: 2024-01-01 DOI: 10.1159/000541631
Adrienne Elisabeth van der Hoeven, Josephus Johannes Kerremans, Tjerk Joppe Lagrand

Introduction: Capnocytophaga canimorsus is a Gram-negative bacterium found in the oral flora of dogs and cats, transmitted to humans through bites, licks, or scratches. Infections can lead to severe manifestations, including meningitis, particularly in immunocompromised individuals.

Case presentation: A 46-year-old immunocompetent man presented with somnolence, headache, and fever after being licked by his dog. Neurological examination revealed signs of meningeal irritation, and cerebrospinal fluid analysis showed an elevated white cell count and protein levels consistent with bacterial meningitis. Treatment followed Dutch guidelines with amoxicillin, ceftriaxone, and dexamethasone, resulting in rapid clinical improvement. Microbiological confirmation of C. canimorsus followed later. The patient was treated with antibiotics for the duration of 1 week and remained symptom-free after being discharged.

Conclusion: C. canimorsus meningitis, although rare, poses diagnostic challenges due to its variable presentation and slow growth in culture. Empirical therapy guided by susceptibility testing contributes to favorable outcomes. This case underscores the importance of considering a C. canimorsus infection in patients with animal exposure and of taking diagnostic findings, precedent, and clinical response into account when determining the treatment duration.

简介Capnocytophaga canimorsus是一种革兰氏阴性细菌,存在于狗和猫的口腔菌群中,通过咬伤、舔舐或抓挠传播给人类。感染可导致包括脑膜炎在内的严重症状,尤其是在免疫力低下的人群中:病例介绍:一名 46 岁免疫力低下的男子在被狗舔后出现嗜睡、头痛和发烧。神经系统检查显示有脑膜刺激症状,脑脊液分析显示白细胞计数和蛋白质水平升高,与细菌性脑膜炎相符。按照荷兰指南,患者接受了阿莫西林、头孢曲松和地塞米松治疗,临床症状迅速好转。随后经微生物学确诊为卡尼莫司菌。患者接受了为期一周的抗生素治疗,出院后仍无症状:结论:卡尼莫索斯氏杆菌脑膜炎虽然罕见,但由于其表现形式多变、培养生长缓慢,给诊断带来了挑战。以药敏试验为指导的经验疗法有助于取得良好的疗效。本病例强调了在有动物接触的患者中考虑卡尼莫氏菌感染的重要性,以及在确定治疗时间时考虑诊断结果、先例和临床反应的重要性。
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引用次数: 0
Periodic Paralysis: A Case Series with a Literature Review. 周期性麻痹:病例系列与文献综述。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-02 eCollection Date: 2024-01-01 DOI: 10.1159/000541585
Bassem Al Hariri, Muad Abdi Hassan, Muhammad Sharif, Obada Adel Alsakaji, Yussuf Abdi Hassan, Muayad Kasim Khalid

Introduction: Periodic paralysis is a condition that causes recurrent episodes of flaccid paralysis, and it can be primary or secondary. Hypokalemic periodic paralysis is the most common type of primary periodic paralysis, and it is inherited through autosomal dominant gene transmission. Males are affected three times more often than females, and the paralysis attacks usually occur at night after a period of vigorous exercise. It is crucial to exclude other diagnostic entities based on the nature of presentation, physical examination, and paraclinical studies. Thyrotoxic periodic paralysis is more prevalent in Asian or Hispanic males with thyrotoxicosis, where up to 10% of thyrotoxic patients may experience periodic paralysis.

Case presentations: Here, we present 6 cases of patients who came to our care with varying degrees of muscle weakness, each showing interesting and diverse laboratory results.

Conclusion: In patient assessment, it is crucial to consider social and family history. Even without this information, awareness of potential diagnoses is vital. The cause should be carefully considered for possible simple treatments. Failing to recognize and address this condition promptly could lead to severe outcomes. Timely identification and intervention are essential for effective disease management and patient welfare.

简介周期性麻痹是一种导致反复发作的弛缓性麻痹的疾病,可分为原发性和继发性两种。低钾性周期性麻痹是原发性周期性麻痹中最常见的一种,通过常染色体显性基因遗传。男性患者是女性患者的三倍,瘫痪发作通常发生在剧烈运动后的夜间。根据表现性质、体格检查和辅助临床研究排除其他诊断实体至关重要。甲状腺毒症周期性瘫痪在患有甲状腺毒症的亚洲或西班牙裔男性中更为常见,多达10%的甲状腺毒症患者可能会出现周期性瘫痪:在此,我们介绍了6例因不同程度肌无力而就诊的患者,每例患者的实验室检查结果都很有趣且各不相同:在评估病人时,考虑社会和家族病史至关重要。即使没有这些信息,对潜在诊断的认识也至关重要。应仔细考虑病因,以便采取可能的简单治疗方法。如果不能及时发现和处理这种情况,可能会导致严重后果。及时发现和干预对有效的疾病管理和患者福利至关重要。
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引用次数: 0
Suspected Postpartum Depression Revealed to be CSF1R-Related Leukoencephalopathy: A Case Report. 产后抑郁症疑似 CSF1R 相关性白质脑病:病例报告。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-10-02 eCollection Date: 2024-01-01 DOI: 10.1159/000541551
Masahiko Mikuni, Kazuhiro Horiuchi, Ayako Ishikura, Soichiro Kimura, Sho Masutani, Shinya Watanabe, Akihiro Mikami, Shuhei Ishikawa, Hisashi Narita, Ichiro Kusumi, Hidenao Sasaki

Introduction: This is a case of a 32-year-old woman who developed postpartum depression (PPD). She became anxious and depressive about caring for her child, and the Edinburgh Postnatal Depression Scale (EPDS) test showed a score of 9 at 2 weeks after delivery, and at 7 months postpartum, she presented with major melancholic depression followed by mild cognitive decline without any neurological symptoms except cluttering speech.

Case presentation: Cerebral magnetic resonance imaging showed confluent fluid-attenuated inversion recovery hyperintensities in the periventricular and frontal deep white matter, with multiple spotty calcifications in the frontal white matter by cerebral CT. Genetic testing revealed a mutation in the colony-stimulating factor 1 receptor (CSF1R).

Conclusion: This case report is consistent with evidence that PPD may have organic causes in some cases, including CSF1R mutations. Atypical findings such as mild cognitive decline combined with PPD in psychiatric interview may justify brain imaging to avoid misdiagnosis, since CSF1R-related leukoencephalopathy is probably an under-recognized disease in medical psychiatry. Further investigations are needed to clarify a pathophysiological correlation between CSF1R signaling abnormality and PPD as well as major depression.

导言:这是一个 32 岁产妇产后抑郁(PPD)的病例。她因照顾孩子而变得焦虑和抑郁,爱丁堡产后抑郁量表(EPDS)测试显示她在产后2周得了9分,在产后7个月时,她出现了严重的忧郁性抑郁,随后出现了轻度认知功能衰退,除了说话杂乱无章外,没有任何神经系统症状:脑磁共振成像显示,脑室周围和额叶深部白质呈弥漫性液体增强反转恢复高密度,脑CT显示额叶白质多发斑点状钙化。基因检测发现集落刺激因子1受体(CSF1R)发生了突变:本病例报告与某些病例可能有器质性病因(包括 CSF1R 突变)的证据一致。精神科访谈中的非典型发现,如轻度认知功能减退合并 PPD,可能需要进行脑成像检查以避免误诊,因为 CSF1R 相关性白质脑病可能是内科精神病学中一种未得到充分认识的疾病。要明确 CSF1R 信号异常与 PPD 和重度抑郁症之间的病理生理学相关性,还需要进一步的研究。
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引用次数: 0
Spinal Teratoma with Recurrent Epileptic Episodes in Adults: A Case Report. 脊柱畸胎瘤伴成人癫痫反复发作:病例报告。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-18 eCollection Date: 2024-01-01 DOI: 10.1159/000541522
Jinlong Wang, Bo Cao

Introduction: Spinal teratomas are rare, accounting for nearly 0.2-0.5% of all spinal tumors and 2% of all teratomas. Teratomas at the conus medullaris location do not inherently lead to epilepsy. However, potential epileptic seizures are caused when teratoma ruptures and the chemical stimulation of teratoma components enter the dural sac.

Case presentation: A 31-year-old Asian male patient suffering from epileptic onset and poor antiepileptic treatment was demonstrated. The spinal imaging examination was performed, and the patient suffered a space-occupying lesion within the conus medullaris related to spinal deformity, spinal embolism, etc. The autoimmune encephalitis spectrum revealed mGluR5 antibody IgG (+) 1:10 response. The patient stabilized after treatment with hormones and human immunoglobulin. Some hair and lipid droplets could be observed in the dural sac intraoperatively, and more hair and lipid-like material were present in the spinal cord. Postoperative pathology established the diagnosis as a conus medullaris teratoma in adults. Epileptic seizures stopped after surgery, and no additional seizures were reported during the 33-month follow-up period.

Conclusions: Conus medullaris teratoma rupture in adults rarely causes epileptic seizures. For spinal deformity patients with unexplained epileptic symptoms, spinal MRI can be helpful in early diagnosis, and more appropriate treatment improves disease prognosis.

导言:脊柱畸胎瘤非常罕见,约占所有脊柱肿瘤的 0.2%-0.5%,占所有畸胎瘤的 2%。位于圆锥髓部的畸胎瘤本身不会导致癫痫。但是,当畸胎瘤破裂,畸胎瘤成分的化学刺激进入硬膜囊时,就会引起潜在的癫痫发作:病例展示:一名 31 岁的亚洲男性患者因癫痫发病,抗癫痫治疗效果不佳。经脊柱影像学检查,患者髓圆锥内的占位性病变与脊柱畸形、脊柱栓塞等有关。自身免疫性脑炎谱显示 mGluR5 抗体 IgG (+) 1:10 反应。使用激素和人免疫球蛋白治疗后,患者病情稳定。术中可在硬膜囊内观察到一些毛发和脂滴,脊髓内有更多的毛发和类脂物质。术后病理确诊为成人锥髓畸胎瘤。术后癫痫发作停止,在33个月的随访期间没有再出现癫痫发作:结论:成人髓圆锥畸胎瘤破裂很少引起癫痫发作。对于有不明原因癫痫症状的脊柱畸形患者,脊柱磁共振成像有助于早期诊断,更适当的治疗可改善疾病预后。
{"title":"Spinal Teratoma with Recurrent Epileptic Episodes in Adults: A Case Report.","authors":"Jinlong Wang, Bo Cao","doi":"10.1159/000541522","DOIUrl":"https://doi.org/10.1159/000541522","url":null,"abstract":"<p><strong>Introduction: </strong>Spinal teratomas are rare, accounting for nearly 0.2-0.5% of all spinal tumors and 2% of all teratomas. Teratomas at the conus medullaris location do not inherently lead to epilepsy. However, potential epileptic seizures are caused when teratoma ruptures and the chemical stimulation of teratoma components enter the dural sac.</p><p><strong>Case presentation: </strong>A 31-year-old Asian male patient suffering from epileptic onset and poor antiepileptic treatment was demonstrated. The spinal imaging examination was performed, and the patient suffered a space-occupying lesion within the conus medullaris related to spinal deformity, spinal embolism, etc. The autoimmune encephalitis spectrum revealed mGluR5 antibody IgG (+) 1:10 response. The patient stabilized after treatment with hormones and human immunoglobulin. Some hair and lipid droplets could be observed in the dural sac intraoperatively, and more hair and lipid-like material were present in the spinal cord. Postoperative pathology established the diagnosis as a conus medullaris teratoma in adults. Epileptic seizures stopped after surgery, and no additional seizures were reported during the 33-month follow-up period.</p><p><strong>Conclusions: </strong>Conus medullaris teratoma rupture in adults rarely causes epileptic seizures. For spinal deformity patients with unexplained epileptic symptoms, spinal MRI can be helpful in early diagnosis, and more appropriate treatment improves disease prognosis.</p>","PeriodicalId":9639,"journal":{"name":"Case Reports in Neurology","volume":"16 1","pages":"249-255"},"PeriodicalIF":0.6,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521525/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142544015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Head-Up Tilt Sleeping to Treat Orthostatic Intolerance in a Patient with Advanced Parkinson's Disease: A Case Report. 用仰卧睡眠法治疗晚期帕金森病患者的直立性不耐受:病例报告。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-17 eCollection Date: 2024-01-01 DOI: 10.1159/000541424
Amber H van der Stam, Sharon Shmuely, Nienke M de Vries, Roland D Thijs, Mirjam van Kesteren-Biegstraaten, Bastiaan R Bloem

Introduction: Orthostatic hypotension is common in people with Parkinson's disease (PD) due to autonomic dysfunction and medication use and can have a significant negative impact on quality of life. Pharmacological treatment is often complicated due to complex blood pressure regulation problems. This case report presents a patient whose symptoms of orthostatic intolerance were successfully treated with the non-pharmacological method of head-up tilt sleeping (HUTS).

Case presentation: A 69-year-old man with PD and prominent autonomic failure received recommendation from the neurologist to use HUTS to battle orthostatic intolerance, of which complaints were worst in the early morning. The patient noted a marked improvement of the orthostatic intolerance after a period in which he slowly step-by-step inclined the bed to an angle just over 10°. When ceasing HUTS for a brief period, complaints of orthostatic intolerance immediately returned and the patient returned to tilted sleeping right away. After a follow-up of 3 months, the patient did not report orthostatic intolerance during a standing test.

Conclusion: This case illuminates that, despite difficulties intrinsic to this method, whole-body HUTS can ameliorate orthostatic intolerance and improve the daily life of people with advanced movement disorders.

简介:帕金森病(Parkinson's disease,PD)患者由于自主神经功能障碍和药物使用,常会出现直立性低血压,并对生活质量产生严重的负面影响。由于复杂的血压调节问题,药物治疗往往比较复杂。本病例报告介绍了一位患者,他采用非药物治疗方法--抬头仰卧睡眠法(HUTS)成功治疗了正压性不耐受症状:一位 69 岁的男性患者患有帕金森病,自主神经功能明显衰竭,神经科医生建议他使用 HUTS 来对抗正压性不耐受症状,该症状在清晨最为严重。患者注意到,在他一步步缓慢地将床倾斜到刚刚超过 10° 的角度一段时间后,他的直立性不耐受症状得到了明显改善。短暂停止 HUTS 后,患者立即又出现了正压性不耐受的症状,并立即恢复了倾斜睡眠。经过 3 个月的随访,患者在进行站立测试时未再报告正压性不耐受:本病例表明,尽管这种方法本身存在困难,但全身 HUTS 可以改善晚期运动障碍患者的正压性不耐受,并改善其日常生活。
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引用次数: 0
Effective Management of Chronic Intestinal Pseudo-Obstruction in MELAS Using Acotiamide: A Case Report. 使用阿考替酰胺有效治疗 MELAS 的慢性假性肠梗阻:病例报告。
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-09-14 eCollection Date: 2024-01-01 DOI: 10.1159/000541012
Yuri Kawano, Akira Taniguchi, Yugo Narita, Ken Kagawa, Tetsuro Harada, Akihiro Shindo

Introduction: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is reported to be a secondary cause of chronic intestinal pseudo-obstruction (CIPO). Although few case reports have described CIPO in MELAS, effective treatment for CIPO has not been established. Here, we present a case report of amelioration of CIPO symptoms using acotiamide in a patient with MELAS.

Case presentation: A 51-year-old Japanese female with a mitochondrial disorder with m.3243A>G mutation and a history of anorexia for 2 years presented to our hospital with a left temporal headache and acute paraphasia. A stroke-like episode of MELAS was suspected and combined therapy with arginine, edaravone, and levetiracetam was initiated. Although her symptoms improved, she presented with nausea and vomiting and abdominal distension 6 days following admission. Abdominal contrast-enhanced computed tomography revealed dilatation from the stomach to the intestine, particularly marked the stomach, with neither obstruction nor impaired blood flow in the intestine. CIPO exacerbation with MELAS was suspected, and the patient's symptoms gradually improved with acotiamide.

Conclusion: Patients with MELAS could possibly experience stroke-like episodes during CIPO. Since acetylcholine possibly plays an important role in the pathophysiology of CIPO and acotiamide possesses prokinetic activity by inhibiting acetylcholinesterase, acotiamide could possibly improve CIPO symptoms.

导言:据报道,线粒体脑肌病、乳酸酸中毒和中风样发作(MELAS)是慢性肠假性梗阻(CIPO)的继发原因。尽管有少数病例报告描述了 MELAS 中的 CIPO,但 CIPO 的有效治疗方法尚未确立。在此,我们报告了一例使用阿考替酰胺改善 MELAS 患者 CIPO 症状的病例:一名 51 岁的日本女性线粒体疾病患者(m.3243A>G 基因突变)因左颞部头痛和急性肢体瘫痪到我院就诊。患者被怀疑是 MELAS 中风样发作,并开始接受精氨酸、依达拉奉和左乙拉西坦联合治疗。虽然她的症状有所改善,但入院 6 天后又出现恶心、呕吐和腹胀。腹部造影剂增强计算机断层扫描显示,从胃到肠都有扩张,胃部尤为明显,肠道既无梗阻也无血流障碍。患者的症状在服用阿考替胺后逐渐好转:结论:MELAS 患者在 CIPO 期间可能会出现中风样发作。由于乙酰胆碱可能在CIPO的病理生理学中起着重要作用,而阿考替胺通过抑制乙酰胆碱酯酶具有促动力活性,因此阿考替胺有可能改善CIPO症状。
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引用次数: 0
Multiphasic Acute Disseminated Encephalomyelitis with a Temporal Gap of 26 Years: A Case Report with Novel Presentation. 时隔 26 年的多相急性播散性脑脊髓炎:一个表现新颖的病例报告
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-19 eCollection Date: 2024-01-01 DOI: 10.1159/000540951
Muhammad Hamza Asif, Armeen Butt, Khadija Riaz, Faizan Shahzad, Maimoonah Asif, Muhammad Farhan, Waqas Ahmed, Besher Shami

Introduction: Acute disseminated encephalomyelitis (ADEM) is an acute autoimmune demyelinating disease of the central nervous system that typically follows an acute viral infection or post-vaccination. It is more common in children than in adults and is characterized by widespread demyelination of the white matter of the brain and spinal cord. ADEM typically presents as a monophasic illness. Multiphasic ADEM has been an emerging topic with case reports highlighting the importance of the need for further investigation and literature on this topic. Multiphasic ADEM in adults is a rare presentation with an atypical course that can cause a delay in diagnosis leading to disability in patients.

Case presentation: Multiple case reports have shown the presence of multiphasic ADEM without an established cause, emphasizing the importance of additional insight and data in this context. This is a unique case of multiphasic ADEM in a 59-year-old male with a temporal gap of 26 years with an idiopathic cause. He was suffering from many neurologic symptoms involving his upper and lower extremities and level of consciousness, and after he was diagnosed with ADEM, he was commenced on steroids and achieved a marked improvement in his clinical status.

Conclusion: Rarely, ADEM can present with recurrence or multiple phases, which requires prompt diagnosis and treatment. These phases may or may not be preceded by an identifiable trigger. Most patients respond well to steroids. However, further studies are required to achieve a good understanding and guidelines when dealing with such cases.

简介急性播散性脑脊髓炎(ADEM)是中枢神经系统的一种急性自身免疫性脱髓鞘疾病,通常发生在急性病毒感染后或接种疫苗后。它在儿童中的发病率高于成人,特点是大脑和脊髓白质广泛脱髓鞘。ADEM 通常表现为单相性疾病。多相 ADEM 是一个新出现的话题,其病例报告强调了进一步调查和文献研究的必要性。成人多相 ADEM 表现罕见,病程不典型,可能导致诊断延误,导致患者残疾:多份病例报告显示,多相性 ADEM 的存在没有确定的病因,这强调了在此背景下获得更多见解和数据的重要性。这是一例独特的多相 ADEM 病例,患者是一名 59 岁的男性,与特发性病因存在 26 年的时间差。在确诊为 ADEM 后,他开始服用类固醇药物,临床症状得到明显改善:结论:罕见的 ADEM 可出现复发或多个阶段,需要及时诊断和治疗。这些阶段之前可能有可识别的诱因,也可能没有。大多数患者对类固醇反应良好。不过,还需要进一步研究,以便在处理此类病例时获得良好的理解和指导。
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引用次数: 0
Isolated Respiratory Failure as the Presenting Symptom of Muscle-Specific Kinase Myasthenia Gravis: A Case Report and Literature Review. 以孤立性呼吸衰竭为主要症状的肌肉特异性激酶性肌萎缩症:病例报告与文献综述》(Isolated Respiratory Failure as the Presenting Symptom of Muscle-Specific Kinase Myasthenia Gravis: A Case Report and Literature Review.
IF 0.6 Q4 CLINICAL NEUROLOGY Pub Date : 2024-08-14 eCollection Date: 2024-01-01 DOI: 10.1159/000540916
Hassan Doumiati, Ali Ezzeddine

Introduction: Anti-MuSK antibodies obstruct MuSK binding sites, leading to acetylcholine receptor (AChR) displacement within the postsynaptic membrane. MuSK MG patients often exhibit bulbar involvement and respiratory crises, setting them apart from other MG subtypes.

Case presentation: A case of a 51-year-old male with MuSK MG that presented as isolated respiratory failure was compared to similar cases in the literature. The objectives were to explore the varied clinical presentations, treatment approaches, and outcomes, and to better understand the management of this subgroup of MG. The patient responded well to treatment with pyridostigmine, steroids, and intravenous immunoglobulins and immunosuppressive therapy.

Conclusion: A review of the literature revealed varied clinical presentations and treatment approaches among reported cases. Long-term prognosis appears favorable, requiring ongoing immunosuppressive management. Although the reported outcomes of MuSK MG patients with respiratory insufficiency show substantial heterogeneity, long-term prognosis appears favorable.

简介抗MuSK抗体会阻碍MuSK结合位点,导致突触后膜上乙酰胆碱受体(AChR)移位。MuSK MG 患者通常表现为球部受累和呼吸危象,这使他们有别于其他亚型的 MG:我们将一例 51 岁的男性 MuSK MG 患者表现为孤立性呼吸衰竭的病例与文献中的类似病例进行了比较。目的是探讨不同的临床表现、治疗方法和结果,更好地了解这一亚型 MG 的治疗方法。该患者对吡啶斯的明、类固醇、静脉注射免疫球蛋白和免疫抑制治疗反应良好:文献综述显示,已报道病例的临床表现和治疗方法各不相同。长期预后似乎良好,但需要持续的免疫抑制治疗。尽管呼吸功能不全的MuSK MG患者的报道结果显示出很大的异质性,但长期预后似乎良好。
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引用次数: 0
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Case Reports in Neurology
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