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Amantadine as a Potential Treatment for Marchiafava–Bignami Disease: Case Reports and a Possible Mechanism 金刚烷胺作为一种潜在的治疗Marchiafava-Bignami病的方法:病例报告和可能的机制
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-04-11 DOI: 10.1155/2022/4585206
Leenil C Noel, Martin A. Myers, Tigran Kesayan
Introduction Several reports have described the use of amantadine for managing symptoms in Marchiafava–Bignami disease (MBD); however, amantadine's role for the treatment of MBD symptoms is unclear. Here, we describe 2 patients with MBD who were treated with amantadine and hypothesize a potential mechanism responsible for clinical benefit. Case 1. A 38-year-old woman with excessive wine drinking presented with agitation, impaired speech, and a minimally conscious state. MRI revealed lesions in the splenium and genu. After being diagnosed with MBD, she was treated with intravenous thiamine, multivitamins, and 100 mg of amantadine twice a day for 2 weeks. She recovered to near baseline after 3 weeks. Case 2. A 54-year-old woman with years of heavy alcohol use presented with sudden bradyphrenia, acalculia, disinhibited behavior, weakness, and urinary incontinence. MRI revealed a large anterior callosal lesion. Two years after initial recovery from MBD, she noted that consuming “energy drinks” resulted in a transient, near-complete resolution of her residual behavioral, fatigue, and language symptoms. 100 mg of amantadine twice a day was trialled. After noted improvement, a further escalation to 200 mgs 3 times a day resulted in significant improvement in language and behavioral symptoms. Conclusion Amantadine in addition to vitamins may be beneficial in the treatment of MBD. It is possible that the dopaminergic effect of amantadine leads to improved recovery and function in dopamine-mediated pathways, including mesocortical and mesolimbic pathways during initial recovery, as well as improved speech, behavior, and fatigue in the following months. The role of amantadine in the treatment of MBD warrants further study.
一些报告描述了金刚烷胺用于治疗marchiafawa - bignami病(MBD)的症状;然而,金刚烷胺在治疗MBD症状中的作用尚不清楚。在这里,我们描述了2例MBD患者,他们接受金刚烷胺治疗,并假设了一个潜在的机制,负责临床获益。案例1。一名38岁女性,饮酒过量,表现为躁动、语言障碍和最低限度的意识状态。MRI显示脾和膝病变。在诊断为MBD后,她接受了静脉注射硫胺素、多种维生素和100毫克金刚烷胺的治疗,每天两次,持续2周。3周后恢复至接近基线水平。例2。54岁女性,多年重度饮酒,表现为突发性腹泻、失算、行为失调、虚弱和尿失禁。MRI显示胼胝体前部大病变。在MBD最初康复两年后,她注意到,饮用“能量饮料”导致她残留的行为、疲劳和语言症状短暂、几乎完全消失。100毫克的金刚烷胺,每天两次的试验。在明显改善后,进一步增加至200毫克,每天3次,可显著改善语言和行为症状。结论金刚烷胺加维生素治疗MBD可能有益。金刚烷胺的多巴胺能作用可能导致多巴胺介导通路的恢复和功能改善,包括初始恢复期间的中皮层和中边缘通路,以及随后几个月的语言、行为和疲劳改善。金刚烷胺在MBD治疗中的作用有待进一步研究。
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引用次数: 1
Artery of Percheron Infarction: A Case Report of Bilateral Thalamic Stroke Presenting with Acute Encephalopathy 双侧丘脑卒中并发急性脑病1例
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-30 DOI: 10.1155/2022/8385841
C. Donohoe, Nooshin Kiani Nia, P. Carey, Vamsi Vemulapalli
The artery of Percheron (AOP) is a relatively rare anatomic variant in which a solitary arterial trunk branches from the proximal segment of the posterior cerebral artery and provides arterial supply to the paramedian region of the thalami bilaterally and often to the rostral part of the midbrain. Occlusion of the artery of Percheron results in bilateral paramedian thalamic infarcts with and without midbrain involvement. Recognition of this condition as an acute stroke may be challenging due to various nonlocalized clinical presentations, given the wide range of neurological functions subserved by the thalamus. Prompt neuroimaging, preferably with magnetic resonance imaging (MRI), in conjunction with familiarity with this relatively rare vascular variation can facilitate initiation of appropriate time contingent thrombolytic treatment and improved long-term prognosis. We present a case of a 56-year-old African American female with a bilateral thalamic infarct secondary to the artery of Percheron thromboembolism. This patient presented unresponsive without focal neurologic findings but with an initial Glasgow Coma Score (GCS) of 7, and subsequent computed tomographic (CT) head revealed bilateral thalamic hypodensities. Confirmatory MRI exhibited bilateral subacute thalamic infarcts, which were thought to be embolic with the source from the left ventricular thrombus as the patient had at least three distinct clots. Unfortunately, the patient's mental status did not improve significantly, and she was discharged to a nursing facility for extended care. AOP infarction may be missed on vascular imaging utilizing CT, MRI, and even catheter angiography. Clinical recognition that the AOP is one of the only single artery occlusions that can affect bilateral structures and frequently present solely as altered mental status without focal neurologic deficits is crucial to the diagnosis.
Percheron动脉(AOP)是一种相对罕见的解剖变异,其中一个孤立的动脉干从大脑后动脉近段分支出来,向丘脑的旁脉区提供动脉供应,经常向中脑的吻侧部分提供动脉供应。Percheron动脉闭塞导致双侧丘脑旁脉梗死伴或不伴中脑受累。鉴于丘脑广泛的神经功能,由于各种非局部临床表现,将这种情况识别为急性中风可能具有挑战性。及时的神经成像,最好是磁共振成像(MRI),结合对这种相对罕见的血管变异的熟悉,可以促进开始适当的时间偶发溶栓治疗,改善长期预后。我们提出了一个56岁的非裔美国女性的双侧丘脑梗死继发于Percheron动脉血栓栓塞。该患者无反应性,无局灶性神经病变,但初始格拉斯哥昏迷评分(GCS)为7,随后的头部计算机断层扫描(CT)显示双侧丘脑低密度。证实性MRI显示双侧亚急性丘脑梗死,由于患者至少有三个不同的凝块,因此被认为是栓塞性的,其来源是左心室血栓。不幸的是,患者的精神状态没有明显改善,她被送往护理机构进行延长护理。在CT、MRI甚至导管血管造影中,AOP梗塞可能会被遗漏。临床认识到AOP是唯一一种可以影响双侧结构的单一动脉闭塞,并且经常仅表现为精神状态改变而没有局灶性神经功能缺陷,这对诊断至关重要。
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引用次数: 3
Fatigue and Exercise Intolerance as Initial Manifestations of a Nonsyndromic Mitochondrial Disorder Due to the Variant m.3243A>G 疲劳和运动不耐受是由m.3243A>G变异引起的非综合征性线粒体疾病的初始表现
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-23 DOI: 10.1155/2022/7846852
J. Finsterer, Sinda Zarrouk
Objectives Fatigue and exercise intolerance have been only rarely reported as initial- and sole-onset manifestations of a mitochondrial disorder (MID). We present a patient with nonsyndromic MID with fatigue and exercise intolerance as its initial manifestations of the disease. Case Report. A 39 yo female experienced fatigue since age 18 and exercise intolerance since age 21. Later on, she developed Hashimoto thyroiditis, recurrent diffuse headache, and double vision upon exercise. Clinical exam revealed short stature, bilateral ptosis, partially reduced tendon reflexes, and hypertrophic calves. Serum lactate was elevated, and the lactate stress test was abnormal. Workup for suspected MID revealed ragged-red fibers and NADH-deficient muscle fibers, and biochemical investigations revealed a mild complex-I defect. mtDNA sequencing revealed the variant m.3243A>G with a heteroplasmy rate of 70% in the muscle. Conclusions This case shows that the initial manifestation of a MID can be fatigue and exercise intolerance. MIDs due to the m.3243A>G variant may have a slowly progressive course and only delayed multisystem involvement. The variant m.3243A>G may not only manifest as syndromic MID, particularly MELAS but also as nonsyndromic phenotype. MIDs should be considered as differentials of chronic fatigue even if no other phenotypic manifestation of a MID is present.
疲劳和运动不耐受很少被报道为线粒体疾病(MID)的初始和单发表现。我们提出了一个病人与疲劳和运动不耐受的非综合征性MID作为其疾病的初始表现。病例报告。一名39岁的女性从18岁开始出现疲劳,从21岁开始出现运动不耐受。后来,她出现桥本甲状腺炎、复发性弥漫性头痛和运动时复视。临床检查显示身材矮小,双侧上睑下垂,部分肌腱反射减少,小腿肥大。血清乳酸升高,乳酸应激试验异常。对疑似MID的检查显示红色纤维和nadh缺乏肌纤维,生化检查显示轻度复合物- 1缺陷。mtDNA测序显示m.3243A>G变异在肌肉中的异质性率为70%。结论本病例表明,MID的最初表现可能是疲劳和运动不耐受。由m.3243A>G变型引起的MIDs可能具有缓慢进行性病程,仅延迟多系统受累。变异m.3243A>G不仅可能表现为综合征型MID,特别是MELAS,也可能表现为非综合征型表型。即使没有其他表型表现,MIDs也应被视为慢性疲劳的鉴别。
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引用次数: 0
Early and Delayed Rebound Intracranial Hypertension following Epidural Blood Patch in a Case of Spontaneous Intracranial Hypotension 自发性颅内低血压1例硬膜外补血后早期和延迟反弹性颅内高压
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-18 DOI: 10.1155/2022/5637276
E. Jafari, M. Karaminia, M. Togha
Background Spontaneous intracranial hypotension (SIH) is a secondary headache that has been attributed to a cerebrospinal fluid (CSF) leak. It may resolve spontaneously or require conservative treatment. An epidural blood patch (EBP) with autologous blood is performed in cases exhibiting an inadequate response to conservative methods. Rebound intracranial hypertension (RIH) can develop following an EBP in up to 27% of patients. It is characterized by a change in the headache features and is often accompanied by nausea, blurred vision, and diplopia. Symptoms commonly begin within the first 36 hours, but could develop over days to weeks. It is important to differentiate this rebound phenomenon from unimproved SIH, as the treatment options differ. Case Presentation. Here, we present an interesting case of a patient with SIH who was treated with EBP and developed both immediate RIH after 24 hours and delayed RIH 3 weeks following EBP. Conclusions Following EBP for treatment of SIH, new onset of headache having a different pattern and location should always be monitored for the occurrence of RIH. A lumbar puncture should be done if the symptoms of elevated CSF pressure become intolerable or if the diagnosis is uncertain. Lack of early diagnosis and treatment and differentiation from SIH can cause complications and could affect the optic nerves.
自发性颅内低血压(SIH)是一种继发性头痛,已归因于脑脊液(CSF)泄漏。它可以自行消退或需要保守治疗。硬膜外血贴片(EBP)与自体血液进行的情况下,表现出不充分的反应保守方法。反弹性颅内高压(RIH)可在高达27%的患者发生EBP后发生。其特点是头痛特征的改变,并常伴有恶心、视力模糊和复视。症状通常在最初的36小时内开始,但可能在几天到几周内发展。重要的是要区分这种反弹现象和未改善的SIH,因为治疗方案不同。案例演示。在这里,我们报告了一个有趣的病例,SIH患者接受EBP治疗,在EBP后24小时发生立即RIH,并在EBP后3周发生延迟RIH。结论EBP治疗SIH后,新发不同类型和部位的头痛应始终监测RIH的发生。如果脑脊液压力升高的症状变得无法忍受或诊断不确定,则应进行腰椎穿刺。缺乏早期诊断、治疗和鉴别可引起并发症,并可能影响视神经。
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引用次数: 4
Demyelinating Neurological Adverse Events following the Use of Anti-TNF-α Agents: A Double-Edged Sword 使用抗tnf -α药物后脱髓鞘神经系统不良事件:一把双刃剑
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-03-07 DOI: 10.1155/2022/3784938
Miral H Gharib, M. AlKahlout, Beatriz Garcia Canibano, Dirk Theophiel Deleu, Hani Malallah AlEssa, S. AlEmadi
Background Tumor necrosis factor antagonists (anti-TNF-α) are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported, and literature data suggest a potential role of anti-TNF-α in the induction of demyelination. Case Presentation. In this series, we present three cases of demyelination after the use of anti-TNF-α agents. The first case involved a 21-year-old man with HLA-B27 negative peripheral spondylarthritis who had been taking adalimumab for 2 years. He developed headache, urinary incontinence, and bilateral lower extremity numbness that progressed to the middle of the trunk for 2 days. Magnetic resonance imaging (MRI) showed multiple hyperintense enhancement lesions in the left paramedian anterior pons consistent with multiple sclerosis (MS). The second case included a 17-year-old woman who was on 2 years of adalimumab treatment for juvenile idiopathic arthritis and chronic anterior uveitis and developed new-onset dizziness and tremors. The clinical examination showed signs of cerebellar dysfunction. MRI findings were consistent with multiple sclerosis. The third case was a 34-year-old male who was on 5 years of infliximab treatment for ankylosing spondylitis when he developed left hand numbness and weakness. Cerebrospinal fluid (CSF) analysis and MRI findings were consistent with demyelination. Discontinuation of tumor necrosis factor antagonists (anti-TNF-α) resulted in resolution of the symptoms with no recurrence in the first case, but there was evidence of recurrence in the other 2 cases, where one was managed with rituximab and the second one improved with pulse steroid therapy. Conclusion Despite the small number of patients, our series adds to the growing body of evidence supporting a causal link between anti-TNF-α agents and demyelination. Thus, we can conclude that on suspicion of any neurological side effects, early discontinuation of the TNF-α blockers and requesting urgent MRI scan to confirm the diagnosis is of utmost importance.
肿瘤坏死因子拮抗剂(抗tnf -α)是几种自身免疫性和炎症性肠病的既定治疗选择。尽管它们的临床有效性,神经系统不良事件已被报道,文献数据表明抗tnf -α在诱导脱髓鞘中的潜在作用。案例演示。在这个系列中,我们提出了使用抗tnf -α药物后脱髓鞘的三个病例。第一个病例涉及一名患有HLA-B27阴性周围性脊柱炎的21岁男性,他已经服用阿达木单抗2年。患者出现头痛、尿失禁和双侧下肢麻木,并进展至躯干中部,持续2天。磁共振成像(MRI)显示左侧桥前旁位多发高强度强化病灶,符合多发性硬化症(MS)。第二个病例包括一名17岁的女性,她因青少年特发性关节炎和慢性前葡萄膜炎接受了2年的阿达木单抗治疗,并出现了新发头晕和震颤。临床检查显示有小脑功能障碍的迹象。MRI表现符合多发性硬化症。第三例为34岁男性,因强直性脊柱炎接受英夫利昔单抗治疗5年后出现左手麻木和无力。脑脊液(CSF)分析和MRI结果与脱髓鞘一致。停止使用肿瘤坏死因子拮抗剂(抗tnf -α)导致第一例症状缓解,无复发,但其他2例有复发迹象,其中1例使用利妥昔单抗治疗,另1例使用脉冲类固醇治疗改善。结论:尽管患者数量较少,但我们的系列研究增加了越来越多的证据,支持抗tnf -α药物与脱髓鞘之间的因果关系。因此,我们可以得出结论,在怀疑任何神经系统副作用时,早期停用TNF-α阻滞剂并要求紧急MRI扫描以确认诊断是至关重要的。
{"title":"Demyelinating Neurological Adverse Events following the Use of Anti-TNF-α Agents: A Double-Edged Sword","authors":"Miral H Gharib, M. AlKahlout, Beatriz Garcia Canibano, Dirk Theophiel Deleu, Hani Malallah AlEssa, S. AlEmadi","doi":"10.1155/2022/3784938","DOIUrl":"https://doi.org/10.1155/2022/3784938","url":null,"abstract":"Background Tumor necrosis factor antagonists (anti-TNF-α) are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported, and literature data suggest a potential role of anti-TNF-α in the induction of demyelination. Case Presentation. In this series, we present three cases of demyelination after the use of anti-TNF-α agents. The first case involved a 21-year-old man with HLA-B27 negative peripheral spondylarthritis who had been taking adalimumab for 2 years. He developed headache, urinary incontinence, and bilateral lower extremity numbness that progressed to the middle of the trunk for 2 days. Magnetic resonance imaging (MRI) showed multiple hyperintense enhancement lesions in the left paramedian anterior pons consistent with multiple sclerosis (MS). The second case included a 17-year-old woman who was on 2 years of adalimumab treatment for juvenile idiopathic arthritis and chronic anterior uveitis and developed new-onset dizziness and tremors. The clinical examination showed signs of cerebellar dysfunction. MRI findings were consistent with multiple sclerosis. The third case was a 34-year-old male who was on 5 years of infliximab treatment for ankylosing spondylitis when he developed left hand numbness and weakness. Cerebrospinal fluid (CSF) analysis and MRI findings were consistent with demyelination. Discontinuation of tumor necrosis factor antagonists (anti-TNF-α) resulted in resolution of the symptoms with no recurrence in the first case, but there was evidence of recurrence in the other 2 cases, where one was managed with rituximab and the second one improved with pulse steroid therapy. Conclusion Despite the small number of patients, our series adds to the growing body of evidence supporting a causal link between anti-TNF-α agents and demyelination. Thus, we can conclude that on suspicion of any neurological side effects, early discontinuation of the TNF-α blockers and requesting urgent MRI scan to confirm the diagnosis is of utmost importance.","PeriodicalId":9615,"journal":{"name":"Case Reports in Neurological Medicine","volume":"1205 ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2022-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72494887","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Magnetic Resonance-Guided Diagnosis of Spontaneous Intracranial Hypotension in a Middle-Aged Woman 磁共振引导诊断中年妇女自发性颅内低血压
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-02-21 DOI: 10.1155/2022/4438923
Jordan Hughes, Briana Chavez
Spontaneous intracranial hypotension (SIH) is a rare condition caused by a cerebrospinal fluid (CSF) leak. It is diagnosed by clinical features that include an orthostatic headache combined with imaging findings demonstrating intracranial hypotension and a CSF leak. We present the case of a 45-year-old woman with an orthostatic headache who was found to have a sagging brain with a downward-displaced cerebellum and pachymeningeal enhancement with gadolinium contrast. This was initially misidentified as a Chiari I malformation, but the constellation of symptoms and MRI findings were later recognized as characteristic of SIH. Diagnosis of SIH and a CSF leak was confirmed with CT myelography. She was treated with a nontarget epidural blood patch, and her symptoms resolved. An orthostatic headache, a sagging brain, and pachymeningeal enhancement on MRI are highly specific for SIH, raising suspicion for this uncommon and often missed diagnosis.
自发性颅内低血压(SIH)是一种罕见的条件,引起脑脊液(CSF)泄漏。临床表现包括直立性头痛,影像学表现为颅内低血压和脑脊液泄漏。我们提出的情况下,45岁的妇女直立性头痛谁被发现有一个下垂的大脑与一个向下移位的小脑和厚脑膜增强与钆造影剂。这最初被误诊为Chiari I型畸形,但一系列症状和MRI结果后来被认为是SIH的特征。CT脊髓造影证实了SIH和脑脊液泄漏的诊断。她接受了非靶向硬膜外血液贴片治疗,症状消失。直立性头痛,脑下垂和MRI上的厚脑膜增强是SIH的高度特异性,引起了对这种罕见且经常被遗漏的诊断的怀疑。
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引用次数: 1
A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy 视神经脊髓炎1例:波多黎各妇女诊断滞后时间增加,对埃曲利珠单抗治疗反应高
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-02-18 DOI: 10.1155/2022/4311382
Ramón Vega, Benjamín González, Kiara Ortiz, Viviana Martínez, D. Carmona, Ivonne Vicente, Javier Chapa, Á. Chinea
A link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are essential to prevent disease progression. We report the case of a 46-year-old Puerto Rican female who presented intractable hiccups when she was 31 (in 2004). Almost 15 years passed since the initial symptom, and after two severe relapses, she received a formal NMOSD diagnosis in March 2019. Treatment started with rituximab 1000 mg IV in April 2019. However, a lack of response to treatment led to a switch to eculizumab therapy in August 2019. The patient had cervical and brain magnetic resonance imaging (MRI) conducted in June 2020, which depicted a remarkable decrease in swelling and hyperintensity within the cervical spinal cord with no enhancing lesions when compared with the first MRI from February 2019. In addition, the patient suffered no new relapses, an improvement regarding disability, and a reduction of the cervical spinal cord lesion size. Nonetheless, this substantial decrease does not occur on all NMOSD patients, but more awareness of the disease is needed, especially in Puerto Rico. This case illustrates the efficacy of eculizumab therapy and the importance of differentiating the clinical, histopathological, and neuroimaging characteristics that separate demyelinating autoimmune inflammatory disorders, such as NMOSD and multiple sclerosis (MS).
作为初始症状的顽固性打嗝与可能的视神经脊髓炎谱系障碍(NMOSD)诊断之间的联系令人困惑,但至关重要,如果卫生保健提供者(HCPs)不了解2015年NMOSD标准,他们可能不会做出诊断。早期诊断和适当治疗对于预防疾病进展至关重要。我们报告一位46岁的波多黎各女性,她在31岁时(2004年)出现难治性打嗝。自最初症状出现以来已过去了近15年,在两次严重复发后,她于2019年3月接受了正式的NMOSD诊断。2019年4月,利妥昔单抗1000mg IV开始治疗。然而,对治疗缺乏反应导致2019年8月改用eculizumab治疗。患者于2020年6月进行了颈椎和脑磁共振成像(MRI),与2019年2月的第一次MRI相比,颈脊髓肿胀和高强度明显减少,无强化病变。此外,患者没有出现新的复发,残疾得到改善,颈脊髓病变面积减小。尽管如此,并不是所有NMOSD患者都出现这种显著减少,但需要提高对这种疾病的认识,特别是在波多黎各。该病例说明了eculizumab治疗的疗效,以及区分脱髓鞘自身免疫性炎症性疾病(如NMOSD和多发性硬化症(MS))的临床、组织病理学和神经影像学特征的重要性。
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引用次数: 0
Diabetic Striatopathy: Case Report and Possible New Actors. 糖尿病纹状体病:病例报告和可能的新参与者。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-01-01 DOI: 10.1155/2022/4176419
Chiara Mozzini, Raffaele Ghirardi, Mauro Pagani

Diabetic striatopathy is a very rare neurological complication of diabetes. We report the case of an 86-year-old woman with poorly controlled type 2 diabetes admitted to the internal medicine ward for sudden onset of altered sensorium and severe bilateral choreiform and ballistic movements. The precise pathophysiology of this condition is not well understood. Our communication aims to remind clinicians to consider the possibility of diabetic striatopathy when poor-controlled diabetic patients have sudden-onset choreiform and ballistic movements. Moreover, this case suggests the possibility that oxidative and endoplasmic reticulum stress may be involved in this process.

糖尿病纹状体病是一种非常罕见的糖尿病神经系统并发症。我们报告一例86岁的2型糖尿病患者,因突然发生感觉改变和严重的双侧舞蹈症和弹道运动而住进内科病房。这种情况的确切病理生理机制尚不清楚。我们的交流的目的是提醒临床医生考虑糖尿病纹状病的可能性,当控制不良的糖尿病患者突然出现舞状和弹道运动。此外,该病例提示氧化应激和内质网应激可能参与了这一过程。
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引用次数: 0
Ipsilateral Limb Extension of Referred Trigeminal Facial Pain due to Greater Occipital Nerve Entrapment: A Case Report. 枕大神经压迫致三叉神经面痛的同侧肢体延伸一例。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2022-01-01 DOI: 10.1155/2022/9381881
Byung-Chul Son, Changik Lee

We report a very rare case of referred pain associated with entrapment of the greater occipital nerve (GON) occurring not only in the ipsilateral hemiface but also in the ipsilateral limb. There is an extensive convergence of cutaneous, tooth pulp, visceral, neck, and muscle afferents onto nociceptive and nonnociceptive neurons in the trigeminal nucleus caudalis (medullary dorsal horn). In addition, nociceptive input from trigeminal, meningeal afferents projects into trigeminal nucleus caudalis and dorsal horn of C1 and C2. Together, they form a functional unit, the trigeminocervical complex (TCC). The nociceptive inflow from suboccipital and high cervical structures is mediated with small-diameter afferent fibers in the upper cervical roots terminating in the dorsal horn of the cervical cord extending from the C2 segment up to the medullary dorsal horn. The major afferent contribution is mediated by the spinal root C2 that is peripherally represented by the greater occipital nerve (GON). Convergence of afferent signals from the trigeminal nerve and the GON onto the TCC is regarded as an anatomical basis of pain referral in craniofacial pain and primary headache syndrome. Ipsilateral limb pain occurs long before the onset of the referred facial pain. The subsequent severe hemifacial pain suggested GON entrapment. The occipital nerve block provided temporary relief from facial and extremity pain. Imaging studies found a benign osteoma in the ipsilateral suboccipital bone, but no direct contact with GON was identified. During GON decompression, severe entrapment of the GON was observed by the tendinous aponeurotic edge of the trapezius muscle, but the osteoma had no contact with the nerve. Following GON decompression, the referred trigeminal and extremity pain completely disappeared. The pain referral from GON entrapment seems to be attributed to the sensitization and hypersensitivity of the trigeminocervical complex (TCC). The clinical manifestations of TCC hypersensitivity induced by chronic entrapment of GONs are diverse when considering the occurrence of extremity pain as well as facial pain.

我们报告了一个非常罕见的病例牵涉到疼痛与大枕神经卡压(GON)不仅发生在同侧半面,而且发生在同侧肢体。皮肤、牙髓、内脏、颈部和肌肉传入神经广泛汇聚到三叉神经尾核(髓质背角)的伤害性和非伤害性神经元。此外,来自三叉神经、脑膜传入的伤害性输入投射到三叉神经尾核和C1、C2背角。它们一起构成了一个功能单元,三叉神经复合体(TCC)。枕下和高颈结构的痛觉流入由颈上根的小直径传入纤维介导,止于颈髓背角,从C2段向上延伸至髓背角。主要的传入神经是由脊椎根C2介导的,其周围以枕大神经(GON)为代表。来自三叉神经和根神经的传入信号收敛到TCC被认为是颅面疼痛和原发性头痛综合征疼痛转诊的解剖学基础。同侧肢体疼痛早于面部疼痛发作。随后出现的严重的半面部疼痛提示神经根夹持。枕神经阻滞可暂时缓解面部和四肢疼痛。影像学检查发现同侧枕下骨有一良性骨瘤,但未发现与骨毒素直接接触。在GON减压过程中,斜方肌腱膜边缘观察到严重的GON压迫,但骨瘤未与神经接触。GON减压后,三叉及四肢疼痛完全消失。神经根压陷引起的疼痛似乎归因于三叉神经颈复合体(TCC)的致敏和超敏。考虑到肢体疼痛和面部疼痛的发生,慢性GONs卡压致TCC超敏反应的临床表现是多种多样的。
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引用次数: 2
Optic Neuritis Presented as Syndrome of Inappropriate Antidiuretic Hormone Secretion in an 8 Year Old. 视神经炎表现为8岁儿童抗利尿激素分泌不当综合征。
IF 0.9 Q4 CLINICAL NEUROLOGY Pub Date : 2021-01-01 DOI: 10.1155/2021/6672827
T G M Prasadani, Kapila Panditha, D Irugalbandara

Optic neuritis is a rare demyelinating disorder, which involves the optic nerve. It can be a monophasic self-limiting illness due to postinfectious or postvaccination etiology. It can also be an initial presentation of a relapsing demyelinating disorder such as multiple sclerosis or neuromyelitis optica spectrum of disorders. It is characterized to aquaporin-4 antibody-rich areas in the brain, optic nerve, and spinal cord. The hypothalamus and periventricular area are also rich in specific antibodies and may lead to dysfunction in the hypothalamic-pituitary axis. Antidiuretic hormone (ADH) is synthesized in the hypothalamus and stored in the posterior pituitary and may secrete inappropriately due to this disturbance. This will impair water excretion from the kidney, leading to hyponatremia. When hyponatremia is significant, the patient will present with confusion, agitation, and convulsions. This case report discusses acute symptomatic hyponatremia as the initial presentation of optic neuritis due to syndrome of inappropriate ADH secretion (SIADH).

视神经炎是一种罕见的脱髓鞘疾病,累及视神经。它可以是一种单相自限性疾病,由于感染后或接种后的病因。它也可能是复发性脱髓鞘疾病的初始表现,如多发性硬化症或视神经脊髓炎。它的特点是在大脑、视神经和脊髓中富含水通道蛋白-4抗体的区域。下丘脑和脑室周围区域也富含特异性抗体,可能导致下丘脑-垂体轴功能障碍。抗利尿激素(ADH)在下丘脑合成并储存在垂体后叶,并可能由于这种干扰而分泌不当。这会损害肾脏的水分排泄,导致低钠血症。当低钠血症严重时,患者会出现精神错乱、躁动和抽搐。本病例报告讨论急性症状性低钠血症作为视神经炎的最初表现,由于不适当的ADH分泌综合征(SIADH)。
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Case Reports in Neurological Medicine
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