Miller-Fisher syndrome

Miller - Fisher 综合征
  • 文章类型: Case Reports
    Miller-Fisher综合征(MFS)的症状是三联症,共济失调,和眼肌麻痹。这种情况是格林-巴利综合征(GBS)的罕见变种,一种急性免疫介导的神经紊乱.这两种情况都涉及异常的自身免疫反应,通常可能由空肠弯曲杆菌等感染引发,人类免疫缺陷病毒,爱泼斯坦-巴尔病毒,和寨卡病毒,在其他人中。因此,免疫系统错误地攻击人体自身的神经组织。MFS的特征是眼瘫,可以进展到完成眼外肌麻痹,可能包括上下垂,面神经麻痹,感觉障碍,肌肉无力。腰椎穿刺支持诊断,显示白蛋白细胞学解离,尽管最初的测试可能并不总是指示性的。MFS的诊断标志物是抗GQ1b抗体的存在,它靶向神经中的GQ1b神经节苷脂,特别影响动眼功能。电诊断研究通常显示不存在或减少的感觉反应而没有降低的传导速度。治疗选择包括静脉注射免疫球蛋白治疗和血浆置换,两者同样有效。该案例研究表明,由于经济限制,接受血浆置换的患者的临床显着改善。强调这种治疗方法的疗效。一名50岁的女性出现肢体感觉异常,进行性上睑下垂,不平衡,和最近发烧后的短暂性复视。检查显示生命体征稳定,深肌腱反射减少,减少振动感觉,小脑共济失调,和颅神经异常。脑脊液分析显示蛋白质升高,建议MFS。正常的磁共振成像和神经传导研究显示GBS,抗GQ1b抗体阳性。经过五次血浆交换循环,1个月后,病人有了很大的改善,出院后没有残留症状。
    The symptoms of Miller-Fisher syndrome (MFS) are a triad of areflexia, ataxia, and ophthalmoplegia. The condition is a rare variant of Guillain-Barré syndrome (GBS), an acute immune-mediated nerve disorder. Both conditions involve abnormal autoimmune responses that may often be triggered by infections such as Campylobacter jejuni, human immunodeficiency virus, Epstein-Barr virus, and Zika virus, among others. As a result, the immune system mistakenly attacks the body\'s own nerve tissues. MFS is characterised by ophthalmoparesis, which can progress to complete external ophthalmoplegia and may include ptosis, facial nerve paralysis, sensory impairments, and muscle weakness. Diagnosis is supported by lumbar puncture, revealing albumin-cytologic dissociation, although initial tests may not always be indicative. A diagnostic marker for MFS is the presence of anti-GQ1b antibodies, which target the GQ1b ganglioside in nerves and affect oculomotor function in particular. Electrodiagnostic studies often show absent or reduced sensory responses without reduced conduction velocity. Treatment options include intravenous immunoglobulin therapy and plasmapheresis, which are both equally effective. This case study demonstrated significant clinical improvement in a patient undergoing plasmapheresis due to financial constraints, highlighting the efficacy of this treatment approach. A 50-year-old female presented with limb paraesthesia, progressive ptosis, imbalance, and transient diplopia following a recent fever. Examination revealed stable vitals, decreased deep tendon reflexes, reduced vibratory sensation, cerebellar ataxia, and cranial nerve abnormalities. Cerebrospinal fluid analysis showed elevated protein, suggesting MFS. Normal magnetic resonance imaging and nerve conduction studies indicated GBS, with positive anti-GQ1b antibodies. After five plasma exchange cycles, the patient improved substantially and was discharged with no residual symptoms after one month.
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  • 文章类型: Case Reports
    格林-巴利综合征/米勒-费希尔综合征(GBS/MFS)重叠综合征是格林-巴利综合征(GBS)的一种极为罕见的变种,其中米勒-费希尔综合征(MFS)与GBS的其他特征共存,比如四肢无力,感觉异常,和面瘫。我们报告了一名12岁患者的临床病例,没有病理史,患有眼肌麻痹的人,无反射,面部瘫痪,吞咽和发声障碍,其次是进步,下降,对称的轻瘫首先影响上肢,然后影响下肢。在脑脊液研究中发现了白蛋白细胞学解离。脊髓的磁共振成像显示马尾神经根的增强和增厚。患者接受免疫球蛋白治疗,临床结果良好。
    Guillain-Barré syndrome/Miller-Fisher syndrome (GBS/MFS) overlap syndrome is an extremely rare variant of Guillain-Barré syndrome (GBS) in which Miller-Fisher syndrome (MFS) coexists with other characteristics of GBS, such as limb weakness, paresthesia, and facial paralysis. We report the clinical case of a 12-year-old patient, with no pathological history, who acutely presents with ophthalmoplegia, areflexia, facial diplegia, and swallowing and phonation disorders, followed by progressive, descending, and symmetrical paresis affecting first the upper limbs and then the lower limbs. An albuminocytological dissociation was found in the cerebrospinal fluid study. Magnetic resonance imaging of the spinal cord showed enhancement and thickening of the cauda equina roots. The patient was treated with immunoglobulins with a favorable clinical outcome.
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  • 文章类型: Case Reports
    Miller-Fisher综合征(MFS)是格林-巴利综合征(GBS)的罕见变体,在地理上具有不同的发病率。MFS主要根据临床特征诊断,比如共济失调和反射障碍,尽管也可能出现其他神经系统症状。已经提出了一个MFS的案例,以共济失调为特征,无反射,双侧脚和手疼痛和吞咽困难。在这方面,采用了儿科康复方法,利用结果衡量标准,比如伊拉斯谟·格林-巴利综合征呼吸功能不全评分儿童,WeeFIM和儿科平衡量表,除了临床评估。值得注意的是,该病例证明了准确诊断和治疗这种罕见的神经系统疾病MFS的重要性。通过实施适当的康复战略,有可能提高患者的生活质量。
    Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS) with varying incidence rates geographically. MFS is primarily diagnosed based on clinical features, such as ataxia and areflexia, although other neurological symptoms may also present. A case of MFS has been presented, characterized by complaints of ataxia, areflexia, bilateral foot and hand pain and difficulty in swallowing. In this regard, a paediatric rehabilitation approach has been adopted, utilizing outcome measures, such as the Erasmus Guillain-Barre Syndrome Respiratory Insufficiency Score-Kids, WeeFIM and paediatric balance scale, in addition to clinical evaluation. It is worth noting that the presented case demonstrates the importance of accurately diagnosing and treating this rare neurological condition MFS. Through the implementation of appropriate rehabilitation strategies, it is possible to enhance patients\' quality of life.
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  • 文章类型: Case Reports
    背景:格林-巴利综合征(GBS),作为全球急性弛缓性麻痹的最常见原因,被认为是临床频谱的一部分,完成,或不完全形式的GBS和重叠综合征取决于其临床特征。当患有MFS的患者也患有四肢进行性运动无力时,使用术语重叠的MillerFisher综合征(MFS)/GBS。抗神经节苷脂GQ1b与MFS和眼肌麻痹特异性相关。
    方法:这里,我们报道了一名中国女孩,她被诊断为重叠的MFS/GBS,显示所有四肢的急性弛缓性麻痹,感觉症状,颅神经功能障碍,自主神经参与,眼肌麻痹,和共济失调.在急性期,她的单特异性抗GM4IgG抗体而不是抗GQ1b抗体的血清和脑脊液滴度很高。
    结论:抗GM4抗体通常与其他抗神经节苷脂抗体共存,导致漏诊。本研究的结果表明,神经节苷脂GM4抗体可能在重叠的MFS/GBS中作为唯一的免疫因子。
    BACKGROUND: Guillain-Barré syndrome (GBS), as the most common cause of acute flaccid paralysis worldwide, is considered a part of a clinical spectrum in which discrete, complete, or incomplete forms of GBS and overlapping syndromes lie on the basis of their clinical features. The term overlapping Miller Fisher syndrome (MFS)/GBS is used when patients with MFS also suffer from progressive motor weakness of the limbs. Anti-ganglioside GQ1b has been specifically associated with MFS and ophthalmoplegia.
    METHODS: Here, we report a Chinese girl who was diagnosed with overlapping MFS/GBS showing acute flaccid paralysis of all four limbs, sensory symptoms, cranial nerve dysfunction, autonomic involvement, ophthalmoplegia, and ataxia. She had high serum and cerebrospinal fluid titres of monospecific anti-GM4 IgG antibody instead of anti-GQ1b antibody in the acute phase.
    CONCLUSIONS: Anti-GM4 antibodies usually coexist with other antiganglioside antibodies, leading to missed diagnoses. The findings of the present study show that antibodies to ganglioside GM4 may in overlapping MFS/GBS as the lone immunological factors.
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  • 文章类型: Case Reports
    在Miller-Fisher综合征(MFS)患者中很少报道Rhinolaliaaperta(鼻超言语)。这里,我们报告了一名MFS患者,他出现了rhinolaliaaperta。一名35岁的有酗酒和肝硬化病史的男子表现为三天的急性鼻音高变化和双手/大腿麻木。入院后,检查还显示腭部健性不足,双侧手/大腿感觉降低,共济失调步态,dysmetria,无反射,和双侧外展麻痹。血清免疫球蛋白G(IgG)抗GQ1b抗体滴度升高(1:6400)。给予5天的静脉内IgG,具有强烈的临床反应。MFS中的口咽受累最初可以表现为孤立的高鼻音语音。
    Rhinolalia aperta (hypernasal speech) is rarely reported in patients with Miller-Fisher syndrome (MFS). Here, we report a patient with MFS who presented with rhinolalia aperta. A 35-year-old man with a history of alcohol abuse and hepatic cirrhosis presented with a three-day acute hypernasal voice change and numbness of both hands/thighs. After admission, the exam also revealed palatal hypomobility, decreased bilateral hand/thigh sensation, ataxic gait, dysmetria, areflexia, and bilateral abducens palsy. Serum immunoglobulin G (IgG) anti-GQ1b antibody titer was elevated (1:6400). A five-day intravenous IgG was administered with a robust clinical response. Oropharyngeal involvement in MFS can initially manifest with isolated hypernasal speech.
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  • 文章类型: Systematic Review
    背景:MillerFisher综合征(MFS)是格林-巴利综合征(GBS)的一种亚型,其特征是眼肌麻痹的三个组成部分,共济失调,和弹性反射。一些研究报告说,MFS是COVID-19疫苗接种的不良反应。我们的目标是对人口统计进行详细的评估,临床,以及接受COVID-19疫苗后患有MFS的受试者的临床特征。
    方法:设计了一个彻底的搜索策略,和PubMed,WebofScience,和Embase进行了搜索以查找相关文章。每个筛选步骤进行两次,如果有分歧,咨询了另一位作者。提取了患者的不同特征和疫苗类型的数据。使用JoannaBriggs研究所(JBI)工具评估研究偏倚的风险。
    结果:在这项研究中,从15个病例研究中确定了15个患者。患者的中位年龄为64岁,范围为24至84岁。10名患者(66.6%)是男性,辉瑞公司占注射疫苗的46.7%。从疫苗接种到症状发作的中位时间为14天,从7到35天不等。此外,14例患者有眼部体征,78.3%(11/14)的眼部表现为双侧。在神经系统疾病中,除了MFS三合会,面部无力或面神经麻痹是最常见的副作用,在7名受试者中(46.7%)。静脉免疫球蛋白(IVIg)是最常用的治疗方法(13/15,86.7%)。六名患者接受0.4g/kg,四名患者接受2g/kg。患者在医院停留了5到51天。没有报告致命的结果。最后,40.0%(4/15)的患者完全恢复,其余的经历了改善。
    结论:COVID-19免疫后MFS预后良好。然而,在一些研究中,从疾病出现到治疗的时间间隔较长,这表明作为疫苗接种的不利影响,应更多地关注MFS。由于具有挑战性的诊断,在最近有COVID-19疫苗接种史和任何眼部疾病的患者的鉴别诊断列表中,必须考虑MFS,共济失调,或者反应能力丧失,特别适合60多岁和70多岁的男性患者。
    BACKGROUND: Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia. Some studies reported MFS as an adverse effect of the COVID-19 vaccination. We aimed to have a detailed evaluation on demographic, clinical, and para-clinical characteristics of subjects with MFS after receiving COVID-19 vaccines.
    METHODS: A thorough search strategy was designed, and PubMed, Web of Science, and Embase were searched to find relevant articles. Each screening step was done by twice, and in case of disagreement, another author was consulted. Data on different characteristics of the patients and types of the vaccines were extracted. The risk of bias of the studies was assessed using Joanna Briggs Institute (JBI) tools.
    RESULTS: In this study, 15 patients were identified from 15 case studies. The median age of the patients was 64, ranging from 24 to 84 years. Ten patients (66.6%) were men and Pfizer made up 46.7% of the injected vaccines. The median time from vaccination to symptoms onset was 14 days and varied from 7 to 35 days. Furthermore,14 patients had ocular signs, and 78.3% (11/14) of ocular manifestations were bilateral. Among neurological conditions, other than MFS triad, facial weakness or facial nerve palsy was the most frequently reported side effect that was in seven (46.7%) subjects. Intravenous immunoglobulin (IVIg) was the most frequently used treatment (13/15, 86.7%). Six patients received 0.4 g/kg and the four had 2 g/kg. Patients stayed at the hospital from five to 51 days. No fatal outcomes were reported. Finally, 40.0% (4/15) of patients completely recovered, and the rest experienced improvement.
    CONCLUSIONS: MFS after COVID-19 immunization has favorable outcomes and good prognosis. However, long interval from disease presentation to treatment in some studies indicates that more attention should be paid to MFS as the adverse effect of the vaccination. Due to the challenging diagnosis, MFS must be considered in list of the differential diagnosis in patients with a history of recent COVID-19 vaccination and any of the ocular complaints, ataxia, or loss of reflexes, specially for male patients in their 60s and 70s.
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  • 文章类型: Case Reports
    文献中已经报道了格林-巴利综合征(GBS)与其变体(包括MillerFisher综合征(MFS))之间的关联并进行了辩论。在这里,我们正在报告一名59岁的男性患者,在表现为快速进行性虚弱之前有10天的流感样症状,吞咽困难,和构音障碍.他的COVID-19检测呈阳性,进一步检查显示抗GQ1b和GQ1d抗体呈阳性。推测了MFS的诊断,并促使开始使用静脉注射免疫球蛋白(IVIG)。呼吸恶化促使插管和拔管失败需要血浆置换。这种治疗最终成功拔管并出院到长期护理机构。该病例增加了目前报告罕见GBS变异与COVID-19感染相关的有限病例。在报告的COVID-19相关MFS病例中,只有少数病例抗GQ1b抗体阳性。这很可能是第一例报告的COVID-19相关MFS,抗GQ1b和抗GQ1d抗体阳性。
    The association between Guillain-Barré Syndrome (GBS) and its variants including Miller Fisher syndrome (MFS) has been reported and debated in the literature. Herein, we are reporting a 59-year-old male patient who had flu-like symptoms for 10 days prior to presentation with rapidly progressive weakness, dysphagia, and dysarthria. He tested positive for COVID-19 and further workup showed positive anti-GQ1b and GQ1d antibodies. The diagnosis of MFS was presumed and prompted the commencement of intravenous immunoglobulin (IVIG). Respiratory deterioration prompted intubation and failure of extubation necessitated plasmapheresis. This treatment culminated in successful extubation and discharge to a long-term care facility. This case adds to the currently limited body of cases that report the association of a rare GBS variant with COVID-19 infection. Only a few of the reported cases of COVID-19-related MFS cases had positive anti-GQ1b antibodies. This may well be the first reported case of COVID-19-related MFS with positive anti-GQ1b and anti-GQ1d antibodies.
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  • 文章类型: Case Reports
    2019年冠状病毒病(COVID-19)诱发的急性炎症性脱髓鞘性多发性神经病(AIDP)中神经性疼痛的特异性文献有限。我们介绍了一例独特的病例,即一名20岁的接种疫苗的女性,既往有慢性乙型肝炎病毒病史,未经治疗的焦虑症,在活动性COVID-19感染的情况下,由于顽固性头痛和水平复视出现在急诊科。在急性住院期间,患者被诊断为格林-巴利综合征(GBS)的Miller-Fisher变异型,一种已知与COVID-19相关的疾病。在ICU,病人发展得很严重,10/10额定,远端,对称性灼痛伴相关异常性疼痛,需要多模式方案联合静脉麻醉药,神经性药物,局部用药,和脱敏训练试图控制她的疼痛。在进行慢性通气康复期间,她咨询了康复心理学,以补充行为疼痛管理策略和持续疼痛的药理学方法。经过几个月的全面住院康复计划,该患者断奶了静脉麻醉药,并开了口服止痛药。这个病人对阿米替林反应最佳,这可能有助于共同治疗COVID-19的心理表现和延长住院时间。这项研究强调了COVID-19诱导的AIDP的致病性,其潜在的严重性,以及多学科方法管理它的重要性。
    There is limited literature specific to neuropathic pain in coronavirus disease 2019 (COVID-19)-induced acute inflammatory demyelinating polyneuropathy (AIDP). We present a unique case of a 20-year-old vaccinated female with a past medical history of chronic hepatitis B virus and untreated anxiety who presented to the emergency department due to an intractable headache and horizontal diplopia in the setting of active COVID-19 infection. During acute hospitalization, the patient was diagnosed with the Miller-Fisher variant of Guillain-Barré syndrome (GBS), a disease with a known association with COVID-19. While in the ICU, the patient developed severe, 10/10-rated, distal, symmetric burning pain with associated allodynia requiring a multimodal regimen with combinations of intravenous narcotics, neuropathic medications, topical agents, and desensitization training to attempt to control her pain. Rehabilitation psychology was consulted while she was in chronic ventilatory rehabilitation for supplementation of behavioral pain management strategies with pharmacological approaches for continued pain. After several months and completion of a comprehensive inpatient rehabilitation program, the patient was weaned off intravenous narcotics and prescribed oral pain medications. This patient had the optimal response to amitriptyline, which likely aided in the co-treatment of psychological manifestations of COVID-19 and prolonged hospitalization. This study highlights the pathogenicity of COVID-19-induced AIDP, its potential severity, and the importance of a multidisciplinary approach to managing it.
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  • 文章类型: Case Reports
    格林-巴利综合征(GBS)是一种罕见的炎性脱髓鞘性多发性神经根神经病,以运动障碍为特征,进步,升序,对称弛缓性肢体麻痹,反射减退或反射减退,不管有没有颅神经受累,这是GBS的标志性临床适应症,可以持续几周到几个月。Miller-Fisher综合征(MFS)是GBS的感染后局部变体,包括眼肌麻痹,共济失调,和弹性反射,通常与下颅和面神经受累有关。在这种情况下,一名22岁的年轻人在10天后被送往医院,主诉双侧对称上肢和下肢瘫痪,腿比胳膊更痛苦。在过去的六天里,他发烧了,含糊不清的讲话,双侧滴眼液,吞咽困难。在检查中,患者被确定为MFS,GBS的变体。在治疗的第一天和最后一天,患者的结果测量记录在手动肌肉测试中,休斯(GBS残疾评分),和功能独立性度量量表。治疗方案已被证明可以减少挑战,改善患者的预后和生活质量。所有这些在这个阶段都很重要。这个案例研究结束了一个康复计划,帮助病人增强他的力量,运动范围,功能移动性,姿势控制,平衡能力,承重,并防止继发性损伤。
    Guillain-Barre syndrome (GBS) is a rare inflammatory demyelinating polyradiculoneuropathy characterized by motor impairment, progressive, ascending, symmetrical flaccid limb paralysis, areflexia or hyporeflexia, and with or without cranial nerve involvement, which are the hallmark clinical indications of GBS, which can last over weeks to months. Miller-Fisher syndrome (MFS) is a post-infectious localized variant of GBS that includes ophthalmoplegia, ataxia, and areflexia, and is often associated with lower cranial and facial nerve involvement. In this case, a 22-year-old young man was taken to a hospital after 10 days with complaints of bilateral symmetrical upper extremity and lower extremity paralysis, with the legs being more afflicted than the arms. For the past six days, he had an episode of fever, slurred speech, bilateral eye drops, and swallowing difficulty. On examination, the patient was identified with MFS, a variant of GBS. On the first and last day of treatment, the patient\'s outcome measures were recorded on Manual Muscle Testing, Hughes (GBS disability score), and the Functional Independence Measure Scale. Treatment options have been shown to reduce challenges and improve patient outcomes and quality of life, all of which are important at this stage. This case study concluded with a rehabilitation program that helped the patient to enhance his strength, range of motion, functional mobility, postural control, balancing abilities, weight-bearing, and prevent secondary impairments.
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  • 文章类型: Case Reports
    米勒-费希尔综合征(MFS)是格林-巴利综合征(GBS)的一种罕见变体,表现为共济失调的三联症,无反射,和眼肌麻痹。随着广泛的2019年冠状病毒病(COVID-19)免疫计划,疫苗接种后GBS或MFS病例的报道越来越多.一名64岁的中国男子表现出四肢新发的感觉异常,双侧绑架限制,右侧面神经麻痹,双侧下肢反射障碍,第二剂COVID-19灭活疫苗接种后12d,左优势肢共济失调。脑脊液分析显示白蛋白细胞学解离,抗GQ1bIgG和抗GT1bIgG阳性。四肢的神经传导研究显示轴索神经病变的证据,感觉幅度降低。根据临床表现,症状的时间进展,和实验室发现,诊断为MFS-GBS重叠综合征.患者接受了静脉注射免疫球蛋白和针灸治疗,并在最初的神经系统症状发作后54d完全康复。据我们所知,我们报告了在COVID-19灭活疫苗接种后出现的首例MFS-GBS重叠综合征.然而,不能排除与这种灭活疫苗的巧合关系。尽管COVID-19疫苗接种的益处在很大程度上大于其风险,MFS的预后总体上是有利的,始终有必要密切监测COVID-19疫苗接种后的神经系统并发症,考虑到它对接种疫苗人群的潜在致残和致命影响。
    Miller-Fisher syndrome (MFS) is a rare variant of Guillain-Barré syndrome (GBS) manifesting as the triad of ataxia, areflexia, and ophthalmoplegia. With the extensive 2019 coronavirus disease (COVID-19) immunization program, cases of GBS or MFS following vaccination are increasingly being reported. A 64-y-old Chinese man presented with new-onset paresthesia of the extremities, bilateral abduction limitation, right facial palsy, areflexia of bilateral lower limbs, and left-dominant limb ataxia 12 d after the second dose of inactivated vaccine against COVID-19. Cerebrospinal fluid analysis indicated albumin-cytological dissociation and was positive for anti-GQ1b IgG and anti-GT1b IgG. Nerve conduction studies of limbs showed evidence of axonal neuropathy with reduced sensory amplitudes. Based on the clinical presentations, temporal progression of symptoms, and laboratory findings, the diagnosis of MFS-GBS overlap syndrome was made. The patient was treated with intravenous immunoglobulin and acupuncture and made a complete recovery 54 d after the onset of his initial neurological signs. To the best of our knowledge, we report the first case of MFS-GBS overlap syndrome following the inactivated COVID-19 vaccination. However, a coincidental relationship with this inactivated vaccine cannot be excluded. Although the benefits of COVID-19 vaccination largely outweigh its risk and the prognosis of MFS is generally favorable, a close surveillance of neurological complications post-COVID-19 vaccination is always necessary, considering its potentially disabling and lethal effects on vaccinated populations.
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