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Guillain–Barré syndrome (GBS) is a serious neurological condition with limited treatment options. A recent report demonstrated successful treatment with efgartigimod alone in two patients with GBS, although it did not significantly shorten the disease duration. This case series investigates the effects of sequential efgartigimod administration in patients with different GBS phenotypes and varying levels of disease severity. All three patients tested positive for immunoglobulin G (IgG) antibodies against serum gangliosides. In Case 1, the patient was treated with 0.4 g/kg of intravenous immunoglobulin (IVIg) for 5 days, showing minimal recovery. After receiving 3 weekly doses of efgartigimod (10 mg/kg), the patient achieved independent ambulation 19 days post-onset, with a reduction in serum ganglioside antibody titers and total IgG levels. Case 2 involved a middle-aged man with Miller Fisher syndrome (MFS)-GBS overlap, who experienced worsened autonomic dysfunction following IVIg treatment. After three doses of efgartigimod, the patient showed symptom improvement within 1 month, alongside a reduction in IgG antibody levels. In Case 3, a 27-year-old male with MFS-GBS overlap, initially unresponsive to IVIg, showed significant improvement in ophthalmoplegia following two doses of efgartigimod, with his serum ganglioside antibodies eventually becoming undetectable. Our findings suggest that sequential efgartigimod treatment may effectively reduce serum anti-ganglioside antibody titers and potentially shorten the disease course in severe GBS and MFS-GBS overlap syndrome. Additionally, it may offer clinical benefits for patients with GBS who have a poor or no response to IVIg, particularly in treating ophthalmoplegia. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) is a complex autoimmune disorder characterized by acute onset of motor weakness, often following an infectious illness. The pathophysiology of GBS involves a multifaceted interplay between immune mechanisms and environmental factors, leading to demyelination or axonal degeneration. This systematic review aims to elucidate the autoimmune mechanisms underlying the various subtypes of GBS, including acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and Miller Fisher syndrome (MFS). A thorough literature search identified 71 studies published between 2010 and 2024 that provided insights into the immunopathological features, clinical implications, and future directions for research. Key findings indicate that specific autoantibodies, such as anti-GM1 and anti-GQ1b, are associated with distinct subtypes of GBS, contributing to the disease's heterogeneity. Understanding these autoimmune mechanisms is crucial for improving diagnostic accuracy, therapeutic strategies, and prognostic indicators in GBS. This review highlights significant gaps in current research, emphasizing the need for further studies to explore the genetic and environmental factors that influence GBS susceptibility and the role of vaccinations in triggering autoimmune responses. Trial registration: PROSPERO CRD42024606718. [ABSTRACT FROM AUTHOR]

The article reviews the expanding clinical spectrum of anti-GQ1b antibody syndrome, emphasizing its association with various autoimmune peripheral neuropathies, particularly highlighting Miller Fisher syndrome (MFS). Topics discussed include the overlap of anti-GQ1b syndrome with conditions like Guillain-Barré syndrome, atypical presentations without ophthalmoplegia, and the role of intravenous immunoglobulin (IVIG) in treatment.

Neuromuscular ultrasound has emerged as a beneficial, complementary tool to electromyography (EMG) in the diagnosis of neuromuscular diseases as it provides high-resolution anatomic imaging of peripheral nerves and muscles. It has been used previously as an adjunct to EMG to diagnose Charcot-Marie-Tooth disease. Here, we present a case of a 64-yr-old man with bilateral sensorineural hearing loss of 14 yrs who presented to an outpatient neuromuscular clinic at a tertiary medical center with very slow progressive paresthesias. This case highlights the application of neuromuscular ultrasound to help confirm the presence of a likely Charcot-Marie-Tooth disease type 4C despite indeterminate genetic testing results and challenging EMG results. He had genetic testing which revealed a normal PMP22 gene; however, he had a variant of uncertain significance in the SH3TC2 gene which has shown associations with autosomal recessive CMT4C. Neuromuscular ultrasound revealed mild median and significant tibial nerve uniform enlargement throughout their course. There may also be utility in performing neuromuscular ultrasound on similarly affected family members if the same variant of uncertain significance returns for SH3TC2, with consideration of both peripheral nerve and nerve root assessment. [ABSTRACT FROM AUTHOR]

Immune-mediated cerebellar ataxias (IMCAs) represent a group of disorders in which the immune system targets mainly the cerebellum and related structures. We address fundamental questions on the diagnosis and immunological pathogenesis of IMCAs, as illuminated by recent advances in the field. Various types of IMCAs have been identified, including post-infectious cerebellitis, Miller Fisher syndrome, gluten ataxia, paraneoplastic cerebellar degeneration (PCD), opsoclonus and myoclonus syndrome, and anti-GAD ataxia. In some cases, identification of several well-characterized autoantibodies points to a specific etiology in IMCAs and leads to a firm diagnosis. In other cases, various autoantibodies have been reported, but their interpretation requires a careful consideration. Indeed, some autoantibodies have only been documented in a limited number of cases and the causal relationship is not established. In order to facilitate an early treatment and prevent irreversible lesions, new entities have been defined in recent years, such as primary autoimmune cerebellar ataxia (PACA) and latent autoimmune cerebellar ataxia (LACA). PACA is characterized by autoimmune features which do not align with traditional etiologies, while LACA corresponds to a prodromal stage. LACA does not imply the initiation of an immunotherapy but requires a close follow-up. Concurrently, accumulation of clinical data has led to intriguing hypotheses regarding the mechanisms of autoimmunity, such as a pathogenesis of autoimmunity against synapses (synaptopathies), and the vulnerability of the entire nervous system when the immunity targets ion channels and astrocytes. The development of PCD in patients treated with immune-checkpoint inhibitors suggests that molecular mimicry specifically determines the direction of autoimmunity, and that the strength of this response is modulated by co-signaling molecules that either enhance or dampen signals from the antigen-specific T cell receptor. [ABSTRACT FROM AUTHOR]

Copyright of Acta Neurológica Colombiana is the property of Colombian Association of Neurology / Asociacion Colombiana de Neurologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Foco (Interdisciplinary Studies Journal) is the property of Revista Foco and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We report a case of a 6-year-old boy with autism spectrum disorder presenting with new-onset squint and 'ptosis' following a recent infection. Clinical examination revealed ataxia and areflexia alongside a dilated pupil poorly reactive to light. Subsequently, his eye movements deteriorated to near-complete ophthalmoplegia at 1-week review. Further investigations inclusive of a magnetic resonance imaging (MRI) brain scan, a computed tomography (CT) venogram and a lumbar puncture were conducted to consider and rule out differential diagnoses. Cerebrospinal fluid analysis revealed an albuminocytologic dissociation. The clinical triad of progressive ophthalmoplegia, areflexia and areflexia alongside albuminocytologic dissociation led to the diagnosis of Miller Fisher syndrome. The patient was commenced on intravenous immunoglobulin and his symptoms showed significant improvement. We use this interesting case to provide context for key learning points about diagnosing Miller Fisher syndrome in children. [ABSTRACT FROM AUTHOR]

The article in the Current Journal of Neurology discusses a rare case of polyneuritis cranialis in a 9-year-old boy, presenting with ocular, speech, and swallowing problems. The boy had lower motor neuron facial palsy involving multiple cranial nerves, with symptoms appearing after a flu-like illness and bloody diarrhea. The patient responded well to intravenous immunoglobulin treatment, showing full recovery. The article highlights the importance of considering post-infectious cranial polyneuropathy in cases of multiple cranial nerve involvement, emphasizing the need for early diagnosis and intervention for optimal outcomes. [Extracted from the article]

Background: Anti-GQ1B syndrome includes a group of diseases characterized by antibody-mediated polyneuropathy. Guillain Barre syndrome (GBS) and the Miller-Fisher syndrome (MFS) have been related to COVID-19 vaccine application. Clinic case: 48-year-old man, with history of Pfizer-BioNTech vaccination against COVID-19, 5 days prior to the symptoms, who assisted to the Emergency room with blurred vision and diplopia; adding dysarthria, facial diplegia and left upper limb weakness after 48 hours. In his first evaluation it was found ophthalmoplegia, facial diplegia, decreased gag reflex, weakness of thoracic limbs, bilateral trapezius muscle and areflexia. Serum studies and nuclear magnetic resonance of the brain were performed without alterations. It was complemented with IgG anti-ganglioside GQ1b antibodies with a positive result. Once the diagnosis was confirmed, treatment was started with immunoglobulin calculated at 2 g per kg for 5 days. The patient was discharged once the immunoglobulin was administered with evaluation at 2 months without ataxia, ophthalmoplegia, areflexia and weakness. Conclusions: Following the documented reports of GBS and its variants secondary to vaccination, neurological side effects have been catalogued as being of great importance. Therefore, the reported case can be used as a point of reference to consider this clinical spectrum as a differential diagnosis in patients with post-vaccination neurological symptomatology. [ABSTRACT FROM AUTHOR]

Introduction: Vessel Wall Imaging (VWI) is a relatively novel magnetic resonance imaging (MRI) technique primarily aimed at diagnosing vascular pathologies. In this study, we aimed to evaluate the diagnostic value of contrast-enhanced (CE) VWI in identifying various intracranial non-vascular pathologies. Methods: This retrospective study was approved by our institutional ethics committee with approval number 2022-05/17 on March 11, 2022. We retrospectively evaluated cranial CE-MRI, including the VWI sequence, of 189 patients (76 females and 113 males) who were referred to our radiology department for brain imaging for various reasons. MRI examinations were performed using a 3 Tesla unit. A single observer evaluated anonymized cranial MRI images without CE-VWI in addition to the relevant clinical information in a random order. The same observer interpreted the CE-VWI with relevant clinical information six weeks later. The findings, which could only be visualized on VWI in the second session, were noted. Results: In 10 patients of our study cohort (5.3%), VWI demonstrated pathological signal alterations or contrast enhancement (e.g., post-status frontal lobe pial enhancement in a patient with autoimmune epilepsy, contrast enhancement in the hippocampus in a diffusion-negative hyper-acute ischemic stroke patient, and optic disc enhancement in a patient with intracranial hypertension) that apparently reflected underlying clinical disorders, which otherwise could not be visualized on conventional MRI. Discussion and Conclusion: CE-VWI might serve as a valuable adjunct for the diagnosis of various parenchymal or meningeal intracranial diseases, yet further, more comprehensive studies are needed to reveal the true potential of VWI. [ABSTRACT FROM AUTHOR]

Miller Fisher Syndrome (MFS) is a rare variant of Guillain-Barré Syndrome (GBS), characterised by a unique clinical triad of ophthalmoplegia, ataxia, and areflexia. MFS, which was first described in 1956 by Charles Miller Fisher , is frequently preceded by an infection, usually respiratory or gastrointestinal, and is believed to be triggered by an autoimmune reaction targeting peripheral nervous system components. However the MFS is further categorised into incomplete forms which can be present without the classical triad, such as acute ophthalmoparesis, acute ataxic neuropathy, acute ptosis and acute mydriasis. We report a case of a 50-year-old man presenting with dilated and unresponsive pupils, which progressed rapidly to ophthalmoplegia, ataxia, and areflexia. Given the clinical history supported by the cyto-protein dissociation in cerebrospinal fluid, the patient was diagnosed to have MFS and successfully treated with intravenous immunoglobulin. This case underscores the importance of recognising atypical features of MFS, such as primary mydriasis, and highlights the variable clinical spectrum within the syndrome. Clinicians should maintain a high index of suspicion for MFS, particularly when faced with unusual neurological presentations, to ensure timely intervention and optimal patient outcomes.

The SARS-CoV-2 pandemic has significantly transformed the world. While it was initially viewed as a respiratory virus, it has now been found to cause cardiovascular, gastrointestinal, and psychological complications. Moreover, the nervous system (NS) is also greatly affected. Research has identified dizziness, headaches, as well as disturbances in one's taste and smell abilities as the most frequent symptoms of NS involvement. Even more significant changes may occur in patients who get infected with SARS CoV-2, such as the development of acute cerebrovascular disorders (stroke), multiple sclerosis, acute disseminated encephalitis, Guillain-Barré syndrome, encephalitis, and myelitis. It is important to note these potential complications and monitor patients closely. A retrospective study conducted in Wuhan, China revealed that CNS (central nervous system) involvement occurred three times more frequently than PNS (peripheral nervous system) involvement. This emphasizes the critical importance of studying and describing CNS manifestations. This case report depicts Miller Fisher syndrome in a 5-month-old infant after SARS CoV-2 infection and explores literature on pediatric cases and potential pathogenic mechanisms.

Guillain-Barré syndrome (GBS) is an ascending demyelinating polyneuropathy often associated with recent infection. Miller Fisher syndrome represents a variant with predominant facial and cranial nerve involvement, although Miller Fisher and Guillain-Barré overlap syndromes can occur. Guillain-Barré spectrum syndromes have been thought to be rare among solid organ transplant recipients. We describe an immunocompromised patient with a liver transplant who presented with ophthalmoplegia and bulbar deficits. His symptoms rapidly progressed to a state of descending paralysis involving the diaphragm; he then developed acute respiratory failure and eventually developed quadriparesis. Electromyography and a nerve conduction study demonstrated a severe sensorimotor axonal polyneuropathy consistent with Miller Fisher variant Guillain-Barré syndrome. Despite several negative nasopharyngeal swabs for COVID-19 polymerase chain reaction, a serology for SARS-CoV-2 IgG was positive. He was diagnosed with Miller Fisher-Guillain-Barré overlap syndrome with rapid recovery following treatment with plasma exchange. Although Guillain-Barré is a rare complication in solid organ transplant recipients, this case highlights the importance of rapid diagnosis and treatment of neurologic complications in transplant patients. Furthermore, it demonstrates a possible case of neurological complications from COVID-19 infection.

Background: The typical clinical and radiological presentation of Bickerstaff brainstem encephalitis (BBE) has been highlighted in this case report. Case presentation: Bickerstaff encephalitis is a rare autoimmune inflammatory disorder and is considered a subtype of Guillain–Barré syndrome (GBS) along with Miller Fisher syndrome. The diagnosis of BBE is largely clinical, though laboratory tests and imaging can be of supportive value. We report a case of a 5-year-old child who presented with a classical clinical triad of BBE with characteristic magnetic resonance imaging (MRI) findings. Conclusions: BBE is a rare disease with very few cases being reported with typical clinical and radiological findings. Hence, we reported a typical case of BBE to make an addition to the available literature. [ABSTRACT FROM AUTHOR]

Guillain–Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence‐based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence‐to‐Decision (EtD) frameworks. For the six intervention PICOs, evidence‐based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti‐ganglioside antibodies is of limited clinical value in most patients with typical motor‐sensory GBS, but anti‐GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal–paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute‐onset chronic inflammatory demyelinating polyradiculoneuropathy (A‐CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2–4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12–15 L in four to five exchanges over 1–2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission. [ABSTRACT FROM AUTHOR]

Guillain-Barre syndrome (GBS) is one of the common causes for acute flaccid paralysis in adults and mostly preceded by infection. Miller Fisher syndrome (MFS) is a rare variant of GBS with incidence of 1 to 2 in 1,000,000. This syndrome has a triad of ataxia, areflexia, and ophthalmoplegia and diagnosed when two out above three features are present. It usually preceded by viral infection, most commonly Campylobacter jejuni, cytomegalovirus, and Epstein–Barr virus. However, it is very rarely reported in pulmonary tuberculosis. The pathogenesis involves an aberrant immune response due to molecular mimicry against myelin gangliosides. Hereby we are presenting an unusual case of MFS variant of GBS associated with pulmonary tuberculosis.

Background: Miller Fisher syndrome (MFS) is a rare condition in childhood, characterized by acute-onset ataxia, ophthalmoplegia, and areflexia. It is mostly preceded by viral and bacterial infections, and the pathogenesis is speculated to be an immune response to cross-reacting antigens. We describe a case of acute ataxia which was initially misdiagnosed as antihistaminic toxicity and later emerged to be MFS. Clinical Description: A 4.5-year-old girl presented with acute-onset ataxia, giddiness, and limb pain, following toxic ingestion of an antihistaminic drug (chlorpheniramine) that had been prescribed for an upper respiratory infection. The absence of waning symptoms, new manifestations, and undetectable drug levels prompted us to consider an alternate diagnosis. Management: Magnetic resonance imaging of the cranium and spine was unremarkable. Hence, a central nervous system infection was considered. Cerebrospinal fluid (CSF) analysis revealed albumin-cytologic dissociation, a negative viral panel, and sterile culture. Serum anti-GQ1b antibody of immunoglobulin G type was positive. A nerve conduction study revealed absent H-reflexes in both gastrocnemius muscles. A diagnosis of MFS was made based on clinical features, CSF albumin-cytologic dissociation, positive anti-GQ 1b antibody, and absent H reflexes. She was treated with intravenous immunoglobulin therapy, following which there was an improvement in 1 week and complete recovery within 3 months. Conclusion: Although a rare entity, we need to consider MFS in the differential diagnosis of ataxia when the neurological signs persist beyond the expected time duration so that investigations can be planned accordingly and timely immunotherapy initiated.

Introduction:Miller Fisher syndrome (MFS) is considered a rare variant of Guillain- Barré syndrome (GBS), a group of acute-onset immune-mediated neuropathies characterized by the classic triad of ataxia, areflexia, and ophthalmoparesis. The present review aimed to provide a detailed and updated profile of all aspects of the syndrome through a collection of published articles on the subject, ranging from the initial description to recent developments related to COVID-19. Methods: We searched PubMed, Scopus, EMBASE, andWeb of Science databases and gray literature, including references from the identified studies, review studies, and conference abstracts on this topic. We used all MeSH terms pertaining to "Miller Fisher syndrome," "Miller Fisher," "Fisher syndrome," and "anti-GQ1b antibody." Results: An extensive bibliography was researched and summarized in the review from an initial profile of MFS since its description to the recent accounts of diagnosis in COVID-19 patients. MFS is an immune-mediated disease with onset most frequently following infection. Anti-ganglioside GQ1b antibodies, detected in ~85% of patients, play a role in the pathogenesis of the syndrome. There are usually no abnormalities in MFS through routine neuroimaging. In rare cases, neuroimaging shows nerve root enhancement and signs of the involvement of the central nervous system. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. Although MFS is generally self-limited and has excellent prognosis, rare recurrent forms have been documented. Conclusion: This article gives an updated narrative review of MFS with special emphasis on clinical characteristics, neurophysiology, treatment, and prognosis of MFS patients. [ABSTRACT FROM AUTHOR]

Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) typically presents with respiratory illness and fever, however some rare neurologic symptoms have been described as presenting complaints. We report a case of an acute motor and sensory polyneuropathy consistent with Miller-Fisher Syndrome (MFS) variant of Guillain Barre Syndrome (GBS) as the initial symptom.Case Report: A 31-year old Spanish speaking male presents with two months of progressive weakness, numbness, and difficult walking. He had multiple cranial nerve abnormalities, dysmetria, ataxia, and absent lower extremity reflexes. An extensive workup including infectious, autoimmune, paraneoplastic, metabolic and neurologic testing was performed. Initially SARS-CoV-2 was not suspected based on a lack of respiratory symptoms. However, workup revealed a positive SARS-CoV-2 polymerase chain reaction test as well as presence of Anti-Ganglioside – GQ1b (Anti-GQ1b) immunoglobulin G antibodies.Discussion: Miller Fisher syndrome (MFS) is a variant of Guillain-Barre syndrome (GBS) characterized by a triad of ophthalmoplegia, ataxia, and areflexia. The patient’s exam and workup including Anti-GQ1b is consistent with MFS.Conclusion: SARS-CoV-2 infection in patients can have atypical presentations similar to this neurologic presentation. Prompt recognition and diagnosis can minimize the risk of transmission to hospital staff and facilitate initiation of treatment.

Abstract Introduction Miller-Fisher Syndrome (MFS) is a variant of Guillain–Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited. Case report A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block. Discussion and conclusion Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia).

IntroductionMiller Fisher syndrome (MFS) is considered a rare variant of Guillain-Barré syndrome (GBS), a group of acute-onset immune-mediated neuropathies characterized by the classic triad of ataxia, areflexia, and ophthalmoparesis. The present review aimed to provide a detailed and updated profile of all aspects of the syndrome through a collection of published articles on the subject, ranging from the initial description to recent developments related to COVID-19.MethodsWe searched PubMed, Scopus, EMBASE, and Web of Science databases and gray literature, including references from the identified studies, review studies, and conference abstracts on this topic. We used all MeSH terms pertaining to “Miller Fisher syndrome,” “Miller Fisher,” “Fisher syndrome,” and “anti-GQ1b antibody.”ResultsAn extensive bibliography was researched and summarized in the review from an initial profile of MFS since its description to the recent accounts of diagnosis in COVID-19 patients. MFS is an immune-mediated disease with onset most frequently following infection. Anti-ganglioside GQ1b antibodies, detected in ~85% of patients, play a role in the pathogenesis of the syndrome. There are usually no abnormalities in MFS through routine neuroimaging. In rare cases, neuroimaging shows nerve root enhancement and signs of the involvement of the central nervous system. The most consistent electrophysiological findings in MFS are reduced sensory nerve action potentials and absent H reflexes. Although MFS is generally self-limited and has excellent prognosis, rare recurrent forms have been documented.ConclusionThis article gives an updated narrative review of MFS with special emphasis on clinical characteristics, neurophysiology, treatment, and prognosis of MFS patients.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was identified as the pathogen responsible for the pandemic health emergency declared by the World Health Organization in March 2020. During the first part of the pandemic, adults showed mild to severe respiratory symptoms. Children seemed initially exempt, both from acute and subsequent complications. Hyposmia or anosmia were promptly identified as the main symptoms of acute infection, so neurotropism of SARS-CoV-2 was immediately suspected. (1, 2). As the emergency progressed, post infectious neurological complications were described also in pediatric population (3). Cases of cranial neuropathy in connection with acute SARS-CoV-2 infection have been reported in pediatric patients, as an isolate post infectious complication or in the context of the multisystem inflammatory syndrome in children (MIS-C) (4-6). Neuroinflammation is thought to be caused by several mechanisms, among which immune/autoimmune reactions (7), but so far, no specific autoantibody has been identified. SARS-CoV-2 can enter the central nervous system (CNS) directly and/or infect it retrogradely, through the peripheral nervous system (PNS), after replicating peripherally; several factors regulate invasion and subsequent neuroinflammation. Indeed, direct/secondary entry and replication can activate CNS-resident immune cells that, together with peripheral leukocytes, induce an immune response and promote neuroinflammation. In addition, as we will discuss in the following review, many cases of peripheral neuropathy (cranial and non-cranial) have been reported during or after SARS-CoV-2 infection. However, some authors have pointed out that the increase of cranial roots and ganglia in neurological imaging is not always observed in children with cranial neuropathy. (8). Even if a variety of case reports were published, opinions about an increased incidence of such neurologic diseases, linked to SARS-CoV-2 infection, are still controversial (9-11). Facial nerve palsy, ocular movements abnormalities and vestibular alterations are among the most reported issues in pediatric population (3-5). Moreover, an increased screen exposure imposed by social distancing led to acute oculomotion's disturbance in children, not primarily caused by neuritis (12, 13). The aim of this review is to suggest food for thought on the role of SARS-CoV-2 in neurological conditions, affecting the peripheral nervous system to optimize the management and care of pediatric patients. [ABSTRACT FROM AUTHOR]

Guillain-Barre syndrome (GBS) is one of the common causes for acute flaccid paralysis in adults and mostly preceded by infection. Miller Fisher syndrome (MFS) is a rare variant of GBS with incidence of 1 to 2 in 1,000,000. This syndrome has a triad of ataxia, areflexia, and ophthalmoplegia and diagnosed when two out above three features are present. It usually preceded by viral infection, most commonly Campylobacter jejuni , cytomegalovirus, and Epstein–Barr virus. However, it is very rarely reported in pulmonary tuberculosis. The pathogenesis involves an aberrant immune response due to molecular mimicry against myelin gangliosides. Hereby we are presenting an unusual case of MFS variant of GBS associated with pulmonary tuberculosis. [ABSTRACT FROM AUTHOR]

We report a case of a 76-year-old man with Miller Fisher syndrome presenting with acute ophthalmoplegia and ataxia. Cerebrospinal fluid analysis showed normocytosis with an increased protein level. Serum anti-GQ1b IgG and anti-GT1a IgG antibodies were positive. Based on these results, the patient was diagnosed with Miller Fisher syndrome. He was treated with two courses of intravenous immunoglobulin, which improved his neurological symptoms. Brain perfusion single-photon emission computed tomography showed that cerebellar blood flow was decreased in the acute stage of the disease and improved after treatment. Although the prevailing view is that ataxia in Miller Fisher syndrome patients is of a peripheral origin, this case suggests that cerebellar hypoperfusion contributes to the development of ataxia in Miller Fisher syndrome. [ABSTRACT FROM AUTHOR]

Abstract Background To prevent the spread of the novel coronavirus disease 2019 (COVID-19) infection, various vaccines have been developed and used in a large number of people worldwide. One of the most commonly used vaccines is the mRNA vaccine developed by Moderna. Although several studies have shown this vaccine to be safe, the full extent of its side effects has not yet been known. Miller-Fisher syndrome (MFS) is a rare condition that manifests ophthalmoplegia, ataxia, and loss of tendon reflexes. It is a subtype of Guillain–Barré syndrome and an immune-mediated disease related to serum IgG anti-GQ1b antibodies. Several vaccines including those for COVID-19 have been reported to induce MFS. However, there have been no reports of MFS following Moderna COVID-19 vaccine administration. Case presentation A 70-year-old man was referred to our hospital due to diplopia that manifested 1 week after receiving the second Moderna vaccine dose. The patient presented with restricted abduction of both eyes, mild ataxia, and loss of tendon reflexes. He was diagnosed with MFS based on his neurological findings and detection of serum anti-GQ1b antibodies. The patient was administered intravenous immunoglobulin, and his symptoms gradually improved. Five days after admission, the patient showed peripheral facial paralysis on the right side. This symptom was suggested to be a delayed onset of peripheral facial nerve palsy following MFS that gradually improved by administration of steroids and antiviral drugs. Conclusion There have been no previous reports of MFS after Moderna COVID-19 vaccination. This case may provide new information about the possible neurological side effects of COVID-19 vaccines.

Copyright of Gyermekgyógyászat is the property of Semmelweis Kiado and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Background Neurologic impediments occur in only 0.1% of Mycoplasma pneumoniae infections. Although direct intracerebral infection can occur in these patients, autoimmune-mediated reactions secondary to molecular mimicry are the most common pathophysiology of such neurological complications. These complications include immune-mediated encephalitis, peripheral neuritis such as Guillain–Barré syndrome, and many others. Miller Fisher syndrome is a one of the variants of Guillain–Barré syndrome that has been rarely linked to Mycoplasma pneumoniae infection. It is a condition classically characterized by the triad of ophthalmoplegia, areflexia, and ataxia. Most patients with Miller Fisher syndrome will have positive anti-ganglioside GQ1b antibodies found in their serum, making this autoantibody a very useful serological confirmation parameter. We report a case of a Miller Fisher syndrome in a woman with Mycoplasma pneumoniae infection. To the best of the authors’ knowledge, such cases have been only rarely described in literature. Case presentation A 35-year-old Chinese woman presented with sudden onset of double vision and ataxia 5 days after fever and mild flu symptoms. Her Mycoplasma pneumoniae antigen was positive with 1 over 2560 titer of total mycoplasma antibody and presence of immunoglobulin M antibody, suggesting acute infection, and her nerve conduction study revealed mild sensory axonal polyneuropathy with segmental demyelination. the Miller Fischer syndrome variant of Guillain-Barré syndrome secondary to Mycoplasma pneumonia was suspected and later confirmed by presence of serum anti-GQ1b autoantibody. She was treated with intravenous immunoglobulin 0.4 g/kg once daily for 5 days. Conclusions The objective of this report is to share a case of an uncommon neurological complication of Mycoplasma pneumoniae infection, to increase the level of suspicion among clinicians that Miller Fischer syndrome can occur as an atypical presentation of an atypical pneumonia.

Abstract Background Parinaud’s syndrome, also known as dorsal midbrain syndrome, is characterized by a combination of signs, including upgaze palsy, convergence–retraction nystagmus, eyelid retraction and pupillary dissociation. It is caused by pretectal or tegmental lesions of upper brainstem. Miller Fisher syndrome, characterized by the triad of ataxia, areflexia and ophthalmoplegia, has rarely been reported to present as Parinaud’s syndrome in adults. To our knowledge, this clinical manifestation has not been previously described in children. Case presentation A previously healthy 13-year-old girl presented with blurred vision and diplopia, 10 days after a viral infection. Initial examination revealed incomplete Parinaud’s syndrome, while rest of neurological examination was normal. Brain imaging (MRI, MRA) did not reveal any abnormal findings and CSF findings were also normal. During the first days after admission she gradually deteriorated, showing complete external ophthalmoplegia, unsteady gait, and absent deep tendon reflexes of lower limbs with normal muscle power. With the clinical suspicion of Miller Fisher syndrome IVIG was administered, leading to subsequent resolution of her symptoms. AntiGQ1b and antiGD1b antibodies came back positive confirming diagnosis. On 1 month follow-up, neurological examination revealed diplopia in left gaze, and a second dose of IVIG was administered with good response. She remains asymptomatic 1 year from disease onset. Conclusions Miller Fisher syndrome can rarely present as Parinaud’s syndrome in adults. Herein we described the first pediatric patient with similar clinical presentation. As the typical semiology of ataxia and areflexia may not be present initially, high index of suspicion is required to recognise and treat those patients promptly. Serological detection of anti-ganglioside antibodies, such as anti-GQ1b and anti-GD1b, may help confirm diagnosis.

ObjectiveThis study aimed to retrospectively analyze reported Guillain–Barré syndrome (GBS) cases that occurred after COVID-19 vaccination.MethodsCase reports of GBS following COVID-19 vaccination that were published before May 14, 2022, were retrieved from PubMed. The cases were retrospectively analyzed for their basic characteristics, vaccine types, the number of vaccination doses before onset, clinical manifestations, laboratory test results, neurophysiological examination results, treatment, and prognosis.ResultsRetrospective analysis of 60 case reports revealed that post-COVID-19 vaccination GBS occurred mostly after the first dose of the vaccination (54 cases, 90%) and was common for DNA vaccination (38 cases, 63%), common in middle-aged and elderly people (mean age: 54.5 years), and also common in men (36 cases, 60%). The mean time from vaccination to onset was 12.3 days. The classical GBS (31 cases, 52%) was the major clinical classification and the AIDP subtype (37 cases, 71%) was the major neurophysiological subtype, but the positive rate of anti-ganglioside antibodies was low (7 cases, 20%). Bilateral facial nerve palsy (76% vs 18%) and facial palsy with distal paresthesia (38% vs 5%) were more common for DNA vaccination than for RNA vaccination.ConclusionAfter reviewing the literature, we proposed a possible association between the risk of GBS and the first dose of the COVID-19 vaccines, especially DNA vaccines. The higher rate of facial involvement and a lower positive rate of anti-ganglioside antibodies may be a characteristic feature of GBS following COVID-19 vaccination. The causal relationship between GBS and COVID-19 vaccination remains speculative, more research is needed to establish an association between GBS and COVID-19 vaccination. We recommend surveillance for GBS following vaccination, because it is important in determining the true incidence of GBS following COVID-19 vaccination, as well as in the development of a more safer vaccine.

Introduction: Miller-Fisher Syndrome (MFS) is a variant of Guillain-Barré syndrome (GBS), an acute immune-mediated neuropathy, which manifests as a rapidly evolving areflex motor paralysis. This syndrome presents as a classic triad: ophthalmoplegia, areflexia, and ataxia. MFS is usually benign and self-limited.Case Report: A Caucasian patient was admitted to our hospital with the flu, loss of bilateral strength in the lower limbs and upper limbs and sudden-onset ataxia 7 days after receiving a first dose of the Oxford/AstraZeneca COVID-19 vaccine. On neurological examination, the patient had Glasgow Coma Scale score of 15, with absence of meningeal signs; negative Babinski sign; grade 2 strength in the lower limbs and grade 4 strength in the upper limbs; axial and appendicular cerebellar ataxia; and peripheral facial diparesis predominantly on the right, without conjugate gaze deviation. Cerebrospinal fluid (CSF) was collected on admission, and analysis revealed albuminocytological dissociation with CSF protein of 148.9 mg/dL; leukocytes, 1; chlorine, 122; glucose, 65 mg/mL; red cells, 2; and non-reactive venereal disease research laboratory test result. The COVID-19 IgG/IgM rapid immunological test was negative. Electroneuromyography revealed a recent moderate-grade and primarily sensory and motor demyelinating polyneuropathy with associated proximal motor block.Discussion and Conclusion: Miller-Fisher Syndrome may be related to events other than infections prior to neuropathy, as in the case reported here. The patient presented strong correlations with findings for MFS reported in the literature, such as the clinical condition, the results of electroneuromyography, and results of the CSF analysis typical for MFS. When treatment was provided as proposed in the literature, the disease evolved with improvement. Ultimately, the diagnosis of incomplete MFS was made, including acute ataxic neuropathy (without ophthalmoplegia). [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) is a rare acquired immune-mediated polyneuropathy. Updated population-based data concerning paediatric GBS is needed. Paediatric patients aged below 18 years diagnosed with GBS between 2009 and 2018 in all 11 paediatric departments in Hong Kong were identified from the Hong Kong Hospital Authority Clinical Data Analysis and Reporting System. The collected data from medical health records were reviewed by paediatric neurologist from each department. Estimated incidence of paediatric GBS was calculated. We also compared our findings with other paediatric GBS studies in Asia. 63 subjects of paediatric GBS were identified, giving an estimated annual incidence of 0.62 per 100,000 population. Half of the subjects had acute inflammatory demyelinating polyneuropathy (AIDP) (n = 31; 49.2%), one quarter had Miller Fisher Syndrome (MFS) (n = 16; 25.4%), one-fifth had axonal types of GBS (n = 12; 19.0%), and four were unclassified. Paediatric subjects with axonal subtypes of GBS compared to the other 2 subtypes, had significantly higher intensive care unit (ICU) admission rates (p = 0.001) and longest length of stay (p = 0.009). With immunomodulating therapy, complete recovery was highest in those with MFS (100%), followed by AIDP (87.1%) and axonal GBS (75%). Our study also confirms a higher MFS rate for paediatric GBS in East Asia region and our study has the highest MFS rate (25.4%). Our population-based 10-year paediatric GBS study provides updated evidence on estimated incidence, healthcare burden and motor outcome of each subtype of paediatric GBS and confirmed a higher occurrence of paediatric MFS in East Asia. [ABSTRACT FROM AUTHOR]

Background and Purpose: Miller Fisher syndrome (MFS), a variant of Guillain–Barré Syndrome (GBS), could be underestimated in evaluations of its adverse events (AEs) following COVID‐19 vaccination. We aimed to identify and characterize MFS following COVID‐19 vaccination. Materials and Methods: Relevant studies reported on during the COVID‐19 pandemic were identified in the MEDLINE, Embase, and other databases. Results: Nine cases of MFS following COVID‐19 vaccination from various regions were included. Unlike MFS following COVID‐19 infection, patients with MFS following COVID‐19 vaccination frequently presented with anti‐GQ1b antibody positivity (44%, 4/9). Unlike GBS following COVID‐19 vaccination, only two of nine (22%) cases of MFS following COVID‐19 vaccination had developed after viral‐vector‐related vaccine administration. Conclusions: Miller Fisher syndrome following COVID‐19 vaccination seems to have a different pathophysiology from MFS following COVID‐19 infection and GBS following COVID‐19 vaccination. This neurological syndrome with a rare incidence and difficulty in diagnosis should be considered an AE of COVID‐19 vaccination. [ABSTRACT FROM AUTHOR]

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Miller Fisher syndrome (MFS) is frequently encountered variant of Gillian Barre Syndrome (GBS). It has distinct clinical and serological features. Here we describe an atypical GQ1b seronegative case with significantly elevated anti-glutamic acid decarboxylase antibody (GAD-Ab). A 24-year-old previously healthy Caucasian male presented with rapidly progressive ascending weakness, binocular diplopia and autonomic instability for 2 days. Examination was remarkable for asymmetrical facial weakness (L > R), opthalmoplegia and truncal ataxia without areflexia. MRI brain was normal. CSF analysis showed elevated protein. Electromyography/Nerve Conduction Study (EMG/NCS) within the first week was normal. Antiganglioside antibodies were negative. Extended serological and neoplastic workup revealed negative anti-GQ1b antibody, but significant increase of GAD-Ab, Voltage Gated Calcium Channel (VGCC) Ab, and mild elevation of TPO Ab IgG and Thyroglobulin (Tg) Ab IgG. Clinical diagnosis of partial MFS was made. He received a course of IVIg (2 g/kg over 5 days) and had complete recovery in 3 months. There are incomplete or atypical forms of MFS. Recognition of its various clinical presentations is essential for early diagnosis and optimal management. Further investigation is needed to elucidate the role of anti-GAD-ab and other autoimmune antibodies in the pathogenesis of GQ1b-seronegative MFS patients. [ABSTRACT FROM AUTHOR]

Ptosis, diplopia, and overall weakness in children may have serious underlying causes such as myasthenia, botulism, Guillain-Barré syndrome, and poisoning, which require a systematic and timely evaluation and proper management.(1,2,3) In pediatrics, clinical presentations may be atypical, and diagnostic overlap sometimes makes the final diagnosis challenging. This can be addressed through taking accurate history, performing a physical exam, conducting a comprehensive assessment, and using appropriate diagnostic algorithms. [ABSTRACT FROM AUTHOR]

Abstract Background The coronavirus disease 2019 (COVID-19) pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), began in late 2019. One of the vaccines approved against COVID-19 is the BNT162b2 mRNA COVID-19 vaccine (Pfizer/BioNTech). Case presentation We present the case of a 71-year-old man with no history of the SARS-CoV-2 infection or any recent viral or bacterial illnesses who presented with bilateral oculomotor palsy and limb ataxia after BNT162b2 mRNA COVID-19 vaccination. The diagnosis of Miller Fisher syndrome (MFS) was established based on physical examination, brain magnetic resonance imaging (MRI), cerebrospinal fluid analysis (CSF), and positron emission tomography (PET). There was no evidence of other predisposing infectious or autoimmune factors, and the period from COVID-19 vaccination to the appearance of neurological symptoms was similar to that of other vaccines and preceding events, such as infection. Conclusion Guillain–Barré syndrome (GBS) and its variants after COVID-19 vaccination are extremely rare. Note that more research is needed to establish an association between MFS and COVID-19 vaccines. In our opinion, the benefits of COVID-19 vaccination largely outweigh its risks.