Since 2020, updates in the diagnostic criteria for various neuromuscular diseases have emerged by advancements in electrophysiological studies, biomarker research, and imaging modalities. These developments reflect an effort to improve diagnostic accuracy and allow for earlier intervention. This review summarizes the most recent revisions in the diagnostic criteria for amyotrophic lateral sclerosis, primary lateral sclerosis, Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, and myasthenia gravis.
Background Electromyography and nerve conduction studies (EMG-NCS) often yield nonspecific findings during the early stage of Guillain-Barré syndrome (GBS). Although useful for diagnosis, EMG-NCS are traditionally of little value in predicting a complicated clinical course, which is critical for timely decisions to initiate immunomodulatory treatment. We aimed to identify the early clinical and electrophysiologic predictors of respiratory failure, a prolonged hospital stay, and moderate-to-severe disability at discharge in GBS.
Methods We retrospectively analyzed the clinical and electrophysiologic data of adult GBS patients who were hospitalized during the early course of the disease (<2 weeks from symptom onset).
Results Eighty-one patients aged 47.5 ± 16.1 years were analyzed. The most common clinical variants were Miller-Fisher syndrome (30.9%) and classic sensorimotor GBS (25.9%). The clinical variant was not predictive of a complicated clinical course. Instead, specific clinical features such as dysautonomia (p = 0.006) and marked motor deficits (p = 0.002) were predictive of the primary composite outcome of respiratory failure and/or a prolonged hospital stay, with dysautonomia (p = 0.035) also predictive of moderate-to-severe disability at discharge. The most common abnormalities in EMG-NCS were bilateral absence of the H-reflex (86.4%) and F-wave abnormalities (44.4%). The presence of F-wave abnormalities was predictive of both respiratory failure (p = 0.032) and a prolonged hospital stay (p = 0.001).
Conclusions Early F-wave abnormalities in GBS may serve as an electrophysiologic predictor of a complicated clinical course, suggesting that EMG-NCS can provide prognostic information to guide treatment decisions during the early stage of the disease.
Respiratory muscle weakness caused by neuromuscular disease can lead to both acute and chronic respiratory failure. Respiratory failure caused by Guillain-Barré syndrome and myasthenia gravis can potentially improve with disease-specific therapy. However, respiratory failure in amyotrophic lateral sclerosis is irreversible, and it may be necessary to provide full-time ventilation support along with additional assistance. Noninvasive ventilation is recommended for managing acute or acute-on-chronic hypercapnic respiratory failure caused by neuromuscular disease. It has also been effective in weaning patients off invasive mechanical ventilation. Although noninvasive ventilation offers numerous benefits over invasive mechanical ventilation, it is crucial to consider the specific contraindications and limitations of noninvasive ventilation and ensure its appropriate usage based on the patient's condition and needs. The timely recognition of neuromuscular respiratory failure is critical, as early intervention can be life-saving. This review focused on the clinical assessment and management of acute respiratory failure in neuromuscular diseases.
Sensory Guillain-Barré syndrome (GBS) is a rare heterogeneous subgroup of GBS characterized by the primary involvement of sensory neurons resulting in a distinctive clinical presentation. Sensory GBS usually occurs with acute and monophasic sensory symptoms, and no or minimal muscle weakness. Sensory GBS patient show hypo- or areflexia, distal paresthesia, and normal cerebrospinal fluid finding or albumino-cytologic dissociation which are suggesting that these conditions are a GBS variant. Autonomic dysfunctions have rarely been reported in sensory GBS patient presenting with postural hypotension, abnormal heart rate response to respiration. In this case, we demonstrate a patient with autonomic symptoms dominant sensory GBS in systemic lupus erythematosus.
Neurological complications attributed to coronavirus disease-19 (COVID-19) infection have been reported including acute disseminated encephalomyelitis, Guillain-Barré syndrome, and so on. Herein, we report a 49-year-old woman presented with acute encephalopathy and paraplegia simultaneously after COVID-19 infection. Brain magnetic resonance imaging (MRI) showed symmetric hyperintense basal ganglia lesions on T2-weighted imaging. Cerebrospinal fluid pleocytosis, motor axonal neuropathy and enhancement of conus medullaris nerve roots on spine MRI were observed. We treated her with high-dose corticosteroid and intravenous immunoglobulin.
Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-yearold male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.
Previous studies show few cases of concomitant Guillain-Barré syndrome (GBS) and acute transverse myelitis (ATM), also described as GBS and ATM overlap syndrome. We experienced a patient who presented with acute progressive leg weakness and hyperreflexia at initial stage, but was followed by areflexia. The spine magnetic resonance imaging revealed radiological findings of ATM and nerve conduction studies showed electrodiagnostic findings of acute motor axonal neuropathy, known as GBS subtype. The response to treatment with intravenous immune globulin was good.
Facial nerve palsy is one of major accompanying features in Guillain-Barré syndrome (GBS). In most of the cases, facial weakness develops simultaneously with other symptoms such as motor weakness, sensory change and other cranial neuropathies. However, facial palsy also occurs after the nadir of neurological deficits or even after the beginning of limb weakness improvement, called delayed facial palsy (DFP). DFP has been reported in Miller Fisher syndrome, but it rarely found from the acute motor axonal neuropathy subtype of GBS. Recently, we experienced a patient who diagnosed acute motor axonal neuropathy accompanying with delayed facial diplegia.
Miller Fisher syndrome (MFS) is characterized by the acute ophthalmoparesis, ataxia and areflexia. We describe the case of 70-year-old man with cardinal symptom of MFS and active pulmonary tuberculosis (Tb). A thorough evaluation led to the diagnosis of MFS and treatment with intravenous immunoglobulin (IVIg) was started. The complete resolution of ophthalmoparesis and ataxia was observed from the fourth day of IVIg treatment. This is the first report to describe a case of MFS that developed in patient pulmonary tuberculosis.
Citations
Citations to this article as recorded by
A Precipitant Less Appreciated: A Glance at Cases of Tuberculosis Manifesting with Guillain Barre Syndrome Camelia Porey, Binaya Kumar Jaiswal Indian Journal of Clinical Medicine.2023; 13(1): 43. CrossRef
Miller Fisher Variant of Guillain-Barre Syndrome Secondary to Pulmonary Tuberculosis: A Case Report with Review of Literature Chandra M. Tatikonda, Kaushik R. Juvvadi, Sagarika Panda, Shakti B. Mishra, Abhilash Dash Journal of Neuroanaesthesiology and Critical Care.2023; 10(02): 128. CrossRef
Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) can present with overlapping features. A 56-year-old female developed ptosis and diplopia after an upper respiratory infection, and presented with facial palsy, dysarthria, brachial weakness, ataxia, and areflexia. Mild weakness of both legs appeared after a few days. Anti-ganglioside complex antibody were positive to IgG GM1/GQ1b and GQ1b/sulfatide antibodies. The present case suggests that the manifestation of overlap between MFS/PCB variants and GBS could be caused by antiganglioside complex antibodies.
Citations
Citations to this article as recorded by
Overlap of Miller-Fisher Syndrome and Pharyngeal-Cervical-Brachial Variant Secondary to COVID-19 in Recurrent Guillain-Barré Syndrome: A Case Report Tarek Hammad, Sayeed Hossain, Amin Alayyan Cureus.2024;[Epub] CrossRef
Guillain-Barre syndrome (GBS) is acute inflammatory demyelinating polyradiculoneuropathy, which is often related to post-infectious etiology. However, GBS has also been reported to be caused by non-infectious factors such as trauma. This report describes a rare case of post-traumatic GBS with dramatic response to immunoglobulin therapy. And here, we also discussed about the importance of differential diagnosis with critical illness polyneuropathy.
Citations
Citations to this article as recorded by
MultiSpace Deep Fascial Infection in the Head and Neck Patient Complicated with Guillain-Barre Syndrome: Case Report with a Literature Review Fengxia Fan, Hongli Ni, Wanjie Luo, Qingchuan Yi Ear, Nose & Throat Journal.2025;[Epub] CrossRef
Pain in Guillain-Barré syndrome (GBS) is known as a common symptom, experienced by about 72% of patients. Various types of pain are associated with GBS, including paresthesia, dysesthesia, radicular pain, meningism, myalgia and visceral pain. Pain in GBS can vary from mild to severe, often under-recognized and poorly managed. This article reviews the various pains associated with Guillain-Barré syndrome and their management.
The electrodiagnostic findings in Guillain-Barré syndrome (GBS) play important roles in both understanding its pathophysiology and its diagnosis. Only demyelinating neuropathies were thought to be present when GBS patients were first diagnosed in Western countries, but the concept changed when many axonal GBS patients were reported in Asia. Reversible conduction failure was subsequently revealed, and it was recognized as a pathophysiologic continuum of axonal GBS. Thus, the electrodiagnostic findings in GBS have had a profound effect on the history of this disease.
Citations
Citations to this article as recorded by
Predictors of the Short‐Term Outcomes of Guillain–Barré Syndrome: Exploring Electrodiagnostic and Clinical Features Yi‐Hsiang Chen, Chia‐Lun Wu, Wei‐Chieh Weng, Yi‐Chia Wei Brain and Behavior.2025;[Epub] CrossRef
Guillain–Barré syndrome (GBS) after severe/critical COVID-19 or COVID-19 vaccination Samira Bahrami, Behnaz Ansari, Leyla Norouzi-Barough, Bahram Bagherpour, Farzin Khorvash, Kiana Shirani, Saeed Abbasi, Roya Sherkat European Journal of Medical Research.2025;[Epub] CrossRef
Symmetric Ascending Paralysis Secondary to West Nile Virus Alex Ashkin, Nicole Saccone, Jose Valle, Mark Rasnake Case Reports in Neurology.2023; 15(1): 120. CrossRef
Reversible conduction failure in acute inflammatory demyelinating polyneuropathy Sooyoung Kim, Eun Kyoung Lee, Eunhee Sohn Scientific Reports.2022;[Epub] CrossRef
Review of Acute Inflammatory Demyelinating Polyradiculoneuropathy Gabriela Palombo, Blake Hoppe JBJS Journal of Orthopaedics for Physician Assista.2022; 10(1): e21.00031. CrossRef
Guillain-Barré Syndrome Associated with Shiga Toxin-Producing and Enterotoxigenic Escherichia coli Infection Jaeho Kang, Jeong Ik Eun, Hyunyoung Hwang, Byeol-A Yoon, Kyong Jin Shin Journal of the Korean Neurological Association.2022; 40(3): 256. CrossRef
Invited Commentary – SARS-COV-2-Associated Guillain-Barré Syndrome Requires Appropriate Exclusion of Possible Differentials Jordan Yakoby The Journal of Emergency Medicine.2021; 61(6): 794. CrossRef
Sequential Nerve Conduction Studies in Guillain-Barre Syndrome Madhu Nagappa, Arun B Taly Neurology India.2021; 69(2): 376. CrossRef
Guillain-Barré syndrome (GBS) is nowadays consider as an umbrella term that has heterogenous presentation depend on their subtypes. GBS is clinical diagnosis and its diagnosis can be supported by laboratory findings from cerebral spinal fluid study, nerve conduction study, anti-ganglioside antibodies, spinal magnetic resonance imaging and nerve ultrasound. Understanding atypical subtypes and GBS mimicking diseases are crucial for correct diagnosis. Both proper medical care for respiratory and autonomic dysfunction and immunotherapy are essential to improve outcome of GBS. Here, we summarized the current concept on diagnosis, immunopathophysiology and treatment of GBS.
Intravenous immunoglobulin (IVIG) is a safe treatment to treat various neurological disorders, but fatal thrombotic events as rare complications have been reported. A 54-year-old woman with Guillain-Barre syndrome complained of dyspnea during IVIG treatment. She was finally diagnosed with pulmonary thromboembolism. To the best of our knowledge, this is the first case of pulmonary thromboembolism associated with IVIG treatment in a Korean patient with Guillain-Barre syndrome.
A 69-year-old woman presented with a progressive limb weakness. Both clinical and neurophysiological findings were consistent with diagnosis of Guillain-Barré syndrome (GBS). Two days after admission, the patient suffered from an acute coronary syndrome without stenosis at coronary arteriography. Echocardiography revealed left ventricular inferior wall and apical akinesia and decreased ejection fraction. A diagnosis of Takotsubo cardiomyopathy was then made. Left ventricular dysfunction and electrocardiography normalized within one month. Takotsubo cardiomyopathy can be developed as a complication of GBS.
Citations
Citations to this article as recorded by
Takotsubo Cardiomyopathy: A Possible Rare Complication of Guillain-Barré Syndrome Khalid H Mohamed, Adetola F Oshikoya, Kapil Kumar, Chinyere L Anigbo, Polasu Sri Satya Sai Prashanth , Alaa S Mohamed, Muhammad Haseeb, Hira Nasir Cureus.2023;[Epub] CrossRef
Takotsubo Cardiomyopathy as a Manifestation of Dysautonomia in Guillain-Barré Syndrome: A Case Series and Review of the Literature Dyanet Puentes, Daniela Teijelo, Tamara S Stiep, Sishir Mannava, Jason Margolesky Cureus.2021;[Epub] CrossRef
We report a case with squamous cell lung cancer with concomitant Guillain-Barre syndrome (GBS) as a paraneoplastic syndrome. A 67-year-old patient who was previously diagnosed as metastatic squamous cell lung cancer developed mild symmetrical weakness, paresthesia and sensory ataxia. Nerve conduction study showed sensorimotor polyneuropathy. Analysis of cerebrospinal fluid showed high tilter for monospecific anti-GD1b IgG antibody without onconeuronal antibodies. After treatment with intravenous immunoglobulin, the patient’s symptoms improved.
Background : Guillain-Barre syndrome(GBS) is characterized clinically by acute flaccid paralysis, are flexia, and albumino-cytologic dissociation. Based on electrophysiology and pathology, GBS can be divided into either predominantly demyelinating or presominantly axonal patterns. Objectives : The clinical and laboratory status of probable acute axonal GBS occurring at a mental hospital was evaluated. Methods : Eight schizophrenia patients with probable acute axonal GBS were analyzed. Results : The mean age of the patients was 38 years old. Most of the patients were men. All patients showed an acute ascending paraparesis and/or quadriparesis with areflexia, and all have al history of schizophrenia for 3~20 years. The disease occurred predominantlyin the summer and electrodiagnostic studies revealed axonal patterns. The patients were treated by supportive care, except one patient with intravenous immunoglobulin. The prognosis was improved in 3 ; no change in 4 and 1 became aggravated. One patient with acute motor-sensory axonal neuropathy had a recurrence after 10 months of the first attack. Conclusions : Axonal GBS has been considered uncommon clinically or electrophysiologically, but 8 probable acute axonal GBSs occurring at a mental hospital have been diagnosed in 3.5 years
Both myasthenia gravis and Guillain-Barre syndrome are autoimmune disorder, but it is very rare that both of them occur together. A 53-years old woman with history of myasthenia gravis complained of weakness of extremities, worsening progressively for several days. Electrophysiologic study showed findings of motor axonal neuropathy without sensory involvement. She became improved in aspect f of clinical symptoms and electrophysiologic findings after infusion of immunoglobulin. This is a case of Guillain-Barre syndrome in a patient with myasthenia gravis.
Background There were several studies comparing prognostic factors in Guillain-Barre syndrome treated with intravenousimmunoglobulin and plasmapheresis. However, there were controversies in what were significant factors andthere were few studies so far comparing the therapeutic outcomes in patients treated with immunoglobulin. This studywas aimed to determine the prognostic factors which affected the therapeutic outcome of Guillain-Barre syndrome treatedwith intravenous immunoglobulin.Method: We retrospectively reviewed the medical records of patients with Guillain-Barre syndrome admitted to ourhospital between January 1999 and March 2004. All patients were treated with intravenous immunoglobulin. Outcomeand prognosis were followed up after four weeks using the overall disability sum score.Results: Thirty-six patients were enrolled in this study. According to the clinical and electrophysiological findings,17 patients were AIDP, 10 were axonal forms, two were mixed and seven had electrophysiologically no evidence ofabnormalities. At a follow-up of four weeks, disabilities at the nadir (p<0.001) and admission (P<0.012), initial manifestationsof bulbar symptom (P<0.024) and electrodiagnostic features (P<0.013) were significantly correlated with outcomein patients treated with intravenous immunoglobulin. But only disabilities at the nadir (P<0.033) and electrodiagnosticfeatures (P<0.018) were significant in the multivariate logistic regression analysis.Conclusion: Among the patient treated with intravenous immunoglobulin, the outcomes were significantly differentaccording to the neurological status at the nadir. Therefore early diagnosis, administration of intravenous immunoglobulinand preventing complications during acute stages are essential to minimize neurological deficit and shorten the periodsof recovery.
The Guillain-Barre syndrome (GBS) is an acute polyradiculoneuropathy marked by flaccid areflexic paralysis.Although the pathogenesis of GBS remains incompletely defined, considered as an autoimmune disease most frequentlytriggered by an previous infection. Antecedent infections with Campylobacter jejuni, cytomegalovirus, Ebstein-Barrvirus, Mycoplasma pneumoniae, Haemophilus influenzae, human immunodeficiency virus, enterovirus, rotavirus arecommon. But, it is rare that GBS following typhoid fever. We present a case of typical GBS after antecedentSalmonella typhi infection.
Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has beenused in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.
A 33-year-old women developed weakness in all limbs 3 days prior to admission. Motor examination showed decreased strength in all limbs, but sensory examination was normal. Deep tendon reflexes were areflexia. Electrophysiological examination showed conduction blocks with nearly normal conduction velocities and terminal latencies in motor nerves and normal amplitudes and velocities in sensory nerves. Her serum was positive for IgG antibodies to gangliosides GM1, GD1b, and galactocerebroside. Acute motor conduction block neuropathy may be another variant of Guillain-Barre
By definition, the time to reach nadir in Guillain-Barre syndrome (GBS) is within four weeks. This is in contrast to the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which progress for at least two months. However, CIDP can take a relapsing and remitting form and could mimic treatment related fluctuations of GBS (GBS-TRFs) especially during the early phase of disease. We report a patient with CIDP who initially presented with a rapidly progressive limb weakness mimicking GBS, but finally showed good recovery after long term corticosteroid therapy.
Two separate cranial nerve variants of Guillain-Barre syndrome(GBS) have been reported. One is Miller-Fisher syndrome,the other is polyneuritis cranialis. Involvement of the extraocular muscles in variants of GBS is well recognized,but complete external and internal opthalmoplegia is rare. Optic neuritis remains the only consistent, albeit very uncommon,evidence of inflammation of central nervous system myelin in GBS. This propose that GBS is part of a spectrumof central and peripheral inflammation.This case is an unusual clinical variant who had ptosis, opthalmoplegia, areflexia, ataxia, optic neurritis, markedoropharyngeal, and neck and shoulder weakness. This combined regional from is able to misdiagnose initially as botulismor diphtheria and less so, myasthenia. So if we were consider variant from of GBS, it is possible for make a correctdiagnosis more easily and treatment without delay.
The Guillain-Barre Sydrome (GBS) is post-infectious autoimmune disease and it could be caused by auto-antibodies producedafter infections. Mycoplasma pneumoniae is one of rare cause of GBS and known to be associated with antibodyto galactocerebroside (GalC) which is a major neutral glycolipid constituent of myelin. We report a case of GBS with immunoglobulinM GalC antibody after M. pneumoniae infection.
First
Prev
