Predicting Outcome in Guillain-Barré Syndrome: International Validation of the Modified Erasmus GBS Outcome Score

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000013139
Author(s):  
Alex Y Doets ◽  
Hester F Lingsma ◽  
Christa Walgaard ◽  
Badrul Islam ◽  
Nowshin Papri ◽  
...  
Keyword(s):  
North America ◽  
Characteristic Curve ◽  
Guillain Barre Syndrome ◽  
Full Cohort ◽  
Limb Weakness ◽  
Study Objective ◽  
Outcome Score ◽  
Guillain Barré Syndrome ◽  
The Individual ◽  
Guillain Barré

Background and Objectives:The clinical course and outcome of the Guillain-Barré syndrome (GBS) are diverse and vary among regions. The modified Erasmus GBS Outcome Score (mEGOS) is a clinical model that predicts the risk of walking inability in GBS patients, and was developed with data from Dutch patients. The study objective was to validate the mEGOS in the International GBS Outcome Study (IGOS) cohort and to improve its performance and region-specificity.Methods:We used prospective data from the first 1500 patients included in IGOS, aged ≥ 6 years and unable to walk independently. We evaluated if the mEGOS at entry and week 1 could predict the inability to walk unaided at 4 and 26 weeks in the full cohort and in regional subgroups, using two measures for model performance: (1) discrimination: area under the receiver operating characteristic curve (AUC), and (2) calibration: observed versus predicted probability of being unable to walk independently. To improve the model predictions we recalibrated the model containing the overall mEGOS score, without changing the individual predictive factors. Finally, we assessed the predictive ability of the individual factors.Results:For validation of mEGOS at entry 809 patients were eligible (Europe/North America n=677, Asia n=76, other=56), and 671 for validation of mEGOS at week 1 (Europe/North America n=563, Asia n=65, other=43). AUC-values were >0.7 in all regional subgroups. In the Europe/North America subgroup observed outcomes were worse than predicted, while in Asia observed outcomes were better than predicted. Recalibration improved model accuracy and enabled the development of a region-specific version for Europe/North America (mEGOS-Eu/NA). Similar to the original mEGOS, severe limb weakness and higher age were the predominant predictors of poor outcome in the IGOS cohort.Discussion:The mEGOS is a validated tool to predict the inability to walk unaided at 4 and 26 weeks in GBS patients, also in countries outside The Netherlands. We developed a region-specific version of mEGOS for patients from Europe/North America.Classification of Evidence:This study provides Class II evidence that the mEGOS accurately predicts the inability to walk unaided at 4 and 26 weeks in GBS patients.ClinicalTrials.gov Identifier:NCT01582763

scholarly journals Guillain –Barrè Syndrome following Hepatitis E

2014 ◽  
Vol 2 (1) ◽  
pp. 48-49
Author(s):  
Poly Sengupta ◽  
Rama Biswas ◽  
Hasan Shahrear Ahmed ◽  
Kaniz Fatema
Keyword(s):  
Motor Nerve ◽  
Electrophysiological Study ◽  
Hepatitis E ◽  
Immunoglobulin M ◽  
Guillain Barre Syndrome ◽  
Limb Weakness ◽  
Laboratory Findings ◽  
Guillain Barré Syndrome ◽  
Guillain Barré ◽  
Upper Abdominal

Guillain- Barrè Syndrome is characterized by acute progressive symmetric limb weakness and areflexia. A 32 year old female presented with progressive ascending areflexic muscular weakness and bilateral lower motor neuron type of facial palsy. She had anorexia, nausea and upper abdominal pain for 2 weeks. The findings of motor nerve conduction study are consistent with acute inflammatory demyelinating polyradiculoneuropathy. She had elevated liver enzyme and positive immunoglobulin M antibody against hepatitis E in blood. Based on clinical features, laboratory findings and electrophysiological study, she was diagnosed as Guillain- Barrè Syndrome following hepatitis E. She was treated with intravenous immunoglobulin and recovered fully. DOI: http://dx.doi.org/10.3329/bccj.v2i1.19973 Bangladesh Crit Care J March 2014; 2 (1): 48-49

Case Report on Guillain Barre Syndrome: Acute Inflammatory Demyelinating Polyneuropathy

2021 ◽  
Vol 8 (9) ◽  
pp. 548-550
Author(s):  
Chinnu Roy ◽  
Jobin Kunjumon Vilapurathu ◽  
Dhanya Paul
Keyword(s):  
Case Report ◽  
Medical Condition ◽  
Demyelinating Polyneuropathy ◽  
Lower Limbs ◽  
Guillain Barre Syndrome ◽  
Limb Weakness ◽  
Acute Inflammatory Demyelinating Polyneuropathy ◽  
Guillain Barré Syndrome ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Guillain Barré

Guillain Barre Syndrome (GBS) is an autoimmune disorder which affects the peripheral nervous system. It is a rare disorder affects in 1 per million people in year. It is characterized by symmetrical, progressive limb weakness and tingling. Case Report: A 53 year old male patient was presented with insidious onset of difficulty in moving right upper and lower limbs as well as gradual weakness of left limbs, and breathing difficulty, known case of diabetics’ mellitus and hypertension. Nerve conduction study shows suggest axonopathy; Acute Inflammatory Demyelinating Polyneuropathy (AIDP) is identified, which is a subtype of Guillain Barre Syndrome. Patient gradually develops areflexia, bifacial weakness, and quadriparesis. Patient was treated with IV immunoglobulin and intranasal oxygen therapy. Patient shows slight improvement in his medical condition, shows improvement in the power of lower limbs after one week of therapy. Physiotherapy was suggested. Keywords: Guillain Barre Syndrome, GBS, Acute Inflammatory Demyelinating Polyneuropathy, AIDP.

scholarly journals Concurrent Guillain-Barré syndrome and myositis complicating dengue fever

2020 ◽  
Vol 13 (2) ◽  
pp. e232940 ◽  
Author(s):  
Manisha Gulia ◽  
Preeti Dalal ◽  
Monica Gupta ◽  
Daljinderjit Kaur
Keyword(s):  
Creatine Kinase ◽  
Dengue Fever ◽  
Elevated Serum ◽  
Guillain Barre Syndrome ◽  
Uneventful Recovery ◽  
Motor Neuropathy ◽  
Limb Weakness ◽  
Guillain Barré Syndrome ◽  
Bilateral Lower Limb ◽  
Guillain Barré

Dengue is an arboviral infection that classically presents with fever, headache, joint pain, skin flush and morbilliform rashes. Neurological manifestations are well recognised but their exact incidence is unknown. Though myalgias are common in dengue virus infection, myositis and/or elevated serum creatine kinase is an uncommon complication. Guillain-Barré syndrome is another rare neurological manifestation associated with dengue fever. Here, we report the case of a 21-year-old man with serologically confirmed dengue fever presenting with severe myalgia, bilateral lower and upper limb weakness with raised creatine kinase, MRI suggestive of myositis and myonecrosis and nerve conduction velocity showing bilateral lower limb and axillary sensory motor neuropathy. He was managed conservatively and made an uneventful recovery.

scholarly journals Two Cases of Guillain-Barré Syndrome Variants Presenting With Dysautonomia

2019 ◽  
Vol 6 ◽  
pp. 2329048X1985677 ◽  
Author(s):  
Omar Abdel-Mannan ◽  
Luigi D’Argenzio ◽  
Matthew Pitt ◽  
Felice D’Arco ◽  
Sanjay Bhate ◽  
...  
Keyword(s):  
Intravenous Immunoglobulin ◽  
Posterior Reversible Encephalopathy Syndrome ◽  
Lower Limb ◽  
Guillain Barre Syndrome ◽  
Limb Weakness ◽  
Posterior Reversible Encephalopathy ◽  
Lower Limb Weakness ◽  
Guillain Barré Syndrome ◽  
Guillain Barré ◽  
Reversible Encephalopathy

We describe 2 pediatric cases presenting with posterior reversible encephalopathy syndrome secondary to autonomic dysfunction preceding the onset of motor symptoms in Guillain-Barré syndrome variants. Patient 1 presented acutely with encephalopathy, cerebellar signs, hypertension, lower limb weakness, and respiratory decompensation. Magnetic resonance imaging (MRI) brain showed occipital lesions consistent with posterior reversible encephalopathy syndrome. Nerve conduction studies were consistent with Miller-Fisher syndrome. After intravenous immunoglobulin and plasmapheresis, he improved clinically with radiological resolution. Patient 2 presented with headache, leg pain, seizures, and significant hypertension. Brain MRI was normal but spine MRI revealed enhancement of the cauda equina ventral nerve roots. She was areflexic with lower limb weakness a few days after intensive care unit admission and made a significant improvement after treatment with intravenous immunoglobulin. In children presenting with posterior reversible encephalopathy syndrome in the absent of other causes of primary hypertension, Guillain-Barré syndrome variants are an important differential etiology, presenting with autonomic dysfunction, even before signs of motor weakness become evident.

scholarly journals Guillain-Barré syndrome presenting with COVID-19 infection

2020 ◽  
Vol 13 (9) ◽  
pp. e236978 ◽  
Author(s):  
Nasir Ameer ◽  
Kalyan Mansukhbhai Shekhda ◽  
Ann Cheesman
Keyword(s):  
Neuromuscular Disorder ◽  
Guillain Barre Syndrome ◽  
Limb Weakness ◽  
Neurological Signs ◽  
The Third ◽  
Lower Limb Weakness ◽  
Guillain Barré Syndrome ◽  
Immunoglobulin Treatment ◽  
Systemic Symptoms ◽  
Guillain Barré

A construction worker in his 30s presented three times in 4 days with progressive upper and then lower limb weakness. On the first two occasions he had no systemic symptoms, but on the third presentation he had fever and cough, starting from day 4 of weakness. Examination identified weakness in all four limbs and areflexia, suggesting a peripheral neuromuscular disorder. Investigations were consistent with Guillain-Barré syndrome and additional COVID-19 (SARS-CoV-2) infection. The patient improved after immunoglobulin treatment. At least four cases of Guillain-Barré syndrome have been reported in the literature with concurrent COVID-19 illness in whom respiratory signs appeared a few days after the onset of neurological signs. With the incubation period for COVID-19 respiratory symptoms believed to be up to 14 days, it is possible that neurological symptoms could develop before respiratory and other symptoms. During the current pandemic, presence of concurrent COVID-19 infection needs to be considered in patients presenting with Guillain-Barré syndrome.

scholarly journals Paralytic ileus as the presenting symptom for Guillain–Barré syndrome: a case report

2019 ◽  
Vol 48 (4) ◽  
pp. 030006051989316
Author(s):  
Kuang-Heng Lee ◽  
Tsung-Han Ho ◽  
Jiunn-Tay Lee ◽  
Li-Fan Lin ◽  
Wei-Chou Chang ◽  
...  
Keyword(s):  
Paralytic Ileus ◽  
Early Recognition ◽  
Early Stage ◽  
Immunoglobulin Therapy ◽  
Guillain Barre Syndrome ◽  
Rapid Progression ◽  
Limb Weakness ◽  
Intravenous Immunoglobulin Therapy ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Guillain–Barré syndrome (GBS) is an acute neuroimmunological disorder characterized by rapidly ascending symmetrical limb weakness, areflexia, and sensory deficits. Approximately 65% of patients with GBS present with autonomic dysfunction, which commonly occurs in advanced stages. However, paralytic ileus, a sign of gastrointestinal dysautonomia, is rare as the presenting feature in GBS before motor weakness becomes evident. We report the case of a 54-year-old man admitted to the Emergency Department with paralytic ileus as the prodromal feature in early-stage GBS. Total parenteral feeding and prokinetic use were initiated, but no clinical improvement was observed. The patient showed rapid progression to quadriplegia, which was ultimately determined to be respiratory muscle failure requiring mechanical ventilation and intensive care unit admission. He underwent 5 days of intravenous immunoglobulin therapy and muscle strength was partially improved thereafter. However, the patient’s enteral nutritional support was undesirable because of persistent poor gastric emptying complicated by fungemia and profound sepsis throughout the hospital course. Finally, he died 1 month after admission. Ignorance of this unusual prodrome to GBS could result in delayed treatment, along with potential progression to life-threatening events. Early recognition of GBS and prompt immunotherapy are critical for reducing morbidity and mortality.

scholarly journals Miller-Fisher Syndrome and Guillain-Barre Syndrome overlap syndrome in a patient post Oxford-AstraZeneca SARS-CoV-2 vaccination

2021 ◽  
Vol 14 (11) ◽  
pp. e246701
Author(s):  
Yew Li Dang ◽  
Alexander Bryson
Keyword(s):  
Overlap Syndrome ◽  
Miller Fisher Syndrome ◽  
Guillain Barre Syndrome ◽  
Oculomotor Nerve Palsy ◽  
Limb Weakness ◽  
Patient Counselling ◽  
Fisher Syndrome ◽  
Facial Diplegia ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

We describe a patient who developed bilateral oculomotor nerve palsy, ataxia, facial diplegia and lower limb weakness 2 weeks post-Oxford-AstraZeneca SARS-CoV2 vaccination, consistent with Miller-Fisher syndrome (MFS) and Guillain-Barre syndrome (GBS) overlap syndrome. Although some features of the patient’s presentation were typical of recently reported cases of a rare GBS variant post-Oxford-AstraZeneca vaccination, including severe facial weakness and a lack of respiratory involvement, to our knowledge this is the first reported case of MFS associated with SARS-CoV2 vaccination. While postvaccination GBS remains rare, it appears to have a favourable prognosis, and recognising this entity is therefore important for patient counselling and monitoring for potential complications.

scholarly journals Comprehensive rehabilitation treatment for pregnant women with Guillain-Barré syndrome - a case report

2019 ◽  
Vol 22 (3) ◽  
pp. 57-61
Author(s):  
Katarzyna Hojan ◽  
Berenika Wruk ◽  
Anna Tymińska ◽  
Jan Kurnatowski ◽  
Hanna Norman
Keyword(s):  
Case Report ◽  
Pregnant Woman ◽  
Vital Signs ◽  
Guillain Barre Syndrome ◽  
Sensory Loss ◽  
Limb Weakness ◽  
Rehabilitation Treatment ◽  
Guillain Barré Syndrome ◽  
Comprehensive Rehabilitation ◽  
Guillain Barré

Guillain-Barré syndrome (GBS) is an acute demyelinating poliradioculopathy and autoimmune condition, with a fast rapid natural course, and with high disability and usually episodic immune treatment. Clinical features include areflexia, limb weakness and uncommonly, sensory loss proceeding to neuromuscular paralysis involving bulbar, facial and respiratory function with maximum severity of symptoms atin 2-4 weeks. The eEtiology of GBS is not incompletely understood, however, prognosis is usually good with early detection and prompt treatment. In thise paper, we present a unique case of comprehensive rehabilitation treatment for a pregnant woman with GBS atin the 8th week of pregnancy. On the basis of this case report, we discuss possible and safe rehabilitation treatment for women in the first trimester of pregnancy, with significantly weakened muscle strength, after stabilizsation of vital signs and immunotherapy. The paper describes comprehensive care provided to the pregnant woman with teraplegia, hyperstesia and dysphagia after stabiliszation of vital signs, which was conducted until delivery atin the 39th week of pregnancy. This article is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License. null

scholarly journals Melanomatous Leptomeningeal Carcinomatosis masquerading as Guillain-Barré Syndrome

2010 ◽  
Vol 9 (1) ◽  
pp. 20-23
Author(s):  
James R Noake ◽  
◽  
Andrew Shepherd ◽  
William R Smith ◽  
◽  
...  
Keyword(s):  
Intravenous Immunoglobulins ◽  
Rapid Onset ◽  
Leptomeningeal Carcinomatosis ◽  
Guillain Barre Syndrome ◽  
High Dose ◽  
Limb Weakness ◽  
Protein Levels ◽  
Csf Analysis ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

A 49 year old man presented with rapid onset paraparesis, evidence of lower motor neurone features and sensory impairment following a respiratory tract infection. Initially he was treated with intravenous immunoglobulins for suspected Guillain-Barré syndrome (GBS). Subsequent cerebrospinal fluid (CSF) analysis identified unexpectedly high protein levels (attributable to Froin’s syndrome1) and magnetic resonance imaging (MRI) was highly suggestive of leptomeningeal carcinomatosis and cerebral metastases secondary to disseminated malignant melanoma. The disease progressed with rapid deterioration despite high dose corticosteroids and the patient died 12 days after admission. This case brings several key points to the attention of the acute physician, in particular the need to give serious consideration to the differential diagnosis of cancer in a patient presenting with lower limb weakness.

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