Radiologic grading scores enhance clinical model's prognostic ability for Guillain-Barré syndrome.

OBJECTIVE: To assess the value of magnetic resonance imaging (MRI) grading scores based on lumbosacral muscle denervation edema in predicting the course of Guillain-Barré syndrome (GBS). METHODS: We collected data from 354 GBS patients and developed MRI grading criteria (5-point scale) based on the transverse area and longitudinal length of lumbosacral edema. Univariate and multivariate logistic regression analyses were conducted to identify factors associated with GBS prognosis among 12 demographic and radiological features. Clinical models and clinical-MRI models were separately trained and validated by data from Institution 1. External test was performed using data from Institution 2. Differences between the models were assessed using the z-test. RESULTS: Four clinical factors (sex, albumin cytological dissociation in cerebrospinal fluid, medical research council [MRC] sum score at admission, and MRC sum score at discharge [odds ratio, 0.24-5.15; all p < 0.001]) and MRI grading scores (odds ratio, 2.44; p < 0.001) are independent prognostic factors for GBS patients. The shallow neural network achieved the best prognostic performance both clinical model (accuracy of external test cohort, 83.96%) and clinical-MRI model (accuracy of external test cohort, 90.56%). A significant difference between clinical and clinical-MRI model was also found (clinical model vs. clinical-MRI model, area under the receiver operating curve, 0.84 (95% CI: [0.71, 0.91]) vs. 0.97 (95% CI: [0.86, 0.99]), p < 0.001). INTERPRETATION: The MRI grading scores for muscle denervation edema may serve as a potential prognostic risk factor for GBS. Furthermore, they significantly improve the prognostic performance of standalone clinical model in predicting GBS prognosis.

Molecular, Electrophysiological, and Ultrasonographic Differences in Selected Immune-Mediated Neuropathies with Therapeutic Implications.

The spectrum of immune-mediated neuropathies is broad and the different subtypes are still being researched. With the numerous subtypes of immune-mediated neuropathies, establishing the appropriate diagnosis in normal clinical practice is challenging. The treatment of these disorders is also troublesome. The authors have undertaken a literature review of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain-Barre syndrome (GBS) and multifocal motor neuropathy (MMN). The molecular, electrophysiological and ultrasound features of these autoimmune polyneuropathies are analyzed, highlighting the differences in diagnosis and ultimately treatment. The immune dysfunction can lead to damage to the peripheral nervous system. In practice, it is suspected that these disorders are caused by autoimmunity to proteins located in the node of Ranvier or myelin components of peripheral nerves, although disease-associated autoantibodies have not been identified for all disorders. The electrophysiological presence of conduction blocks is another important factor characterizing separate subgroups of treatment-naive motor neuropathies, including multifocal CIDP (synonyms: multifocal demyelinating neuropathy with persistent conduction block), which differs from multifocal motor neuropathy with conduction block (MMN) in both responses to treatment modalities and electrophysiological features. Ultrasound is a reliable method for diagnosing immune-mediated neuropathies, particularly when alternative diagnostic examinations yield inconclusive results. In overall terms, the management of these disorders includes immunotherapy such as corticosteroids, intravenous immunoglobulin or plasma exchange. Improvements in clinical criteria and the development of more disease-specific immunotherapies should expand the therapeutic possibilities for these debilitating diseases.

Nerve Ultrasound Score in Chronic Inflammatory Demyelinating Polyneuropathy.

Background and Objectives: Studies have suggested that, by applying certain nerve ultrasound scores, demyelinating and axonal polyneuropathies can be differentiated. In the current study, we investigated the utility of ultrasound pattern sub-score A (UPSA) and intra- and internerve cross-sectional area (CSA) variability in the diagnostic evaluation of demyelinating neuropathies. Materials and Methods: Nerve ultrasound was performed in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and acute inflammatory demyelinating polyneuropathy (AIDP) and compared to patients with axonal neuropathies. The UPSA, i.e., the sum of ultrasound scores at eight predefined measurement points in the median (forearm, elbow and mid-arm), ulnar (forearm and mid-arm), tibial (popliteal fossa and ankle) and fibular (lateral popliteal fossa) nerves, was applied. Intra- and internerve CSA variability were defined as maximal CSA/minimal CSA for each nerve and each subject, respectively. Results: A total of 34 CIDP, 15 AIDP and 16 axonal neuropathies (including eight axonal Guillain-Barré syndrome (GBS), four hereditary transthyretin amyloidosis, three diabetic polyneuropathy and one vasculitic neuropathy) were included. A total of 30 age- and sex-matched healthy controls were recruited for comparison. Significantly enlarged nerve CSA was observed in CIDP and AIDP with significantly higher UPSA in CIDP compared to the other groups (9.9 ± 2.9 vs. 5.9 ± 2.0 vs. 4.6 ± 1.9 in AIDP vs. axonal neuropathies, p < 0.001). A total of 89.3% of the patients with CIDP had an UPSA score ≥7 compared to the patients with AIDP (33.3%) and axonal neuropathies (25.0%) (p < 0.001). Using this cut-off, the performance of UPSA in differentiating CIDP from other neuropathies including AIDP was excellent (area under the curve of 0.943) with high sensitivity (89.3%), specificity (85.2%) and positive predictive value (73.5%). There were no significant differences in intra- and internerve CSA variability between the three groups. Conclusion: The UPSA ultrasound score was useful in distinguishing CIDP from other neuropathies compared to nerve CSA alone.

Multiple Cranial Nerve Enhancement in Guillain-Barre Syndrome With Clinicoradiological Dissociation.

ABSTRACT: Guillain-Barre Syndrome (GBS) is an acute inflammatory polyradiculoneuropathy which can lead to rapid neuromuscular respiratory failure, with an estimated annual incidence of 1-2 per 100,000 person-years. Even though cranial nerve involvement is known to occur in GBS, radiological correlation on neuroimaging studies are less frequently reported in pediatric population. We hereby report the case of a 14-year-old boy with acute motor axonal neuropathy variant of GBS, who had extensive contrast enhancement of multiple cranial nerves on Magnetic Resonance Imaging brain, associated with clinicoradiological dissociation on presentation.

Prognostic value of contrast-enhanced MRI in Guillain-Barré syndrome in children.

BACKGROUND: The aim of this retrospective study is to explore the prognostic value of different contrast enhancement imaging patterns in childhood Guillain-Barré syndrome by comparing the clinical, laboratory, and therapeutic outcomes. METHODS: We included a total of 37 patients who were diagnosed and followed up by a pediatric neurology team at Montpellier University Hospital between 2000 and 2016. All images were reinterpreted by the first author and a senior pediatric neuroradiology staff member in two different sessions; in the case of disagreement, the expert's reading was considered. RESULTS: The study group comprised 22 (59.5%) boys and 15 (40.5%) girls. The age ranged from 1.5 year to 14.8 years. Muscle weakness was present in 33 (89.2%) patients. Cranial nerves involvement was observed in 22 (59.5%) patients, while 29 (78.4%) patients had albuminocytological dissociation. In 27 (73%) patients, contrast enhancement or thickening of the lumbosacral nerve roots was found. Simultaneous spinal nerve root and cranial nerve enhancement was noted in five (17.2%) patients, while isolated cranial nerve enhancement was identified in three (10.3%) patients. Clinical and radiological cranial nerve involvement was found in seven (18.9%) patients, while isolated clinical cranial nerves involvement occurred in 13 (35.1%) patients. No significant correlation between different magnetic resonance imaging (MRI) enhancement patterns and short-term or long-term outcomes was found in our cohort. CONCLUSION: Contrast-enhanced brain and spinal MRI is a sensitive and recommended supportive test for diagnosing acute inflammatory polyradiculopathy in children. Its predictive value for clinical, and therapeutic outcomes in the short or long term has not yet been proved.

Nerve ultrasound evaluation of Guillain-Barré syndrome subtypes in northern China.

INTRODUCTION/AIMS: Ultrasound (US) studies have demonstrated patchy enlargement of spinal and peripheral nerves in Guillain-Barré syndrome (GBS). However, whether ultrasound yields useful information for early classification of GBS has not been established. We aimed to evaluate nerve ultrasound in patients with GBS in northern China and compare the sonographic characteristics between demyelinating and axonal subtypes. METHODS: Between November 2018 and October 2019, 38 hospitalized GBS patients within 3 wk of disease onset and 40 healthy controls were enrolled. Ultrasonographic cross-sectional areas (CSA) of the peripheral nerves, vagus nerve, and cervical nerve roots were prospectively recorded in GBS subtypes and controls. RESULTS: Ultrasonographic CSA exhibited significant enlargement in most patients' nerves compared with healthy controls, most prominent in cervical nerves. The CSA tended to be larger in acute inflammatory demyelinating polyneuropathy (AIDP) than in acute motor axonal neuropathy (AMAN)/acute motor and sensory axonal neuropathy (AMSAN), especially in cervical nerves (C5: 5.9 ± 1.6 mm2 vs. 7.0 ± 1.7 mm2 , p = .042; C6: 10.5 ± 1.8 mm2 vs. 12.0 ± 2.1 mm2 , p = .033). The chi-squared test revealed significant differences in nerve enlargement in C5 (p < .001), C6 (p < .001), the proximal median nerve (p < .001), and the vagus nerve (p = .003) between GBS and controls. The vagus nerve was larger in patients with autonomic dysfunction than in patients without it (2.3 ± 1.0 mm2 vs. 1.4 ± 0.5 mm2 , p = .003). DISCUSSION: The demyelinating subtype presented with more significant cervical nerve enlargement in GBS. Vagus nerve enlargement may be a useful marker for autonomic dysfunction.

Sensory Guillain-Barre syndrome following the ChAdOx1 nCov-19 vaccine: Report of two cases and review of literature.

Massive vaccination against COVID-19 has become a global priority. Simultaneously, concerns regarding the safety of vaccines are growing. We describe two patients who developed sensory Guillain-Barre syndrome (GBS) shortly after the first dose of the ChAdOx1 vaccine. We also summarize 12 published cases of GBS after ChAdOx1 vaccination, highlighting their unique clinical and paraclinical features. We propose a possible association between the risk of GBS and the ChAdOx1 vaccine and recommend surveillance for GBS following vaccination. Population-based studies are needed to determine causality and whether specific subpopulations are susceptible.

Guillain-Barré syndrome as only manifestation of COVID-19 infection.

Post-infectious/immune mediated effects of COVID-19 infection include descriptions of Guillain-Barré syndrome (GBS) in patients usually with respiratory failure and after 1-2 weeks from the onset of viral illness. Asymptomatic cases for COVID-19 infection were rarely described. Herein, we studied a 62-year-old patient with progressive weakness of lower extremities, rapidly evolving to a severe, flaccid tetraplegia and dysphagia. Neurological symptoms weren't preceded by fever or pulmonary symptoms. Because of laboratory test abnormalities (thrombocytopenia, lymphocytopenia, high inflammation indexes), the patient underwent to nasopharyngeal swab, resulted positive for SARS-CoV-2 on RT-PCR assay; cerebrospinal fluid (CSF) was negative for SARS-CoV-2. The clinical (severe symmetric distal upper and lower limbs weakness, grade 0/5; decreased proprioceptive sensitivity and hypoesthesia involving the four limbs; loss of deep tendon reflexes), electrophysiological (prevailing axonal polyradiculoneuritis) and CSF features (albumino-cytological dissociation) disclosed the GBS diagnosis (level 1 of diagnostic certainty according to the Brighton criteria). The patient received plasma exchange and immunoglobulin, and, at 4 weeks after treatment and physical therapy, the patient had moderate improvement (weakness at lower and upper extremities was grade 2/5 and 3/5, respectively). Neurologists and clinicians should be aware of the possible link between neurological symptoms and COVID-19 infection, not only after viral prodrome and pulmonary symptoms, but also without COVID-19 symptoms.

Anti-GM1 IgM antibody positive axonal variant of Guillain-Barre-syndrome in a pediatric patient with dengue fever.

INTRODUCTION: While children of all ages may be affected by Guillain-Barre-Syndrome (GBS), there are no reports of Dengue Fever (DF) as the preceding or concurrent infection in this age group. In addition, the presence of anti-GM1 IgM antibody, commonly seen in Multifocal Motor Neuropathy, is rarely encountered in both axonal and demyelinating variants of GBS. Moreover, only few neuromuscular ultrasound findings of the axonal variant in children were reported in the literature. CASE: Here we present a nine-year-old female who developed the classic signs, symptoms and neurophysiologic findings of axonal type of GBS during DF. She had elevated anti-GM1 IgM antibody atypical of this variant and diffusely enlarged nerves via neuromuscular ultrasound. CONCLUSION: In a pediatric patient with DF and acute flaccid paralysis, GBS should always be one of the considerations. Although rare, anti-ganglioside GM1 IgM antibody can still be found in axonal variant of GBS.

Immune-Mediated Neuropathies.

The immune-mediated neuropathies are a broad category of diseases differentiated by time course, affected nerve fibers, and disease associations. This article spans the common, well-defined inflammatory demyelinating polyradiculoneuropathies (Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy) to the rarer, acquired demyelinating neuropathy variants (Miller-Fisher syndrome and multifocal motor neuropathy), vasculitic neuropathies, and sensory neuronopathies (dorsal root ganglionopathies). These case studies illustrate the characteristic clinical patterns of the immune-mediated neuropathies encountered in neurologic practice. Recommendations for diagnostic evaluation and treatment approach accompany each case. Prompt recognition of these disorders is imperative; delays in treatment may result in prolonged morbidity and permanent disability.

Guillain-Barré syndrome associated with leptomeningeal enhancement following SARS-CoV-2 infection.

INTRODUCTION: Patients with coronavirus disease 2019 (COVID-19) typically present with respiratory symptoms, but little is known about the disease's potential neurological complications.We report a case of Guillain-Barré syndrome (GBS) following a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, in association with leptomeningeal enhancement. CASE PRESENTATION: A 56-year-old woman presented with recent unsteadiness and paraesthesia in both hands. Fifteen days earlier, she complained of fever, dry cough and shortness of breath. Her chest X-ray showed a lobar consolidation and PCR was positive for SARS-CoV-2; she was admitted due to mild COVID-19 pneumonia.In the first 48 hours of hospitalisation, she started to experience lumbar pain and weakness of the proximal lower extremities, progressing to bilateral facial nerve palsy, oropharyngeal weakness and severe proximal tetraparesis with cervical flexion 2/5 on the MRC scale. Full spine magnetic resonance imaging (MRI) showed a brainstem and cervical leptomeningeal enhancement. Analysis of cerebrospinal fluid (CSF) revealed albumin-cytological dissociation. Microbiological studies on CSF, including SARS-CoV-2, were negative. Nerve conduction studies were consistent with demyelinating neuropathy. She was treated with intravenous immunoglobulin, with significant neurological improvement noted over the next 2 weeks. CONCLUSION: Leptomeningeal enhancement is an atypical feature in GBS, but could be a marker of its association with SARS-CoV-2 infection.

Posterior reversible encephalopathy syndrome associated with Guillain-Barré syndrome: Case report and clinical management considerations.

Around half of the patients with Guillain-Barré syndrome (GBS) present autonomic dysfunction requiring admission to intensive care unit in up to a quarter of patients. Treatment of GBS consists of plasma exchange (PE) and intravenous immunoglobulins (IVIG). Posterior reversible encephalopathy syndrome (PRES) consists in a reversible subcortical vasogenic brain edema caused by endothelial damage triggered by abrupt blood pressure changes. We report on a woman who presented with PRES in the course of GBS treated first with IVIG, and then with PE. The present report underlines the challenge that the clinicians face when these two rare syndromes concur. The literature is not helpful considering that both blood pressure fluctuations and IVIG are reported to be involved in the pathogenesis of PRES. In the present letter, both pathogenic mechanisms and clinical management considerations are discussed.