Acute Polyradiculoneuropathy Research Articles

The mysterious death of Georges Cuvier (1832): An early case of severe Guillain–Barré syndrome?

Although Guillain-Barré syndrome was officially described in 1916, other cases had been reported earlier, such as some cases of Landry's paralysis. This year is the 250th anniversary of the birth of Georges Cuvier (1769–1832), one of the fathers of comparative anatomy and palaeontology: he died at age 63 from an unknown disease. By reading medical reports about his last days and hours, we conclude Cuvier died from a severe form of Guillain-Barré syndrome. Moreover, we think this observation could be the first complete report of acute polyradiculoneuropathy with pharyngeal-cervical-brachial onset.

A guillain-barre syndrome revealing cutaneous leishmaniasis in an immunocompetent adolescent

Guillain-Barre Syndrome (GBS) is an acute autoimmune polyradiculoneuropathy, that often follows an antecedent infection. Leishmaniasis is a zoonosis due to a flagellate protozoan of the Leishmania genus and transmitted by the sandfly. Here we report a 15 years old teenager who presented with an ascending installation of the motor deficit in three weeks associated with dysphonia and swallowing disorders. The physical examination showed a flaccid proximo-distal quadriparesis, akinesthesia, apallesthesia and cutaneous lesions on the forehead, wrist and calf that appeared two months earlier. The HIV serology was negative and cerebro-spinal fluid examination was normal. The electroneuromyography showed a reduction in motor and sensitive amplitudes with an increase in distal latencies and F waves in the upper limbs and segmental and focal conduction blocks. Parasitological examination of the dermal juice around the edges of the skin lesions revealed amastigote forms of leishmaniasis. The evolution was favorable with only symptomatic treatment.

Advance on immunotherapy and prognosis of Guillain-Barré syndrome

Guillain-Barre syndrome (GBS) is an acute onset immune-mediated polyradiculoneuropathy, and it is the most frequent cause of acute flaccid paralysis.Intravenous immunoglobulin and plasma exchange are proved to be an effective treatment.Despite the efficacy of immunotherapy, a significant number of patients have a poor prognosis, leaving behind severe dysfunction and even death.Therefore, biomarkers and prognostic models are needed to accurately predict outcome, which could help the early recognition of patients with poor outcomes, which may potentially be-nefit from individualized treatments.So, new development of immunotherapy and prognostic evaluation of GBS were summarized in this paper, in order to provide the evidence for clinical diagnosis and treatment. Key words: Guillain-Barre syndrome; Complement inhibitor; Albumin; Prognosis

Guillain-Barre syndrome

Guillain-Barre syndrome is the most common and most severe acute flaccid paralysis, usually preceded by an infection or immune stimulation. A literature review of the last five years in Pubmed, Scopus, Embase, and Web of Science databases was done. Data were collected from epidemiological studies using the keywords. Guillain-Barre syndrome (GBS) is an acute, severe, and fulminant polyradiculoneuropathy. It has ascending muscle weakness, rapid evolution, the involvement of respiratory muscles with an evolution period of up to four weeks. It has the Classic and Variant forms of clinical presentation. Guillain-Barre Syndrome is a rapidly progressive disease. The diagnosis is clinical but requires exams such as electroneuromyography and cerebrospinal fluid analysis, so that treatment can be started as soon as possible to improve prognosis and increase survival of affected patients.

Clinical study of the Guillain-Barré Syndrome

Guillain-Barre Syndrome (GBS) is an acute demyelinating neuropathy characterized by progressive generalized paralysis with areflexia. To study the clinical characteristic and outcome of patient with Guillain-Barre syndrome. It was a retrospective analysis conducted in the Department of Neurology, Third Central Hospital of Mongolia from January 2014 to December 2018. We assessed 10 patients history with GBS by Brighton criteria out of 39 patients who diagnosed with the acute polyradiculoneuropathy. The gender ratio was 1: 1, the average age was 57.4 and antecedent illness preceding diarrhea was 60% and 1 case was the upper respiratory tract infection. All had progressive paralysis with areflexia, 90% had sensory loss, 20% had cranial nerve palsies and 1 case had respiratory paralysis with ventilation. The GBS subtype distribution as electro diagnostic studies was as follows: acute motor sensory axonal neuropathy (AMSAN) in 2 (20%), acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 7 (70%) and equivocal in 1 (10%). Steroids constituted the treatment given in majority of the patients (90%), 2 patients transferred to another hospital to having plasma exchange and intravenous immune globulins (IVIG) was given to 3 cases. Most common antecedent event and presenting feature were diarrhea and progressive paralysis, respectively. Intravenous immune globulins and plasma exchange need to be implemented even further in our practice.

Guillain –Barre syndrome AND central nervous system involvement: Two case reports

Although Guillain Barre Syndrome (GBS) is generally considered as a peripheral neuropathy, there is evidence of central impairment explaining the observed atypies. We report the case of two patients who had a GBS with hyperreflexia and a Babinski sign. Two patients (23 and 70 years old) had simultaneous 4-limb weakness in less than 6 hours (aged patient) and 6-day ascending motor deficit (young). The examination revealed a tetraplegia with hyperreflexia in both patients with a Babinski sign (young patient ). Facing tetraplegia associated with an albumino-cytological dissociation in the cerebrospinal fluid (young), the normality of the spinal imaging as well as the electrophysiological results in favor of an acute motor axonal polyradiculoneuropathy, the diagnosis of pure motor GBS (AMAN) was retained.Immunoglobulins were introduced with a favorable evolution (young). The elderly patient developed on the first day a respiratory distress requiring ventilatory support despite the early treatment. Our patients had a GBS with hyperreflexia and a Babinski sign requiring spinal imaging to eliminate a central lesion that could mimic these symptoms. In fact, in the pure motor forms (AMAN) and pharyngo-cervico-brachial syndrome (variant of GBS), the osteotendinous reflexes can be present even increased with clonus suggesting an associated central nervous system involvement. The clinical atypia of the GBS are more and more described. Their lack of knowledge could delay the early management.

Cardiovascular complications and its relationship with functional outcomes in Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a monophasic disease characterized by acute polyradiculoneuropathy. This study investigated cardiovascular complications in patients with GBS and their relationship with outcomes. We included 96 patients, who were diagnosed with GBS according to Brighton case definitions. All enrolled patients were evaluated according to a predetermined algorithm, which included nerve conduction studies, cerebrospinal fluid analysis, electrocardiography, 2D echo, cardiac markers and autonomic function testing. We enrolled a total of 96 patients. The mean age of patients was 35.75 ± 17.66 years. Furthermore, 54.2% of patients developed cardiovascular complications, of which changes in electrocardiography (ECG) findings (50%), hypertension (28.12%), labile hypertension (12.5), tachycardia (26.04), bradycardia (13.54%) and a fluctuating heart rate (HR) (11.46) were common. Other cardiovascular complications seen in GBS patients were increased pro-BNP (26.04%), raised troponin T levels (3.12%), acute coronary syndrome (2.08%), heart failure (2.08%) and abnormal 2D echo findings (8.33%). The results of the univariate analysis revealed that a history of preceding infection, a Medical Research Council sum score, neck muscle weakness, facial nerve involvement, bulbar involvement, respiratory failure, cardiovascular complications, autonomic dysfunction, acute motor sensory axonal neuropathy subtype and baseline Hughes score were significantly (P < 0.005) associated with poor outcomes. However, none of these factors were found to be independently associated with poor outcomes in the multivariate analysis. A considerable number of patients with GBS developed cardiovascular complications and it needs attention.

Guillain-Barr\xe9 syndrome with associated unilateral ptosis without ophthalmoplegia \u2013 a rare presentation: a case report and review of the literature

BackgroundGuillain-Barré syndrome is an acute inflammatory polyradiculoneuropathy. Nearly half of patients with Guillain-Barré syndrome have cranial nerve involvement. However, isolated bilateral ptosis without ophthalmoplegia is a rare manifestation, and isolated unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome has not previously been reported in the literature. Furthermore, only few cases of Guillain-Barré syndrome with cranial nerve enhancement visualized by gadolinium-enhanced magnetic resonance imaging have previously been reported. We describe the first reported case of unilateral ptosis without ophthalmoplegia in Guillain-Barré syndrome and associated multiple cranial nerve enhancement seen by gadolinium-enhanced magnetic resonance imaging.Case presentationOur patient was a 55-year-old Sinhalese man who was admitted to a tertiary care hospital in Sri Lanka with acute-onset progressive weakness in the lower limbs followed by the upper limbs. He had bilateral symmetrical flaccid quadriparesis with absent reflexes and flexor plantar response. Left-sided isolated partial ptosis without associated ophthalmoplegia was noted with normal pupils. The patient’s neurological examination was otherwise normal. A nerve conduction study showed a severe demyelinating type of polyneuropathy. No decremental response to repetitive nerve stimulation was observed, and the result of a single-muscle-fiber electromyogram was negative. A diagnosis of Guillain-Barré syndrome was made, and the patient was treated with intravenous immunoglobulin. His condition gradually deteriorated over the next few days, and he became quadriplegic despite the completion of immunoglobulin therapy. Later he developed multiple cranial nerve palsies, including bi-lateral lower motor neuron type facial nerve palsy, and he required mechanical ventilation. By this time, he had complete left-sided ptosis with a normal right eye. He never developed ophthalmoplegia or ataxia. Magnetic resonance imaging of the brain showed contrast enhancement in the intracranial part of multiple cranial nerve roots and basal leptomeninges. He gradually improved with plasmaparesis, and ptosis was the first to improve.ConclusionsEven though Guillain-Barré syndrome was recognized a century ago, there are still many unanswered questions about it and its florid presentation. Large-scale studies are needed for better understanding of its pathophysiology and prototypes and to find answers for still-unanswered questions. The clinician must have a high index of suspicion and be familiar with mimics and prototypes to diagnose Guillain-Barré syndrome accurately without delay.

Inflammatory demyelinating polyneuropathies in children

There are presented data on clinical variants and peculiarities of the course of acute demyelinating inflammatory polyneuropathy in children. This disease is the acute post-infectious immune-mediated polyradiculoneuropathy characterized by the destruction of myelin ofperipheral nerves and manifested by the syndrome of symmetrical polyneuropathy with motor, sensor, autonomous disturbances and albuminocytologic dissociation in cerebrospinal fluid. This disease is known to be commonly developed during 2-4 weeks with following stage of stabilization and then improvement. There are considered issues of etiology and pathogenesis of Guillian-Barre syndrome, with the accents on pediatric aspects. There are given data on chronic demyelinating inflammatory polyneuropathy andM. Fischer syndrome also. There are described neurophysiological criteria for demyelinating disorders in children and considered issues of their management.

Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy, and pathophysiologically classified into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor and sensory axonal neuropathy (AMSAN). The main pathophysiological mechanism is complement-mediated nerve injury caused by antibody-antigen interaction in the peripheral nerves. Antiglycolipid antibodies are most pathogenic factors in the development of GBS, but not found in 40% of patients with GBS. One of the principal target regions in GBS is the node of Ranvier where functional molecules including glycolipids are assembled. Nodal dysfunction induced by the immune response in nodal axolemma, termed "nodopathy," can electrophysiologically show reversible conduction failure, axonal degeneration, or segmental demyelination. To detect new target molecules in antiglycolipid antibody-negative GBS and to elucidate the pathophysiology in the subacute and the subsequent phases of the disorder are the next problems.

Guillain–barre syndrome as a presenting feature of systemic lupus erythematosus in a child and it's complete resolution with rituximab treatment

Systemic lupus erythematosus (SLE) can present with varied neurological manifestations in children. Guillain–Barre syndrome (GBS) as a presenting feature of SLE is quite rare in children. We report a 9-year-old girl who presented with acute motor axonal polyradiculoneuropathy and noticed to have features of SLE. She was initially treated with intravenous immunoglobulins and corticosteroids with partial response. After starting rituximab, she showed dramatic recovery, proving the role of rituximab in GBS refractory to first-line medications in SLE. She remained in remission after 12 months of follow-up.

Severe Guillain-Barré Syndrome Following Shingrix® Vaccine Administration

Guillain-Barre syndrome (GBS) is a rare life threatening acute polyradiculoneuropathy with variable clinical presentations, including weakness, paresthesias, diminished deep tendon reflexes, hyponatremia, dysautonomia, and a cytoalbuminological dissociation on cerebral spinal fluid (CSF) analysis. The exact etiology remains unknown but is thought to occur as a post-infective immunemediated process. Vaccines and illnesses, including H1N1 Swine Flu vaccine, pneumococcal vaccine, Campylobacter jejuni bacteria, cytomegalovirus, and herpes zoster virus, have been associated with GBS. One vaccine that has not been identified to cause GBS is the newly released Shingrix® vaccine for herpes zoster infection prevention. We present a case of severe GBS following Shingrix® vaccine immunization.

Increased incidence of axonal Guillain-Barré syndrome in La Spezia area of Italy: A 13-year follow-up study.

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy with a worldwide incidence of 0.81-1.89 per 100 000 person-years. In Europe and North America only 5% of patients with GBS have axonal subtypes, which in South America and Asia account for 30%-47% of cases. The aim of our study is to assess the annual incidence and clinical features of GBS in La Spezia area in Italy. A retrospective (from 1 January 2003 to 31 December 2011) followed by a prospective (from 1 January 2012 to 31 December 2015) analysis was carried out on patients admitted to La Spezia hospital who fulfilled the GBS diagnostic criteria. A total of 86 patients (58 men), mean age of 62.7 years (range 21-90), were included. The mean annual incidence rate was 3/100 000 (range: 0.9/100 000-5.37/100 000) significantly higher than the European incidence (P < 0.001). Forty-seven percent were classified as acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 35% as acute motor and motor-sensory axonal neuropathy (AMAN-AMSAN), 13% as variant forms, and 5% were not defined. AIDP was most common in "Golfo dei Poeti" (50%) and "Val di Magra" (63.2%), whereas AMAN/AMSAN prevailed in "Val di Vara" (63.6%) and "Riviera Spezzina" (62.5%) (P = 0.024). In La Spezia area GBS incidence (especially the AMAN subtype) is significantly higher than the incidence reported in Europe. AIDP predominates in the eastern area whereas AMAN/AMSAN in the western, with a significantly different incidence rate (P = 0.003). Prospective studies to assess possible predisposing environmental factors are needed.

Guillain-Barré Syndrome and Low Back Pain: Two Cases and Literature Review.

Purpose. To describe the clinical, electrophysiological, and lumbar magnetic resonance imaging (MRI) features of two cases of atypical Guillain-Barré syndrome (GBS). Methods We reported two GBS variant cases with initial and prominent symptoms of low back pain. We analysed their clinical, electrophysiological, and lumbar MRI features. Results Two patients with GBS reported low back pain as the initial and prominent symptom, which was not accompanied by limb weakness. The electrophysiological study showed abnormal F-waves in the common peroneal and tibial nerves, and acute polyradiculoneuropathy in the cauda equina. Examination of the cerebrospinal fluid (CSF) showed albuminocytologic dissociation. Serum was positive for GQ1b-IgM antibodies. Lumbar MRI showed gadolinium enhancement of the nerve roots and cauda equina. A standard regime of intravenous immunoglobulin markedly alleviated the low back pain. Conclusions Low back pain caused by GBS should be differentiated from other diseases. This initial or early prominent symptom may delay the diagnosis of GBS; therefore, it is important to conduct a detailed electrophysiological, CSF, and gadolinium-enhanced lumbar MRI analysis.

Bilateral Third Nerve Paralysis as a Manifestation of Guillain–Barré Syndrome

Gullian–Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy with many variants and distinct presentations. Although cranial neuropathy is a common feature in GBS, third nerve palsy is a rare presentation. Herein, we describe a case of GBS patient who has presented by acute flaccid quadriparesis coexisting bilateral third nerve palsy. We tried to highlight the importance of other cranial nerve involvement in the natural history of GBS.

Incidence of Guillain–Barre's Syndrome in Children under 15 Years of Age in Oman

Objective Guillain–Barre's syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy seen in all ages but mostly in the adult population. We aim to report the incidence of GBS in children under 15 years of age in Oman, a Gulf country. Materials and Methods All children with GBS under 15 years of age were included in the study from January 2002 to December 2016. The data were compiled in Microsoft Excel format and analysis was performed using SPSS, version 17.0. Population-based age- and sex-specific rates of GBS were calculated using the estimated population for each 5-year period based on 2004, 2009, and 2014 mid-year population, respectively. Relative risk and 95% confidence intervals of GBS at different age categories were calculated using the overall incidence of GBS in those under 15 years as reference. Results The average annual incidence in those under 15 years was 3.1/100,000. Age-specific incidence was 4.0/100,000 in the age group 0 to 9 years and 4.7/100,000 in age group 0 to 4 years. Sixty percent had evidence of preceding infections, and the male to female ratio was 1.8:1. Fifty percent had cranial nerve involvement and 18% needed mechanical ventilation. Five percent had residual weakness. Intravenous immunoglobulins (IVIGs) were used in all, and 3.3% required plasmapheresis when they did not improve with IVIG. Clinical profile of the GBS was not different from the rest reported in the literature. Conclusion The incidence of GBS in Oman was 3.1/100,000 (range: 2.7–3.5 cases/100,000) in children under 15 years of age. The GBS is a smaller proportion among the total acute flaccid paralysis cases.

F30. Axonal variants of Guillain Barre Syndrome are predominant among nepalese population: A tertiary care center study

Introduction Guillain Barre Syndrome (GBS) is an acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature. Our aim is to classify GBS subtypes and evaluate the clinical severity. Methods All consecutive patients recruited prospectively in the study were of age ⩾ 16 years and were being admitted in department of Neurology of Tribhuvan University Teaching Hospital, Kathmandu, Nepal from 2016 March to 2017 February. All demographic, historical and clinical data were collected. Nerve conduction study, cerebrospinal fluid analysis along with clinical features were evaluated to assess the diagnostic certainty of Brighton Criteria. Overall disability sum score and GBS disability score were assessed in all patients. Results A total of 46 patients were included: male patients being predominant (70% vs 30%), mean age = 36.5 ± 16.2, range = 16–80 years. Thirty-two patients (70%) were axonal variant, acute motor axonal neuropathy being more common (18 patients), and 14 patients were acute motor and sensory axonal neuropathy. 14 patients (30%) were demyelinating type, out of which 11 patients had both motor and sensory features, and only 3 patients were pure motor demyelinating type. Axonal variants were found to have higher Overall disability sum score during nadir (p = 0.024) and discharge (p = 0.004), and also higher GBS disability score during nadir (p = 0.012) and discharge (p = 0.021). Acute motor axonal neuropathy (AMAN) had higher GBS disability score than acute inflammatory demyelinating polyneuropathy (AIDP) at nadir (p = 0.034) and discharge (p = 0.039). Conclusion AMAN subtype are predominant among Nepalese population. Axonal variants of GBS are clinically severe than demyelinating variants.

BS21. Neurophysiological assessment of autonomous nervous system in patients with Guillain–Barré syndrome in Brazil

Guillain–Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy with a high risk of clinical and subclinical autonomic dysfunction even in early stages of evolution, which carries an increase in their morbidity and mortality. In our study, we evaluated the incidence of early and subclinical autonomic disturbance in patients with GBS in a brazilian university center. We evaluated 14 patients with GBS confirmed by the criteria of Brighton and Hadden for demyelinating GBS (AIDP) or axonal GBS (AMAN), admitted in the neurology department of University of Sao Paulo Medical School, throughout the year 2016. The cases were also classified by Erasmus’s gravity score at admission. Autonomic evaluation was conducted in the first four weeks of the onset of symptoms, with patients at rest, without autonomic interfering drugs, just before nerve conduction studies. Autonomic studies consisted of sympathetic skin response (SSR) elicited by electric stimulation and heart rate variability (HRV) during deep breathing maneuver for one minute (expiration: inspiration ratio) and spectral analysis of a five minutes long recording during spontaneous breathing. Two out of ten patients with AIDP (20%) and two out of four patients with AMAN (50%) presented evidence of autonomic dysfunction at the time of the evaluation. These four patients presented an Erasmus score greater than or equal to 4 on the occasion and evolved with clinical dysautonomia during hospitalization. HRV was abnormal in three of these cases. SSR was absent in only one case. No patient with an Erasmus score less than 4 presented neurophysiologic evidence of autonomic dysfunction, but three out of ten (30%) eventually evolved with mild clinical dysautonomia during hospitalization. General sensitivity of neurophysiologic evaluation was 57%. No patient died or suffered severe dysautonomia in our sample. HRV and SSR were able to detect early dysautonomia in patients with GBS, especially in those with Erasmus score greater than or equal to 4. HRV seems to be more sensitive than SSR.

Anti-MAG antibodies in 202 patients: Clinicopathological and therapeutic features

The purpose of this study was to assess the clinicopathological and therapeutic features of patients with low (≥ 1000– ® Titre Units) (BTU), medium (10 000–70 000) or high (≥ 70 000) anti-Myelin-Associated Glycoprotein (MAG) antibody titres. Methods We retrospectively and prospectively analysed standardized report forms and medical records of two hundred and two patients from fourteen neuromuscular centres. Results Mean age at onset and mean time between symptom onset to last follow-up were respectively 62.6 years (25–91.4) and 8.4 years (0.3–33.3). Anti-MAG antibody titres at diagnosis were low, medium or high in 11, 51 and 38% of patients. Patients presented with monoclonal gammopathy of undetermined significance (MGUS) in 68% of cases. About 17% of patients presented with “atypical” clinical phenotype independently of anti-MAG titres, including acute or chronic sensorimotor polyradiculoneuropathies (12.4%), and asymmetric or multifocal neuropathy (3%). At the most severe disease stage, 22.4% of patients were significantly disabled. Seventy eight percent of patients received immunotherapies. Transient clinical worsening was observed in 12% of patients treated with rituximab (11/92). Stabilisation after rituximab treatment during the 7–12-month follow-up period was observed in 29% of patients. Clinical response to rituximab during the 6-month and/or 7–12-month follow-up period was observed in 31.5% of patients, and correlated with anti-MAG titre ≥ 10 000 BTU. Our study highlights the extended clinical spectrum of patients with anti-MAG neuropathy, which appears unrelated to antibody titre. Besides, it may also suggest beneficial use of rituximab in the early phase of anti-MAG neuropathy.

Guillain-Barré syndrome, transverse myelitis and infectious diseases.

Guillain-Barré syndrome (GBS) and transverse myelitis (TM) both represent immunologically mediated polyneuropathies of major clinical importance. Both are thought to have a genetic predisposition, but as of yet no specific genetic risk loci have been clearly defined. Both are considered autoimmune, but again the etiologies remain enigmatic. Both may be induced via molecular mimicry, particularly from infectious agents and vaccines, but clearly host factor and co-founding host responses will modulate disease susceptibility and natural history. GBS is an acute inflammatory immune-mediated polyradiculoneuropathy characterized by tingling, progressive weakness, autonomic dysfunction, and pain. Immune injury specifically takes place at the myelin sheath and related Schwann-cell components in acute inflammatory demyelinating polyneuropathy, whereas in acute motor axonal neuropathy membranes on the nerve axon (the axolemma) are the primary target for immune-related injury. Outbreaks of GBS have been reported, most frequently related to Campylobacter jejuni infection, however, other agents such as Zika Virus have been strongly associated. Patients with GBS related to infections frequently produce antibodies against human peripheral nerve gangliosides. In contrast, TM is an inflammatory disorder characterized by acute or subacute motor, sensory, and autonomic spinal cord dysfunction. There is interruption of ascending and descending neuroanatomical pathways on the transverse plane of the spinal cord similar to GBS. It has been suggested to be triggered by infectious agents and molecular mimicry. In this review, we will focus on the putative role of infectious agents as triggering factors of GBS and TM.