Autoimmune Polyradiculoneuropathy Research Articles

Frequency of exposure to arboviruses and characterization of Guillain Barré syndrome in a clinical cohort of patients treated at a tertiary referral center in Brasília, Federal District.

ABSTRACTBackground: Guillian Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy often associated with previous exposure to infectious agents. Methods: A clinical cohort of 41 patients with GBS admitted to the Base Hospital Institute of the Federal District between May 2017 and April 2019 was followed up for 1 year. Serological tests for arbovirus detection and amplification of nucleic acids using polymerase chain reaction for zika virus (ZIKV), dengue virus (DENV), and chikungunya virus (CHIKV) were performed. Results: The cohort consisted of 61% men with a median age of 40 years, and 83% had GBS-triggering events. A total of 54% had Grade 4 disability, 17% had Grade 3, 12% had Grade 2, 10% had Grade 5, and 7% had Grade 1. The classic form occurred in 83% of patients. Nerve conduction evaluations revealed acute demyelinating inflammatory polyneuropathy (51%), acute motor axonal neuropathy (17%), acute sensory-motor neuropathy (15%), and indeterminate forms (17%). Four patients were seropositive for DENV. There was no laboratory detection of ZIKV or CHIKV infection. Ninety percent of patients received human immunoglobulin. Intensive care unit admission occurred in 17.1% of the patients, and mechanical ventilation was used in 14.6%. One patient died of Bickerstaff’s encephalitis. Most patients showed an improvement in disability at 10 weeks of follow-up. Conclusions: GBS in the Federal District showed a variable clinical spectrum, and it was possible to detect recent exposure to DENV.

Incidence and mortality rates of Guillain-Barré syndrome in Serbia.

Guillain-Barré syndrome (GBS) is an acute auto-immune polyradiculoneuropathy. A huge variety of GBS incidence and mortality rates has been noted across the world. The objective of the present multi-centric study was to assess the incidence and mortality rates of GBS during a 10-year period in Serbia. We collected data of adult GBS patients who were hospitalized from 2009 to 2018 in all five tertiary healthcare centers in Serbia. The incidence rates per 100 000 inhabitants with 95% confidence intervals (CI) were calculated and further corrected for the estimated number of patients hospitalized in secondary centers. Mortality rates were also assessed. GBS was considered severe if patients were not able to walk at least 10 m without assistance. Six hundred and forty GBS patients were registered in tertiary centers in a 10-year period. The proportion of severe cases was 75% at nadir, and 52% on discharge. GBS incidence rate in Serbia was 1.1 per 100 000 inhabitants, and estimated incidence if patients from secondary centers included 1.2 per 100 000. Peak incidence was observed during the sixth decade of life. During the acute phase, 5.6% of GBS patients died, while overall 9.7% of them died during 6-month period from disease onset. This study contributes to our knowledge about GBS epidemiology. Results will allow us to improve the diagnosis and treatment of GBS patients in Serbia.

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.

Characterization of Patients with Guillain-Barré Syndrome in the General Hospital of Mexicali

Background: Guillain-Barré Syndrome is a progressive autoimmune polyradiculoneuropathy characterized by symmetrical flaccid paralysis accompanied by areflexia, hyporeflexia or hyperreflexia on rare occasions. Worldwide, it remains the first cause of flaccid paralysis. It is usually associated with infectious disease history; however, there are various clinical variants, each with a different outcome. Prognosis is usually good, although 20% of patients could suffer a severe clinical variant of Guillain-Barré Syndrome and 5% will die despite treatment.
 Methods: This is a cross-sectional study, including the records of hospitalized patients with Guillain- Barré Syndrome at Mexicali’s General Hospital within a five-year period.
 Results: In a five-year span there were 64 patients with Guillain-Barré Syndrome, most of the patients were men (70.3%), with age ranging 1 to 76 years. A total of 8 (12.5%) patients died, from which 7 (87.5%) required mechanical ventilation during hospitalization. Immunoglobulin therapy was provided to 56 (87.5%) patients, and 6 (10.7%) of them perished due to acute kidney injury.
 Conclusion: Guillain-Barré Syndrome is a common disease among male population, with no dominating onset age, however, leaning for the young and elder. Most of the patients that were hospitalized at the General Hospital of Mexicali had a history of previous infection (gastrointestinal, respiratory, or other infectious diseases). The need for mechanical ventilation represents a higher severity index, nonetheless, this does not mean that assisted ventilation is directly associated with mortality. As for treatment, immunoglobulin is the most common choice for therapy, though some of the patients died from acute kidney injury.

Prognostic Value of "A" Waves in Patients With Guillain Barre Syndrome.

The "A" wave is a compound action potential evoked consistently from a muscle by submaximal electric stimulation to the nerve. "A" waves were usually associated with the presence of conduction block and abounded in lower limbs more than in upper limbs. Guillain Barre syndrome is an autoimmune polyradiculoneuropathy that usually follows respiratory or gastrointestinal infections. To study the prognostic value of "A" waves in patients with Guillain Barre syndrome with different recovery patterns. In this study, 50 patients with GuillainBarreSyndrome were enrolled. They were divided into two groups based on the presence or absence of "A" waves in their neurophysiologic study at onset of illness. Strength of limb muscles of the involved patients was assessed at onset of the study and 6 weeks later (short-term clinical recovery) using the Medical Research Council Scale. Patients with waves (29 patients: 58%) showed poorer short-term follow-up parameters than those without "A" waves (21 patients: 42%). Presence of "A" waves is usually associated with conduction block and marked degree of demyelination. "A" waves carry poor prognostic value in short-term follow-up parameters in patients with Guillain Barre syndrome.

An Overview of Guillain-Barré Syndrome

Guillain-Barré Syndrome (GBS) is an acute, autoimmune polyradiculoneuropathy that carries great patient morbidity, and significant mortality, worldwide. The manifestations are highly heterogeneous at the clinical, electrophysiological and biochemical levels, which means that it is better to conceptualise GBS as a spectrum of disorders rather than a singular one. Despite the diverse range of presentations, the management of GBS is relatively stereotyped, albeit guided by the level of clinical severity. Treatment is largely restricted to general supportive measures, Intravenous Immunoglobulin (IVIG) and Plasma Exchange (PLEX), with no current role for oral or intravenous corticosteroids in clinical practice. Several validated prognostic-scoring systems, which can predict the probability of long-term residual disability, may assist in targeting intensive therapies to high-risk patient groups. The aim of this article is to provide a practical overview of GBS, with particular emphasis on the clinical presentation, investigation and management of this important spectrum of neurological conditions.

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.

Guillain Barre Syndrome Associated with Brucellosis: A Case Report and Review of the Literature

Introduction: Guillain-Barre syndrome (GBS) ranks as the most frequent cause of acute flaccid paralysis in the world. It is an autoimmune polyradiculoneuropathy, usually preceded by an acute infection. Rarely, brucellosis may induce a GBS.Objective: To evaluate the clinical and microbiological diagnostic properties of Brucella-induced GBS.Case Report: A 54-year-old woman, with no past medical history, was followed in infectious disease department. She was diagnosed with brucellosis. She had received antibiotic therapy (Rifampicin 600 mg/day and Doxycycline 200 mg/day). After 4 days of treatment, she was referred to our department because of rapidly progressive, ascending, symmetric weakness and bilateral paralysis of muscles of the face. On admission, she was alert. The deep tendon reflexes (DTRs) were absent in all extremities. Muscle strength was 3/5 in the upper extremities and 2/5 in the lower extremities. Proprioception in the lower extremities was impaired, but she did not have any sensory problems. Our patient also presented a facial diplegia. Physical examination was normal, except for splenomegaly. A lumbar puncture showed an albumin-cytologic dissociation in the CSF. Nerve-conduction studies were suggestive of demyelinating polyradiculoneuropathy. Coombs Wright titration was 1/160..Discussion: With a diagnosis GBS preceded by brucellosis, our patient was given an antibiotic therapy (Rifampicin 600 mg/day and Doxycycline 200 mg/day). During hospitalization, she had four plasma exchange sessions and a motor rehabilitation. In a follow-up after 4 weeks, our patient presented a partial recovery, and she was able to walk without support.Conclusion: This case demonstrates that brucellosis can present with a rare neurologic manifestation including GBS. Molecular mimicry seems to be responsible for this complication, through the synthesis of autoantibodies against myelin gangliosides. Thus, brucellosis should be ruled out in all patients who develop acute flaccid paralysis, especially in those who live in endemic areas.

CD1A and CD1E gene polymorphisms are not associated with susceptibility to Guillain-Barré syndrome in the Bangladeshi population

The post-infectious autoimmune polyradiculoneuropathy Guillain-Barré syndrome (GBS) is triggered by molecular mimicry between microbial glycolipid antigens and human peripheral nerve gangliosides. Single nucleotide polymorphisms in exon 2 of CD1A (*01/*02) and CD1E (*01/*02) were assessed using PCR-RFLP; no significant differences in genotype or allele frequency were observed between 200 patients with GBS and 200 healthy controls. CD1 gene polymorphisms cannot be recognized as a susceptibility or disease-causative factor for GBS in the Bangladeshi population. However, further studies are necessary to investigate the CD1A*01/CD1E*01 haplotype distribution and its potential causative role in the axonal form of GBS.

Characterization of Guillain Barre syndrome in a city on the Colombian Caribbean coast: 24 cases

Cartagena de Indias, Colombia. is located on the shores of the Caribbean Sea. The city has a humid tropical climate.Has a relative humidity + 90%, with the rainy season typically between April-May and October-November. In 2015 there was an unusual increase in cases of guillain barre syndrome. Guillain-Barré syndrome (GBS) is a Acute autoimmune polyradiculoneuropathy that attacks mainly Myelin, characterized by the rapid onset Symmetrical weakness and paralysis, with a decrease in Osteotendinous reflexes.

Czech National Guillain-Barré Syndrome Registry

Introduction: The Guillain-Barre syndrome is an acute infl ammatory autoimmune polyradiculoneuropathy and belongs among rare neurologic diseases. Since poliomyelitis has been almost completely eliminated, it is currently the most frequent cause of acute fl accid paralysis worldwide. The registry was formed in the Czech Republic in 2012 and is an important source of prospectively recorded data. Aim: The goal of the registry is to follow up the clinical profi le, treatment and 6-month prognosis of Guillain-Barre syndrome patients. Methods: The data are collected prospectively by persons delegated by neuromuscular centres in the Czech Republic. The clinical course is assessed on the basis of the GBS disability scale and muscle strength according to the Medical Research Council sum score for facial muscles and extremity muscles. Detailed clinical investigation is done on admission, before the treatment, immediately after the treatment, 1 month and 6 months after the end of the treatment. Results: We enrolled a total 272 cases of Guillain-Barre syndrome patients in the period from 1st January 2012 to 17th August 2016. We have complete data for 6 months in 137 of them. 52 were treated with intravenous human immunoglobulin and 85 with plasma exchange. A good clinical outcome after 6 months (ability to walk unassisted) was achieved in 85% of patients. Severe residual neurological defi cit persisted after 6 months in 11% of patients. The remaining 6 patients died. We did not fi nd any diff erence in the effi ciency of immunoglobulin vs. plasma exchange in the whole group, in the subgroup of the severely disabled patients and according to sex. Conclusion: Medical care centralisation and adherence to the standard of care lead to a good clinical outcome in Guillain-Barre syndrome patients, which is comparable with relevant sources.

Hyperreflexia ? an Uncommon Presentation of Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is an acquired acute autoimmune polyradiculoneuropathy. Progressive motor weakness and areflexia are essential for diagnosis. But in some cases hyperreflexia can be seen. Diagnosis of GBS was made based on history and clinical findings and was supported by cerebrospinal fluid (CSF) studies and nerve conduction study (NCS). We hereby report a case of a 42-year-old male presenting with acute onset flaccid quadriparesis. There was frank hyperreflexia in all four limbs. Although reflex preservation and hyperreflexia can be noted in axonal variant of GBS in Chinese, Japanese, and European populations, it is uncommon in India.J Enam Med Col 2017; 7(2): 111-112

An Incidence of Chronic Inflammatory Demyelinating Polyneuropathy in Pregnancy [28L

INTRODUCTION: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a long-term form of Guillain-Barre syndrome (GBS), an acute inflammatory autoimmune polyradiculoneuropathy linked to various infectious agents. It is a rare disease process associated with symmetric motor paralysis with or without sensory deficits, affecting between 0.8 and 7.7 per 1,000,000. There is limited representation of antepartum management of CIDP in the literature. METHODS: We detail our experience in a primigravid patient with previously diagnosed CIDP during an acute exacerbation in pregnancy. The clinical case was analyzed followed by a literature review of CIDP antepartum management. RESULTS: A 29 year old primigravid female at 18 weeks gestation with a past medical history notable for GBS presented with reports of numbness and weakness in bilateral upper and lower extremities for 1 week. The patient was assessed, admitted and underwent evaluation with electromyogram and nerve conduction studies. Studies demonstrated progression of CIDP, with worsened demyelinating features with reduced conduction velocity and axonal involvement. Due to prior poor response to intravenous immunoglobulin, treatment with a limited course of plasmapheresis to prevent disease progression was successfully conducted. The patient subsequently experienced a successful term delivery of a female neonate. CONCLUSION: CIDP is an autoimmune neurologic disease process that has been minimally studied in pregnancy due to its low incidence. Our case details the successful evaluation and management of CIDP with plasmapheresis during pregnancy with subsequent successful term delivery. Increased awareness of CIDP and associated management is necessary due to the debilitating nature of the disease with potential fetal and maternal effects.

Guillain-Barr´E Syndrome with Bilateral Extensor Plantar Reflexes

Introduction Background: Guillain-Barr e syndrome (GBS) usually presents with lower motor neuron (LMN) symmetrical weakness, areflexia, and hypotonia. GBS has rarely been reported with up going plantars’ response. We report a case of GBS with up going plantars during the course of the disease. Citation: Ahmad SF, Al-Bader SY, Hashel JY (2014) Guillain-Barr E Syndrome with Bilateral Extensor Plantar Reflexes. J Neurol Neurol Disord 1(1): 105 . doi: 10.15744/2454-4981.1.105 Case presentation: A 52-year-old woman developed acute paralysis with areflexia following a diarrheal illness. There was no fever at the time of the onset. She developed numbness and asymmetrical weakness of both lower limbs which progressed to involve the upper limbs bilaterally. There were no cranial nerves, respiratory or sphinecteric involvements. Few days later, the plantars’ became upgoing bilaterally, with absent of other upper motor neuron signs. Cerebrospinal fluid (CSF) analysis showed albuminocytological dissociation. Early Nerve conduction study (NCS) showed absent H-reflex dispersed F-waves with prolonged maximum F-latencies and M-amplitude showed no clear block. MRI brain and spine were unremarkable. She was treated with intravenous immunoglobulin (IVIG). Repeated NCS two weeks after the onset, definitely confirmed conduction block. She was able to walk with support three weeks after the onset. Conclusion: GBS should be considered as a differential diagnosis in a patient with acute quadriparesis, even if there is asymmetrical muscle weakness and extensor plantars’’ response. Guillain-Barre syndrome (GBS) is an acute autoimmune polyradiculoneuropathy. Clinical features include progressive, symmetrical ascending muscle weakness usually of more than two limbs, and areflexia with or without sensory, autonomic or brainstem involvements. Weakness is prominent in the lower limbs muscles compared to the upper limbs; there is absence of fever at the onset of neural symptoms [1]. Although, the diagnosis of GBS is based on clinical criteria, the presence of suggestive findings in the nerve conduction studies (NCS) or albuminocytological dissociation in the cerebrospinal fluid (CSF) analysis help to confirm the diagnosis [2].The involvement of the central nervous system (CNS) in the GBS is rare [3]. We report a case of GBS with asymmetrical weakness and up going plantars’ during the course of the disease. A 52-year-old female, known case of diabetes, hypertension, and hypothyroidism, was admitted with acute weakness of both lower limbs. She had history of diarrhea 4 days prior to presentation, which lasted 2 days and improved with symptomatic treatment. Then she developed numbness and weakness of both lower limbs that involved the left side more than the right. The weakness started distally and ascended up proximally. The weakness started to involve the upper limbs 4 days later. There were no cranial nerves, respiratory muscles involvement, nor sphincteric symptoms. There was no fever at the time of onset and no history of recent vaccination. On examination, there was weakness of both lower limbs with hypotonia. The weakness of both lower limbs was asymmetrical with proximal muscle power of 2/5 (Medical Research Council grading) in the left side and 4/5 in the right side, while the power of the distal muscles was 1/5 in the left side and 3/5 in the right side. Examination of the upper limbs showed weakness of distal muscles of 4/5. Deep tendon reflexes were absent all over, and the plantars’ were flexors bilaterally. Decrease in fine touch and temperature sensations were seen in L4, L5, S1 distribution of the left side with loss of joint position and vibration sense bilaterally. There was no cranial nerves involvement. The investigations showed normal total and differential leukocyte counts, erythrocyte sedimentation rate (4 mm/h), creatine kinase level, and electrolytes (Na, Ca, K, Mg, phosphate). Vasculitis and paraneoplastic work up were all negative. CSF examination showed albuminocytologic dissociation. CSF sugar was 6.3 mmol/L (plasma glucose 11 mmol/L), protein was 1517 mg/L, and there are only 2 cells (100% lymphocytes) were detected. All causes of

Auto‐immune polyradiculoneuropathy and a novel IgG biomarker in workers exposed to aerosolized porcine brain

Twenty-four patients, all of whom were exposed to aerosolized porcine brain tissue through work-place environment (abattoir), developed a syndrome of immune-mediated polyradiculoneuropathy; three also had central nervous system manifestations (transverse myelitis, meningoencephalitis, and aseptic meningitis). Patients had characteristic electrophysiological findings of very distal and proximal conduction slowing (prolonged distal and F-wave latencies, regions where the blood-nerve barrier is the most permeable) and all patients' serum contained a novel IgG immunofluorescence pattern. Nerve pathology, when available, showed mild changes of segmental demyelination, axonal degeneration, and inflammatory changes. Patients had meaningful improvement of symptoms and electrophysiologic findings with immune therapy and with removal of exposure to aerosolized brain tissue. We postulate that this outbreak is an auto-immune polyradiculoneuropathy triggered by occupational exposure to multiple aerosolized porcine neural tissue antigens that result in neural damage where the blood-nerve barrier is the least robust.

Guillain-Barre syndrome and mood disorders

Background Guillain-Barre syndrome (GBS) is an acute, autoimmune polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, acute inflammatory demyelinating polyneuropathy (AIDP). Clinical hallmarks of this syndrome include symmetric progressive flaccid muscle paresis, areflexia, ataxia, dysautonomia, and respiratory insufficiency in the presence of an increased cerebrospinal fluid protein content, as well as electromyography studies demonstrating evolving demyelination.

Hyperreflexic Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is an acquired acute autoimmune polyradiculoneuropathy. The 2 features considered essential for the diagnosis of GBS are progressive motor weakness and areflexia. There have been several descriptions of reflex preservation and hyperreflexia in axonal variant of GBS in Chinese, Japanese, and European populations but it is not common in the Indian subcontinent. We report 2 such cases discussing the pathophysiology and management aspects. This case report is to impress upon treating physicians and neurologists in training that a hyperreflexic variant of GBS albeit rare, should not be missed in a given clinical setting.

Guillain-Barré syndrome: An update in rehabilitation

Background Guillain-Barré syndrome (GBS) is an autoimmune mediated polyradiculoneuropathy which causes acute neuromuscular paralysis and ventilatory failure. It mainly affects young adults, and most patients (80%) make a good recovery; being ambulant again at 6 months post-onset. However, residual deficits can cause significant long-term disability, which impacts on activities of daily living, work, social activities, and health-related quality of life. Content This article provides an overview of the aetiology, pathogenesis and clinical presentation of GBS. Current evidence regarding use of outcome measures and the symptomatic and rehabilitation treatment for adult GBS survivors is outlined. Issues impacting on rehabilitation are also discussed, including complications as a result of immobility, autonomic dysfunction and deafferent pain syndromes. Conclusions GBS is a complex and challenging condition that requires integrated and coordinated health-care delivery and services. Rehabilitation is aimed at improving a person's functional ability in daily living tasks and social reintegration. However, evidence for many rehabilitation treatments is currently lacking. Outcome measurement for disability in this group is also limited and not well quantified. The longer term impact of disability in GBS survivors and ‘aging’ effects are not well understood. Further research in these areas is therefore required.

Improved Serological Diagnosis Stresses the Major Role of Campylobacter jejuni in Triggering Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a postinfectious autoimmune polyradiculoneuropathy. The most frequent antecedent pathogen is Campylobacter jejuni, followed by cytomegalovirus. However, more than 40% of GBS cases currently cannot be attributed to triggering events. This might be due to the shortcomings of the serological assays used for diagnosing infections, in particular for C. jejuni. In our study investigating 36 patients with acute GBS, standard serological methods identified the triggering viral or bacterial etiology in only 25% of cases. However, using a highly specific enzyme-linked immunosorbent assay based on two recombinant outer antigens encoded by C. jejuni genes Cj0017 (P39) and Cj0113 (P18), we found serological evidence of a preceding C. jejuni infection in 80.6% of the patients but in only 3.5% of the controls. We conclude that the role of C. jejuni in triggering GBS has been greatly underestimated.

Pseudomeningoencephalitic presentation of pediatric Guillain-Barré syndrome.

Guillain-Barré syndrome is an acute, autoimmune polyradiculoneuropathy that improves with immune-modulating treatment if instituted early in the illness. Preliminary diagnosis relies on the clinician's recognition of the typical symptoms and signs as supporting evidence of the illness, such as nerve conduction studies, which may not be available emergently. We report eight children with Guillain-Barré syndrome in whom the initial presentation was atypical and suggested a primary central nervous system illness. In these patients, the predominant clinical symptoms included drowsiness, headache, irritability, and meningismus, although the classic features of Guillain-Barré syndrome (weakness, hyporeflexia) were also present. The atypical presentation caused delay in diagnosis in some cases. It is important to recognize this variant of pediatric Guillain-Barré syndrome to ensure expeditious diagnosis and treatment.