Acute Polyradiculoneuropathy Research Articles

Recurrent GBS in Children: A Case Report

Background: Guillain-Barré Syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy characterized by rapidly progressing muscle weakness. GBS usually follows a monophasic course, but rarely it can recur after an asymptomatic period of several months to years. Limited cases of recurrent GBS have been reported in the literature. We present two cases of recurrent GBS encountered in a tertiary care hospital. This case report describes the clinical presentation, diagnostic workup, management, and outcomes of two children of GBS who experienced recurrence. By presenting the cases, we aim to increase awareness of this rare presentation of GBS among healthcare professionals and enabletimely recognition and better outcomes for children with recurrent GBS. J Com Med Col Teachers Asso Jan 2024; 28(1): 37-40

Early Neurophysiological Abnormalities in Suspected Acute Canine Polyradiculoneuropathy.

Acute canine polyradiculoneuritis (ACP) is a common peripheral neuropathy in dogs, and is generally self-limiting and benign. Electrodiagnostic (EDX) tests are typically performed after 7-10 days. Delaying the definitive diagnosis may hamper the treatment of other causes of acute weakness, which may require specific treatments and may carry different prognoses. This retrospective multicenter study aims to assess whether EDX performed within the first 6 days of clinical signs onset can detect alterations indicative of ACP, and aims to characterize the most prevalent alterations. A total of 71 dogs with suspected ACP were retrospectively analyzed and classified into two groups based on EDX timing: early group (EG, 1-6 days after symptom onset) and late group (LG, 7-15 days after symptom onset). In our study, no significant differences were found between the two groups in motor nerve conduction studies (MNCSs) and F-wave analysis, indicating that EDX is able to demonstrate abnormalities even in the first 6 days from onset. Although the LG showed significantly greater degrees of electromyographic (EMG) alterations compared to the EG, frequent muscle alterations were still observed in the EG group. These findings support the use of EDX in patients with suspected ACP within the first 6 days from the clinical onset. Prompt neurophysiological examinations for suspected ACP patients can be performed effectively and can help allow for early diagnosis and facilitate appropriate treatment.

Acute motor axonal polyradiculoneuropathy; could it occur due to wasp stings–A grey case

Acute motor axonal polyradiculoneuropathy; could it occur due to wasp stings–A grey case

Adverse Events and Infectious Complications in the Critically Ill Treated by Plasma Exchange: A Five-Year Multicenter Cohort Study.

The aim of this study was to determine, in critically ill patients treated with therapeutic plasma exchange (TPE), the incidence of adverse events as well as the incidence of secondary infections and its predictive factors. A multicenter retrospective cohort study of an intensive care population treated with TPE to collect adverse events and infectious complications. The characteristics of patients who developed an infection after plasma exchange were compared with those of patients who did not. Four ICUs of French university hospitals. All adults admitted between January 1, 2015, and December 31, 2019, who received at least one plasma exchange session were included. None. A total of 711 TPE sessions were performed on 124 patients. The most frequent TPE indications were thrombotic microangiopathies (n = 32, 26%), myasthenia gravis (n = 25, 20%), and acute polyradiculoneuropathy (n = 12, 10%). Among the 124 patients, 22 (21%) developed arterial hypotension, 12 (12%) fever, and 9 (9%) electrolyte disturbance during TPE. Moreover, 60 (48%) presented at least one infectious complication: ventilator-associated pneumonia 42, pneumonia 13, bacteremia 18 (of which 6 catheter-related infections) viral reactivation 14. Independent risk factors for ICU-acquired infection were the ICU length of stay (24 vs. 7 d; hazard ratio [HR]: 1.09 [1.04-1.15], p < 0.001) and invasive mechanical ventilation (92% vs. 35%; HR: 16.2 [5.0-53.0], p < 0.001). In critically ill patients treated with TPE, adverse events occurring during the procedure remain moderately frequent and are mostly not life-threatening. Infectious complications, mainly ventilation-associated pneumonia, are frequent in this population. The need of mechanical ventilation and longer ICU stay is associated with an increased risk of infection.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

Systemic Lupus Erythematosus Initially Presenting as Acute Motor andSensory Axonal Neuropathy Variant ofGuillain-Barre Syndrome ina Healthy Active Duty Female.

Guillain-Barre syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy characterized by rapidly evolving ascending weakness, mild sensory loss, and hypo- or areflexia, typically progressing to peak symptoms over the course of 4 weeks. The precise mechanism is unclear but is proposed to be an immune-mediated reaction with the generation of antibodies against peripheral nerves triggered by a preceding viral infection. Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe variant of Guillain-Barre syndrome with limited published literature. Discussion of risk factors for this subtype has not been done in a systematic way. This case report involves a 34-year-old, active duty, West African female, who immigrated to the United States in 2019. She presented with worsening diplopia, bilateral distal upper and lower extremity paresthesias as well as progressively worsening bilateral upper extremity weakness. Her clinical picture was complicated by constitutional symptoms, diffuse lymphadenopathy, no preceding viral illness, and marked clinical deterioration. Ultimately, she was diagnosed with acute motor and sensory axonal neuropathy in the setting of a new diagnosis of systemic lupus erythematosus, a rarely described association emphasizing the importance of a thorough evaluation for underlying causes of acute neurologic pathologies.

Guillain-Barré Syndrome with multiple cranial neuropathies

Background. Guillain-Barré Syndrome (GBS) is an autoimmune reaction against peripheral nerves that manifests clinically as acute polyradiculoneuropathy. Classic sensory-motor, pure motor, paraparesis, pharyngeal-cervical-brachial, Bickerstaff brainstem encephalitis, pure sensory, bilateral facial palsy with paresthesia, and Miller-Fisher syndrome are all known variants of GBS. This case report seeks to describe a case of GBS with involvement of the cranial nerves. Case report. A 39-year-old right-handed Balinese woman presented with LMN (Lower Motor Neuron) type paraparesis with ascending paralysis and involvement of cranial nerves III through XII. The results of the LCS (Liquor Cerebro-Spinal) demonstrate albuminocytological dissociation. According to the patient’s EMNG (electromyoneurography) findings, Guillain-Barré Syndrome was of the AMAN (acute motor axonal neuropathy) variety. The patient was diagnosed with multiple cranial neuropathies and GBS of the AMAN type. EGRIS (Erasmus GBS Respiratory Insufficiency Score) was 3, and EGOS (Erasmus GBS Obstructive Sleep Apnea Score) was also 3. Following IVIG (Intravenous Immunoglobulin) treatment, the patient exhibited clinical improvement (Hughes score decreased from 4 to 2). Discussion. The patient exhibits clinical manifestations of flaccid tetraparesis and multiple cranial nerve paresis. The clinical condition of the patient did not meet the criteria for GBS clinical variation. Symptoms of flaccid tetraparesis that do not involve the senses are classified as the motor-only variant. This patient’s differential diagnosis includes the possibility of MFS and ophthalmoplegia. This patient, however, had flaccid tetraparesis and no ataxia. Electromyoneurography (EMNG) examination produces AMAN-type GBS. The patient was diagnosed with one of the clinical variants of GBS, AMAN-type GBS with multiple cranial neuropathies. Conclusion. A case of GBS of the AMAN type accompanied by multiple cranial neuropathies has been reported.

Guillain-Barre Syndrome, Clinical Spectrum and Electrophysiological Sub Types: A Local Experience

Background: Guillain-Barre syndrome is uncommon neurological disease that occurs in acute phase as generalized polyradiculo-neuropathy. It has multiple variants some of them being uncommon and rarely encountered. Objective: The objective of the study was to determine the frequency and clinical characteristics of Guillain-Barre syndrome variants. Methods: A cross-sectional study was carried out in neurology department, Mayo hospital from July to December 2022. Patients clinically diagnosed as Guillain-Barre syndrome by using Brighton criteria were included. All the patients underwent electro diagnostic procedures and they were categorized into five basic variants i.e., Acute motor axonal neuropathy, Acute motor sensory axonal neuropathy, acute inflammatory demyelinating polyradiculo-neuropathy, acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy. Results: Out of total 85 patients, males were 56 (65.9%) and 29 (34.1%) were females. Adults were more affected with a mean age of 36±15.3. Frequency of acute motor axonal neuropathy (35.3%) was high followed by acute motor sensory axonal neuropathy (31.8%), mixed variety acute polyradiculo-neuropathy (16.5%), acute inflammatory demyelinating polyradiculo-neuropathy (12.9%) and acute sensory axonal neuropathy (3.5%) respectively. Conclusion: Acute motor axonal neuropathy is frequently encountered Guillain-Barre syndrome variant in our setup. Also, the frequency of acute sensory axonal neuropathy and mixed variety acute polyradiculo-neuropathy has increased with a declining trend of acute inflammatory demyelinating polyradiculo-neuropathy.

Clinical features and maternal and fetal outcomes in women with Guillain-Barré syndrome in pregnancy.

Guillain-Barre syndrome (GBS) is an acute inflammatory polyradiculoneuropathy rarely observed during pregnancy. In this retrospective study, we analyzed the characteristics of pregnant women with GBS (pGBS) diagnosed in French University Hospitals in the 2002-2022 period and compared them with a reference group of same-age non-pregnant women with GBS (npGBS) identified in the same institutions & timeframe. We identified 16 pGBS cases. Median age was 31years (28-36), and GBS developed in the 1st, 2nd, and 3rd trimester in 31%, 31% and 38% of cases respectively. A previous infection was identified in six cases (37%), GBS was demyelinating in nine cases (56%), and four patients (25%) needed respiratory assistance. Fifteen patients (94%) were treated with intravenous immunoglobulins, and neurological recovery was complete in all cases (100%). Unscheduled caesarean section was needed in five cases (31%), and two fetuses (12.5%) died because of cytomegalovirus (CMV) infection (1 case) and HELLP (Hemolysis, Elevated Liver enzymes and Low Platelets) syndrome (1 case). In comparison with a reference group of 18 npGBS women with a median age of 30years (27-33), pGBS patients more frequently had CMV infection (31% vs 11%), had a prolonged delay between GBS onset and hospital admission (delay > 7days: 57% vs 12%), more often needed ICU admission (56% vs 33%) and respiratory assistance (25% vs 11%), and more often presented with treatment-related fluctuations (37% vs 0%). This study shows GBS during pregnancy is a severe maternal condition with significant fetal mortality.

Current status of Guillain-Barré syndrome (GBS) in China: a 10-year comprehensive overview.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy; a disease involving the peripheral nervous system which is the most common cause of acute flaccid paralysis worldwide. So far, it is still lack of a comprehensive overview and understanding of the national epidemiological, clinical characteristics, and the risk factors of GBS in China, as well as differences between China and other countries and regions in these respects. With the global outbreak of the coronavirus disease 2019 (COVID-19), an epidemiological or phenotypic association between severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and GBS has attracted great attention. In this review, we outlined the current clinical data of GBS in China by retrieving literature, extracting and synthesizing the data of GBS in China from 2010 to 2021. Besides, we compared the characteristics of epidemiology, preceding events and clinical profiles of GBS between China and other countries and regions. Furthermore, in addition to conventional intravenous immunoglobulin (IVIG) and plasma exchange (PE) therapy, the potential therapeutic effects with novel medications in GBS, such as complement inhibitors, etc., have become the research focus in treatments. We found that epidemiological and clinical findings of GBS inChina are approximately consistent with those in the International GBS Outcome Study (IGOS) cohort. We provided an overall picture of the present clinical status of GBS in China and summarized the global research progress of GBS, aiming to further understand the characteristics of GBS and improve the future work of GBS worldwide, especially in countries with the middle and low incomes.

Evidence of Lumbosacral Plexus Involvement on MR Neurography in Early Guillain-Barré Syndrome (P14-8.015)

<h3>Objective:</h3> To describe a case of early Guillain-Barré Syndrome (GBS) with novel findings of lumbosacral plexus involvement on MR neurography. <h3>Background:</h3> GBS is an acute inflammatory polyradiculoneuropathy characterized by progressive sensory loss, weakness, areflexia, CSF albumino-cytologic dissociation and cauda equina enhancement on MRI. Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic studies can be normal/non-diagnostic in the first 1–2 weeks. We describe a case of early GBS with cauda equina enhancement and lumbosacral plexus abnormalities on MR neurography, seen only three days after symptoms onset and before any diagnostic abnormalities in CSF or electrodiagnostic testing were detected. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 67-year-old man presented with a 3-day history of rapidly progressive gait instability and leg weakness, 14 days after an uncomplicated L5-S1 fusion. Diffuse arm and leg weakness, distal leg sensory loss, hypoactive arm reflexes and absent leg reflexes were detected on exam. CSF analysis showed normal cells and protein (55mg/dL, reference&lt;60mg/dl). Electrodiagnostic testing showed mildly prolonged F-response latencies of the median and ulnar nerves, absent peroneal F-response, normal tibial F-response latency and absent tibial H-responses, without other findings suggestive of demyelination, and normal needle EMG. Lumbar spine MRI showed enhancement of ventrally positioned nerve roots of the cauda equina and no central or foraminal stenosis. Lumbosacral plexus MR neurography showed prominent enlargement, hyperintensity and enhancement of bilateral lower lumbosacral nerve roots and the proximal sciatic nerves. Plasma exchange was initiated with subsequent improvement in his symptoms and exam findings. <h3>Conclusions:</h3> Diagnosis of early GBS can be challenging, as CSF and electrodiagnostic testing can be normal/non-diagnostic. MRI and MR neurography are useful surrogate tests which may have greater sensitivity in detecting early GBS compared to CSF and electrodiagnostic testing. Involvement of the lumbosacral plexus by imaging is a novel finding, which can further assist in diagnosing early GBS cases. <b>Disclosure:</b> Dr. Weng has nothing to disclose. The institution of Darryl Sneag, 12603 has received research support from GE Healthcare. Dr. Pavlakis has nothing to disclose.

Immune-Mediated Neuropathies: Pathophysiology and Management.

Dysfunction of the immune system can result in damage of the peripheral nervous system. The immunological mechanisms, which include macrophage infiltration, inflammation and proliferation of Schwann cells, result in variable degrees of demyelination and axonal degeneration. Aetiology is diverse and, in some cases, may be precipitated by infection. Various animal models have contributed and helped to elucidate the pathophysiological mechanisms in acute and chronic inflammatory polyradiculoneuropathies (Guillain-Barre Syndrome and chronic inflammatory demyelinating polyradiculoneuropathy, respectively). The presence of specific anti-glycoconjugate antibodies indicates an underlying process of molecular mimicry and sometimes assists in the classification of these disorders, which often merely supports the clinical diagnosis. Now, the electrophysiological presence of conduction blocks is another important factor in characterizing another subgroup of treatable motor neuropathies (multifocal motor neuropathy with conduction block), which is distinct from Lewis-Sumner syndrome (multifocal acquired demyelinating sensory and motor neuropathy) in its response to treatment modalities as well as electrophysiological features. Furthermore, paraneoplastic neuropathies are also immune-mediated and are the result of an immune reaction to tumour cells that express onconeural antigens and mimic molecules expressed on the surface of neurons. The detection of specific paraneoplastic antibodies often assists the clinician in the investigation of an underlying, sometimes specific, malignancy. This review aims to discuss the immunological and pathophysiological mechanisms that are thought to be crucial in the aetiology of dysimmune neuropathies as well as their individual electrophysiological characteristics, their laboratory features and existing treatment options. Here, we aim to present a balance of discussion from these diverse angles that may be helpful in categorizing disease and establishing prognosis.

High and Persistent Anti-GM1 Antibody Titers Are Associated With Poor Clinical Recovery in Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy that may follow a preceding infection inducing a cross-reactive antibody response to glycosphingolipids in peripheral nerves. The immune response in GBS is considered to be short lasting, explaining its monophasic clinical course. However, the disease course varies between patients, and residual deficits frequently occur. The duration of the antibody response has not been defined extensively in GBS, and the persistence of these antibodies may impair clinical recovery. The aim of this study was to determine the titer course of serum antibody titers to the ganglioside GM1 in relation to clinical course and outcome in patients with GBS. Acute-phase sera from patients with GBS included in previous therapeutic trials were screened for anti-GM1 IgG and IgM antibodies in ELISA. Anti-GM1 antibody titers were determined in sera collected at entry and during a 6-month follow-up. Clinical course and outcomes were compared between groups based on the titer course. Anti-GM1 antibodies were detected in 78 (20.7%) of 377 included patients. The anti-GM1 IgG and IgM antibody titer course was highly variable between patients. A subset of anti-GM1-positive patients had persistent anti-GM1 antibodies at 3 months (n = 27/43 [62.8%]) and 6 months (n = 19/41 [46.3%]). Patients with a high anti-GM1 IgG and IgM titer at entry recovered more slowly and less complete than anti-GM1-negative patients (IgG: p = 0.015, IgM: p = 0.03). High vs low IgG titers were independently associated with poor outcome after correcting for known prognostic factors (p = 0.046). Among patients with a high anti-GM1 IgG titer at entry, a slow titer decline was associated with poor outcome at 4 weeks (p = 0.003) and 6 months (p = 0.032). Persistent high IgG titers at 3 and 6 months were associated with poor outcome at 6 months (3 months: p = 0.022, 6 months: p = 0.004). High anti-GM1 IgG and IgM antibody titers at entry and persistent high anti-GM1 IgG antibody titers are associated with poor outcome in patients with GBS. Antibody persistency indicates ongoing antibody production long after the acute disease state in GBS. Further research is required to determine whether antibody persistency interferes with nerve recovery and is a target for treatments.

Is Guillain–Barre syndrome following chickenpox a parainfectious disease? A case report and literature review

BackgroundPolyradiculoneuropathy following infection with varicella zoster virus (VZV) is rare and most of the time, happens in the context of reactivation of latent VZV. We report a case of acute polyradiculoneuropathy following primary infection with VZV marked by atypical clinical features raising the hypothesis of a para-infectious disease.Case presentationWe describe a 43-years-old male who developed ataxia, dysphagia, dysphonia, and oculomotor disorders (vertical binocular diplopia and bilateral ptosis) followed by quadriplegia with areflexia which occurred 4 days later. The patient had a history of varicella that occurred 10 days before the onset of these symptoms. Nerve conduction study revealed features consistent with an acute motor-sensory axonal neuropathy (AMSAN). Anti-ganglioside antibodies were negative.Based on clinical presentation and ancillary examination, we retain the Miller Fisher/Guillain-Barré overlap syndrome diagnosis.The patient was treated with high doses of methylprednisolone but the evolution of the disease was nevertheless marked by a complete recovery six weeks after onset of symptoms.ConclusionGBS following varicella is a rare but severe disease occurring most often in adults and marked by greater involvement of the cranial nerves. Its clinical features suggest that it is a para-infectious disease. Antiviral therapy has no effect on the course of the disease but its administration within the first 24 h after the onset of chickenpox in adults can prevent its occurrence.

Spectrum of Neurological Disorders Related to Autoimmune Diseases in Brazzaville, Congo

Background: Autoimmune diseases, which are among the leading causes of morbidity and mortality in the world, are pathologies caused by a dysfunction of the immune system. They can affect the central nervous system, the peripheral nervous system or both nervous systems. Objectives: To describe the epidemiological, clinical, paraclinical, therapeutic and evolutive aspects of neurological disorders related to autoimmune diseases. Methods: This was a prospective cohort study. It was carried out from 1 January 2015 to 31 December 2019 (5 years). It focused on patients aged 15 years and above, who were hospitalized or followed as ambulatory patients for neurological disorders related to autoimmune diseases in the neurology department of the university teaching hospital in Brazzaville. Results: Among the 41 patients who fulfilled inclusion criteria, there were 29 (70.73%) women and 12 (29.27%) men. The average age of patients was 38.3 ± 13.8 years. An increase in the frequency of neurological disorders related to autoimmune diseases was observed every year. The main neurological disorders were neuromyelitis optica spectrum disorders (n = 14; 34.15%), acute polyradiculoneuropathies (n = 13; 31.71%), chronic polyradiculoneuropathies (n = 4; 9.75%) and acute disseminated encephalomyelitis (n = 3; 7.31%). The treatments administered, which consisted of corticosteroids and immunosuppressive drugs, had significantly improved the vital prognosis and functional status of patients (p = 0.025). Conclusion: In our study population, neurological disorders related to autoimmune diseases are rare. The neurological clinico-pathological entities diagnosed are similar to those reported in the literature. The therapeutic approaches used improve the quality of life of patients.

Diagnostic Pitfalls in Guillain-Barré Syndrome: Case Report and Literature Review.

Guillain-Barré syndrome (GBS) represents a group of acute immune-mediated polyradiculoneuropathies that is usually characterized by symmetrical limb weakness and areflexia. GBS can also lead to atypical clinical findings, which may lead to confusion and errors in the diagnosis. In this report, we describe a case of Guillain-Barré syndrome in a 7-year-old child who presented with neck stiffness, headache and vomiting mimicking acute meningoencephalitis, arthritis and myositis. Symptoms of ascending paralysis developed subsequently. Clearly, the atypical presentation of GBS is a significant dilemma for pediatricians and may lead to delays in diagnosis and treatment.

Triggers of Guillain-Barré Syndrome: Campylobacter jejuni Predominates.

Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, and cytomegalovirus. C. jejuni is responsible for about a third of GBS cases. GBS due to C. jejuni is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.

Guillain Barre Syndrome - Acute Inflammatory Demyelinating Polyneuropathy with Unusual Descending Paralysis

Guillain-Barré syndrome (GBS) is a rare autoimmune, acute fulminant polyradiculoneuropathy. Incidence is between 1- 4 cases per 100,000 per year. It can affect age groups such as both young and old. The distribution depending on age-specificity appears to be bimodal, and has high rates in young adults as well as the elderly. Mostly manifests as rapidly evolving areflexic motor paralysis with a usual ascending type of paralysis. Autonomic involvement is common with usual manifestations of loss of vasomotor control such as BP fluctuations, postural hypotension, and arrhythmias. Deep tendon reflexes are diminished or disappear in the first few days of disease onset. Several cases have been reported with descending type of paralysis, but they were patients of Miller Fischer syndrome variant of GBS. Only 15% of the cases were reported regarding descending type of paralysis in AIDP variant of GBS. Our patient presented with unusual descending paralysis, starting with the upper limbs and progressively involving weakness of the lower limbs. The patient did not have any comorbid illnesses rather than recent gastrointestinal infections. In the progress of the disease, he developed respiratory complications for which he needed ventilator support and with all supportive care and keen monitoring, the patient was weaned off from the ventilator, a tracheostomy done, and improved. Thus presenting a case of Guillain Barre syndrome with an unusual descending type of paralysis and with all other typical symptoms of this condition, who have been treated with high dose IV immunoglobulin, creates awareness in view of diagnosing the disease even with unusual presentation would save the life of the patient, instead presenting the usual case scenario or rare disease case.

Clinical genetic counselling and rehabilitation treatment of a patient with Guillain–Barré syndrome after COVID-19

The authors present a clinical case of rehabilitation after the development of GuillainBarr syndrome, an acute autoimmune inflammatory polyradiculoneuropathy, in a patient who underwent a SARS CoV-2 infection. The patient previously manifested with severe comorbidities (arterial hypertension, hypercholesterolemia, type 2 diabetes mellitus, stenosing atherosclerosis of brachiocephalic arteries). A diagnostic panel of single nucleotide gene polymorphisms associated with risk factors of cardiovascular diseases, metabolic disorders, immunopathology, pharmacogenetics was applied using PCR-RT Genetic Passport test system, and the results were interpreted in order to predict potential complications, adverse drug reactions and the choice of biomarkers for preventive measures. We have compared clinical manifestations, comorbid background and the identified genotype features, as follows: minor homo- and heterozygous variants of ACE, AGT, CYP1A2, NOS3, PPARD, EDN, PALLD, SNX19 genes associated with predisposition to cardiovascular diseases, increasing the risk of dysregulation of blood pressure, development of endothelial dysfunction. The following gene variants were revealed: FXII, ITGA2, ITGB3, MTHFR, MTRR, MTR, PAI-1 that increase the risk of venous and arterial thrombosis, along with gene variants of ADRB3, FTO, INSIG2, KCNG11, LEP, PPARD, TCF7L2, ApoC3, PON1 associated with carbohydrate and lipid metabolism disorders; polymorphisms in the genes determining the immune response, i.e., IL4, IL6, IL8, IL10, CDH1, BDNF1, CRP, CCR5 (with del32 allele considered a risk factor of severe SARS-CoV-2), homozygous polymorphism of a gene of FCGR2 associated with risk of antigen-antibody-complement-mediated cytotoxicity, circulation arrest, deposition of immune complexes in endothelium of microvessels, decreased antithrombotic effects and increased procoagulant activity. Pharmacogenetic study revealed a variant of the CYP4F2 gene, a CYP2C19 gene polymorphism associated with delayed metabolism of a number of pharmaceuticals which requires higher drug dosage, or choosing a drug with a different mechanism of action; gene variants of CYP1A2, GSTP1, GSTT1 reducing efficiency of the cellular detoxification system; NAT2*5 and NAT2*6 variants determining a decrease in appropriate enzyme activities when administering a standard dose of drugs with slowdown of their detoxification, accumulation of toxic metabolites causing clinical adverse effects (hepatotoxicity, dyspepsia, lupus-like syndrome, polyneuritis). Based on the genotype that determines pathogenesis of the multifactorial pathology (including immune-mediated complications of COVID-19), a personalized approach is recommended to the patient, in terms of treatment and prevention of complications. On the basis of testing the biochemical, immunological and blood coagulation biomarkers, an adequate choice of pharmaceuticals is recommended for the patient.