Guillain-Barré Syndrome After a SARS-CoV-2 Vaccine.

The worldwide mass vaccination campaign against COVID-19 has been the largest one ever undertaken. Although the short-term safety profile of the different vaccines has been well established, neuroinflammatory complications have been described, including rare cases of acute demyelinating inflammatory polyneuropathy. We report a 63-year-old man who presented to the emergency department with proximal muscle weakness and paresthesia. He had received the first dose of the AZD1222 SARS-CoV-2 vaccine (Oxford, AstraZeneca) two weeks before presentation. The diagnosis of Guillain-Barré syndrome (GBS) was confirmed by clinical features, cerebrospinal fluid analysis, and electromyography. On the second hospital day, progression to flaccid tetraplegia, cranial nerve involvement, and respiratory failure, requiring invasive mechanical ventilation, were noted, and he was admitted to the intensive care unit. Despite the prompt diagnosis and immunosuppressive treatment initiation, the patient was left with severe disability. Although the COVID-19 vaccination was generally safe and socially beneficial, individual adverse reactions, including neuroinflammatory severe complications, may occur.

Utility of Brainstem Auditory Evoked Response as a Diagnostic Tool and Rituximab as a Treatment for Severe Bickerstaff Brainstem Encephalitis: A Case Report.

Bickerstaff brainstem encephalitis (BBE) is a rare disorder that is characterized by ophthalmoplegia, ataxia, and disturbance in consciousness. Definite diagnosis is made primarily through clinical presentation and serology testing with anti-GQ1b antibody. However, in a country where access to serologic testing is scarce, electrophysiologic tests such as brainstem auditory evoked response (BAER) may contribute to the diagnosis. Due to its rarity and generally good prognosis, there is no established consensus for the treatment of BBE. Immunomodulatory treatments such as intravenous immunoglobulin (IVIG), plasma exchange, steroids, or a combination of these therapies are often used with good response. However, there are severe cases that respond poorly to these conventional treatments. We report the case of a 26-year-old Filipino man who came in for sudden onset of diplopia, with a one-week history of upper respiratory tract infection. Subsequently, he developed paresthesias, quadriparesis, and an altered level of consciousness. On initial examination, he only had partial third nerve palsy, but eventually became quadriparetic and obtunded during admission. Initial electromyography and nerve conduction velocity (EMG-NCV) study showed a reduced recruitment pattern of the right rectus femoris, absent H reflexes of bilateral posterior tibial nerves, and no abnormal increase in temporal dispersion. Cranial MRI with contrast was unremarkable. Video electroencephalogram (video-EEG) showed intermittent generalized 5-6 Hz and 6-7 Hz theta slowing of the background activity in the stimulated state. BAER was done revealing bilateral partial dysfunction of the auditory pathways to support brainstem involvement of the disease. He received IVIG and methylprednisolone pulse therapy with no significant clinical improvement. Hence, he was given a rituximab infusion. One week post-rituximab, he had sustained wakefulness and was able to move his extremities.

Treatment Efficacy of Plasmapheresis Versus Intravenous Immunoglobulin in Guillain-Barré Syndrome Management: A Systematic Review.

Guillain-Barré syndrome (GBS) is a rare and debilitating autoimmune disorder that affects the peripheral nervous system. Although the exact etiology of GBS is still unknown, it is thought to be triggered by a preceding gastrointestinal infection in most of the cases. Clinical manifestations include limb weakness, areflexia, and sensory loss that can further progress to neuromuscular paralysis affecting the respiratory, facial, and bulbar functions. Both plasmapheresis (PE) and intravenous immunoglobulin (IVIG) have shown effectiveness in the treatment of GBS, but it is still unclear which treatment approach is superior in terms of therapeutic efficacy. This systematic review acts per Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) 2020 guidelines. For appropriate studies and research, we searched PubMed, PubMed Central (PMC), Medical Literature Analysis and Retrieval System Online (MEDLINE), Science Direct, and Google Scholar. Screening of articles was performed based on relevance and inclusion and exclusion criteria. To check for bias, we used relevant quality appraisal tools. Initially, we found 2454 articles. After removing duplicates and irrelevant papers, we finalized 31 studies based on titles, abstracts, and reading entire articles. We excluded 14 studies because of poor quality; the remaining 17 papers were included in this review. IVIG is equally efficacious as PE in improving primary outcomes and secondary outcomes. IVIG showed a slight advantage over PE in reducing the need for mechanical ventilation (MV) and hospital stay duration. However, in children, PE demonstrated a slight edge in improving secondary outcomes. PE was associated with a slightly higher risk of adverse events and post-treatment worsening symptoms compared to IVIG. IVIG is considered more user-friendly with a significantly lower patient discontinuation rate than PE. IVIG treatment was found to be significantly more expensive than PE.

Reusing Plasma Filters in Resource-Poor Settings: Experience From a Tertiary Care Hospital.

INTRODUCTION: Therapeutic plasma exchange (TPE) is used to manage various life-threatening illnesses. It is widely performed by nephrologists, intensivists, pathologists, or experts in transfusion medicine worldwide. However, the costs of TPE sessions are exceedingly high, and they have a huge impact on patients' financial burden. Herein, we investigated the outcomes of the reuse of plasma filters in TPE on several occasions. METHODS: This is a retrospective analysis of patients receiving TPE from January 1, 2020, to April 30, 2023, in the Department of Nephrology. A formulation of 4.5% peracetic acid and 24% hydrogen peroxide acid with RO water dilution was used for reprocessing. Clinical outcomes, risks, and cost-benefit were evaluated and compared between the plasma filter reuse group (GP-1) and the no-reuse group (GP-2). RESULTS: A total of 70 patients were included in this study. 200 and 112 TPE sessions were performed in GP-1 and GP-2, respectively. The most common indication for TPE in both groups was neurological. The clinical efficacy of TPE was similar in both groups. There was no difference in the clotting of the plasma filter, any allergic reaction, infection, or bleeding in the group. However, there was a significant difference in levels of fibrinogen (p=0.03) pre and post-procedure in both groups. The incidence of hypotension was found to be higher in GP-1 (26%) compared to GP-2 (15.6%), p = 0.05. The cost of overall treatment was 38% less in GP-1. CONCLUSION: The reuse of plasma filters is a safe and effective method for cost minimization in patients requiring TPE. This method can be effectively utilized in resource-poor settings without any increased risk of adverse effects.

Miller-Fisher Syndrome Following Influenza A Infection.

Miller-Fisher syndrome (MFS), characterized by ophthalmoplegia, ataxia, and areflexia, is a Guillain-Barré syndrome (GBS) variant. It is well-known that the causative antibody for MFS is anti-GQ1b antibody. This report describes a rare case of MFS with not only anti-GQ1b antibodies but also anti-GT1a antibodies following Influenza A infection. The patient, a 47-year-old woman, contracted Influenza A three weeks before admission. She complained of double vision followed by areflexia, ataxia in the four extremities, and complete gaze palsy. She was treated with intravenous methylprednisolone pulse and intravenous immunoglobulin therapies. Her neurological symptoms were recovered after these immunotherapies.

A Rare Presentation of Guillain-Barré Syndrome With Associated Horner Syndrome: A Case Report.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy involving the peripheral nervous system. Autonomic dysfunctions are well-known complications of GBS and are major contributors to mortality. Autonomic dysfunctions are classically described during the acute phase of illness. In the literature, Horner syndrome as a manifestation of GBS has been reported in very few cases. Here, we describe a case of GBS with an acute presentation of flaccid paraparesis associated with unilateral Horner syndrome. Detecting the cause of acute flaccid paraparesis with unilateral Horner syndrome poses a diagnostic challenge, making it crucial for clinicians to maintain a heightened awareness for distinguishing between GBS and its variants, as well as other potential mimics.

Intracranial Hemorrhage in a Patient With Guillain-Barré Syndrome: A Potential Complication, a Coincidental Finding or a Treatment-Related Complication.

This case report demonstrates the difficulty of diagnosing and managing the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome (GBS), as well as the rare complication of intracerebral hemorrhage (ICH). A male patient in his mid-60s, presented with bilateral upper limb weakness, bilateral ptosis, and bulbar symptoms. The clinical presentation combined with paraclinical findings supported the diagnosis of PCB. During the course of PCB, the patient required tracheostomy and gastrostomy due to the worsening of his symptoms. Eleven days after hospitalization, and six days after the course of intravenous immunoglobulin (IVIG), the patient developed intracranial bleeding. All clinicians should consider the PCB syndrome in patients with bilateral upper extremity weakness and oropharyngeal involvement, in order to develop a personalized treatment plan and closely monitor potential life-threatening complications such as ICH.

Guillain-Barré Syndrome Presenting as Symmetrical Proximal Muscle Weakness: An Atypical Presentation.

Guillain-Barré syndrome occurs via molecular mimicry when a trigger sets off an immune response on peripheral nerve epitopes. Patients typically report an antecedent infection, such as an upper respiratory infection or Campylobacter jejuni gastroenteritis. It is typically characterized by progressive, symmetric muscle weakness with absent/decreased deep tendon reflexes. Most cases in the literature report that the paralysis begins in the legs distally and ascends to the extremities. Patients may have sensory symptoms or dysautonomia as well. Notable variant forms include acute motor axonal neuropathy, acute motor/sensory neuropathy, Miller Fisher syndrome, and Bickerstaff brainstem encephalitis. Diagnosis is confirmed if a lumbar puncture shows albuminocytologic dissociation (typically 45 to 200 mg/dL). Nerve conduction studies may also be considered but are not necessary. Management is largely supportive, but intravenous immunoglobulin and/or plasmapheresis for more severe cases may be considered.

Acute Hepatitis E-Associated Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is a rare autoimmune disorder of the peripheral nervous system that causes progressive weakness and sensory disturbances, usually following an infection or immunization. It has been associated with multiple causes, including bacterial and viral infections. Hepatitis E virus (HEV) is a common cause of acute viral hepatitis that can rarely develop neurological complications. We report a case of a 72-year-old man who developed GBS secondary to an acute HEV infection. He presented with numbness and weakness of the lower limbs that rapidly evolved into respiratory failure requiring mechanical ventilation and intravenous immunoglobulin therapy (IVIg). This case adds to the literature on the association between HEV infection and GBS and the importance of early detection of this rapidly progressive condition.

Predictors of long-term health-related quality of life in Guillain-Barré syndrome: A hospital-based study.

OBJECTIVE: This study aimed to determine the impact of patients' baseline clinical, neurophysiological data, and management plan of Guillain-Barré syndrome (GBS) on long-term quality of life (QoL) and to identify its potential predictors. METHODS: Seventy-nine GBS patients were recruited. On admission, participants were evaluated using the Medical Research Council (MRC) sumscore, GBS disability scale (GDS), and Erasmus GBS Respiratory Insufficiency Score (EGRIS). Neurophysiological data were collected, and a management plan was devised. MRC sumscore was repeated at nadir. MRC, GDS and Short Form Survey (SF-36) were assessed at first-year follow-up. RESULTS: The mean age was 37.84 ± 17.26 years, with 43 male patients (54.4%). QoL at one year correlated significantly with baseline clinical variables (age, number of days between weakness and admission, MRC sumscore at onset and nadir, high GDS, and EGRIS scores). Antecedent events, especially diarrhoea, neck muscle weakness, autonomic dysfunction, cranial nerve involvement, and mechanical ventilation (MV), associated with worse QoL. Axonal GBS patients had lower QoL than AIDP patients, and PE patients exhibited lower QoL than IVIG patients. Multiple regression analysis showed that older age, diarrhoea, number of days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, and MRC at onset and nadir and high GDS could predict poor QoL. CONCLUSION: Older age, more days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, diarrhoea, low MRC at onset and nadir, high GDS at onset, axonal type, and PE treatment were potential predictors of poor QoL in GBS.

Miller-Fisher Syndrome With Initial Manifestation of Rhinolalia Aperta: A Case Report and Literature Review.

Rhinolalia aperta (hypernasal speech) is rarely reported in patients with Miller-Fisher syndrome (MFS). Here, we report a patient with MFS who presented with rhinolalia aperta. A 35-year-old man with a history of alcohol abuse and hepatic cirrhosis presented with a three-day acute hypernasal voice change and numbness of both hands/thighs. After admission, the exam also revealed palatal hypomobility, decreased bilateral hand/thigh sensation, ataxic gait, dysmetria, areflexia, and bilateral abducens palsy. Serum immunoglobulin G (IgG) anti-GQ1b antibody titer was elevated (1:6400). A five-day intravenous IgG was administered with a robust clinical response. Oropharyngeal involvement in MFS can initially manifest with isolated hypernasal speech.

Predicting of Mechanical Ventilation and Outcomes by Using Models and Biomarker in Guillain-Barré Syndrome.

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) and the modified Erasmus GBS Outcome Score (mEGOS) are prognostic models used in the prediction of mechanical ventilation and outcome. Thus far, there are only few biomarkers for the prognosis prediction of GBS patients, and albumin level is one that is promising. METHODS: Patients diagnosed with GBS from 2013 to 2022 at Renmin Hospital, Wuhan University, China, were included. Patients hospitalized between 2016 and 2022 underwent short- and long-term follow-ups. The correlations between EGRIS/mEGOS and mechanical ventilation and outcome were evaluated. Serum albumin level was examined the day after admission. Furthermore, we also investigated whether the level of serum albumin was useful in predicting disease severity or poor outcome. RESULTS: In all, 145 patients were enrolled. Nineteen patients (13.1%) who required mechanical ventilation had higher Hughes GBS disability score (HGDS) at admission and discharge (P < 0.05 and P < 0.0001, respectively), shorter time from onset to admission and treatment (P < 0.01 and P < 0.001, respectively) and longer hospital stays (P < 0.001) than patients who did not require mechanical ventilation. High EGRIS scores were linked with the need for mechanical ventilation (r = 0.427, P < 0.001, AUC = 0.623). Seventy-one patients were admitted between 2016 and 2022. Of these, 65 patients had a 4-week follow-up and 61 had a 6-month follow-up. Higher mEGOS scores at admission and 7 days after admission significantly correlated with short- (P < 0.0001 and P < 0.0001) and long-term (P < 0.05 and P < 0.05) outcomes, respectively. No significant difference in outcome was found between different subtypes (4 weeks [P = 0.099] and 6 months [P = 0.172]). Patients with lower albumin level tended to have higher HGDS (at admission P < 0.05, at nadir P < 0.001, and at discharge P < 0.001) and higher properties of the need of mechanical ventilation (P < 0.05) and ICU stay (P < 0.05) than those with normal albumin levels. Those with low albumin levels were also unable to walk independently at 6 months (P < 0.01). CONCLUSIONS: mEGOS scores predicted the outcomes of GBS patients in China, and EGRIS score predicted the need for mechanical ventilation in these patients. Albumin level at admission correlated well with disease severity and outcomes.

Gitelman Syndrome Manifesting With Acute Hypokalemic Paralysis: A Case Report.

Gitelman syndrome (GS) is a rare renal tubulopathy, classically characterized by renal salt wasting and metabolic alkalosis. It is usually an incidental diagnosis, being asymptomatic or with mild symptoms. GS manifesting with acute flaccid paralysis is extremely uncommon. We report a case of GS that mimicked Guillain-Barré syndrome (GBS), manifesting with acute hypokalemic paralysis. A middle-aged male with no known comorbidities presented to our center with paresthesias of all four limbs for one month and progressive, asymmetric limb weakness over the past eight days. Neurological examination revealed hypotonia, global areflexia, and power ranging from 3/5 to 4/5 in all four limbs, leading to our initial clinical diagnosis of GBS. Our patient's laboratory panel revealed hypokalemia, hypomagnesemia, metabolic alkalosis, and hypocalcemia, characteristic of GS. Additionally, he had significantly elevated creatine phosphokinase, suggestive of rhabdomyolysis. Further urine studies revealed renal potassium wasting, confirming the diagnosis of GS. Whole exome genome sequencing for common causative genes and workup for autoimmune disease were both negative. With gradual electrolyte correction, the patient rapidly improved symptomatically. Our case highlights an uncommon initial presentation of GS and emphasizes the need for more literature on its manifestations from the Indian subcontinent.

Guillain Barré syndrome after combined diphtheria, tetanus, and acellular pertussis (DTaP) vaccine: A rare pediatric case report and review of literature.

A 20-month-old girl was diagnosed with Guillain - Barré syndrome (GBS) based on progressive muscle weakness, areflexia, and albuminocytologic dissociation of the cerebrospinal fluid. Despite timely and systematic treatment, she eventually became paralyzed. There is a temporal correlation between the girl's GBS and the DTaP vaccination, but the exact causal relationship between the two is still debatable. Furthermore, we summarized clinical features of other 45 published GBS cases after DTP vaccines (or vaccine substances containing tetanus) through a systematic review. The mean onset age, sex distribution, onset time after vaccination, detection of antiganglioside antibodies, and other basic clinical features of GBS after DTP vaccination (or vaccine substances containing tetanus) were analyzed. The temporal pattern of GBS after vaccination was similar to that of GBS after infection. Herein, we report this rare case of presumptive pediatric GBS after DTaP vaccination and review similar cases to draw the attention of medical personnel to similar events after vaccination. An association between DTP vaccines and GBS has been proposed, and the causal relationship between these two incidents are worthy further exploration. Moreover, surveillance and vigilance for GBS after vaccination are highly recommended.

Neuroborreliosis Presenting as Guillain-Barré Syndrome.

Lyme disease (LD) is the most common vector-borne disease in the United States. The early localized disease presents with erythema migrans and nonspecific constitutional symptoms. A neurological manifestation of LD (neuroborreliosis) is only seen in 10-15% of LD cases, and it typically presents as cranial neuritis or painful radiculitis. We report a case of a 33-year-old male who presented with progressive ascending bilateral lower extremities weakness with paresthesia in hands and feet following an upper respiratory tract infection and an abdominal rash. Cerebrospinal fluid (CSF) analysis revealed albuminocytologic dissociation. An electrodiagnostic study showed prolonged distal motor latency, conduction block, and absent F-wave response. Magnetic resonance imaging of the lumbar spine revealed enhancement of the cauda equina nerve roots. After a lack of improvement with intravenous immunoglobulin for presumed Guillain-Barré syndrome (GBS), Lyme serologies were sent and showed positive Lyme antibodies in serum and CSF as well as positive western blot IgM followed by IgG seroconversion a week later. The patient was started on IV ceftriaxone and doxycycline for four weeks with significant improvement in his symptoms. This is a rare case of LD presenting as GBS. Lyme can have diverse neurologic manifestations and should be considered in the differential diagnosis of GBS in the appropriate settings.

Onset of Guillain-Barre Syndrome and Transverse Myelitis Following COVID-19 Vaccination.

Guillain-Barre syndrome (GBS) and transverse myelitis (TM) are both neuro-inflammatory disorders that are attributed to dysfunctions of the peripheral nervous system and spinal cord, respectively. The two conditions involve immune-mediated destruction and inflammation of the nervous system and may present clinically as a weakness in the muscles, loss of normal sensations, and even paralysis of the body or extremities. Although the incidence of GBS and TM is quite rare, there have been reports of the two diseases developing in patients, either independently or concurrently, following COVID-19 vaccinations. In this case report, we present a patient (male) who lost functions and sensations in his lower extremities 60 days after he received the COVID-19 booster vaccine. The patient's blood work was unremarkable. Magnetic resonance imaging (MRI) of his thoracic spine and an electromyography study revealed evidence of nerve demyelination, which supports the diagnosis of GBS/TM overlap syndrome. He was ultimately treated with intravenous immunoglobulins (IVIGs) and gained back functions in his lower extremities.

Rare Sensory Ataxic Variant of Guillain-Barre Syndrome: A Case Report.

The sensory ataxic variant of Guillain-Barre syndrome (GBS) is a rare subtype, with limited case reports available. We present the case of a previously healthy 26-year-old female university student who presented with bilateral foot numbness and unsteady gait for five days, without limb weakness. There were no signs of infection or recent history suggestive of infection. Examination revealed reduced pain and light touch sensation, as well as proprioception impairment in the bilateral distal lower limb, accompanied by an ataxic gait. Bilateral upper and lower limb power was normal. Cerebrospinal fluid (CSF) studies showed albuminocytological dissociation, while nerve conduction studies indicated unrecordable sensory responses with normal motor responses. Through a comprehensive evaluation of history, examination, and investigations, other potential differential diagnoses were excluded. Then the patient was diagnosed with a sensory ataxic variant of Guillain-Barre syndrome and treated with intravenous immunoglobulin (IVIG). Over time, the patient demonstrated gradual improvement and was able to resume her university studies four months after discharge.

Hyperacute and Fulminant Guillain-Barré Syndrome Requiring Emergent Intubation.

Guillain-Barré syndrome (GBS) is an autoimmune-mediated acute polyneuropathy that can progress to life-threatening respiratory failure. The diagnosis and treatment of this pathology are complicated by the rarity of the disease and diversity in clinical presentation due to rarer, more dangerous subtypes of GBS. Understanding the time course of progression from onset to nadir of neurological deficits, maintaining a high index of suspicion, and close airway monitoring are essential in rapid diagnosis, securing the airway, and treatment.

Guillain-Barré syndrome following COVID-19 vaccination: An updated systematic review of cases.

Key Clinical Message: Guillain-Barré syndrome (GBS) is a rare but possible complication that may occur after COVID-19 vaccination. In this systematic review, we found that GBS presented in patients with an average age of 58. The average time for symptoms to appear was 14.4 days. Health care providers should be aware of this potential complication. Abstract: Most instances of Guillain-Barré syndrome (GBS) are caused by immunological stimulation and are discovered after vaccinations for tetanus toxoid, oral polio, and swine influenza. In this systematic study, we investigated at GBS cases that were reported after receiving the COVID-19 vaccination. Based on PRISMA guidelines, we searched five databases (PubMed, Google Scholar, Ovid, Web of Science, and Scopus databases) for studies on COVID-19 vaccination and GBS on August 7, 2021. To conduct our analysis, we divided the GBS variants into two groups, acute inflammatory demyelinating polyneuropathy and non-acute inflammatory demyelinating polyneuropathy (AIDP and non-AIDP), and compared the two groups with mEGOS and other clinical presentation In this systematic review, 29 cases were included in 14 studies. Ten cases belonged to the AIDP variant, 17 were non-AIDP (one case had the MFS variant, one AMAN variant, and 15 cases had the BFP variant), and the two remaining cases were not mentioned. Following COVID-19 vaccination, GBS cases were, on average, 58 years of age. The average time it took for GBS symptoms to appear was 14.4 days. About 56 percent of the cases (56%) were classified as Brighton Level 1 or 2, which defines the highest level of diagnostic certainty for patients with GBS. This systematic review reports 29 cases of GBS following COVID-19 vaccination, particularly those following the AstraZeneca/Oxford vaccine. Further research is needed to assess all COVID-19 vaccines' side effects, including GBS.

Detection of anti-ganglioside antibodies in Guillain-Barré syndrome.

Gangliosides are a class of glycosphingolipid molecules that are highly enriched in cellular membranes of the nervous system. The gangliosides associated with autoimmune diseases of the nervous system are mainly GM1, GD1a, GalNAc-GD1a, GM1b, GD3, CD1b, GT1a, and GQ1b. Multiple antibodies recognizing gangliosides are associated with some acute or chronic peripheral neuropathies, especially Guillain-Barré syndrome (GBS) and its clinical variants. Antibodies binding to gangliosides can activate complement system and recruit macrophages on the axolemma at the nodes of Ranvier of motor fibers, which are found in the course of GBS, causing axonal degeneration and reversible conduction block or conduction failure. Testing of anti-gangliosides autoantibodies is helpful for diagnosis of autoimmune peripheral neuropathies or support the diagnosis of the subtypes. These anti-gangliosides antibodies are usually detected by several qualitative or quantitative methods, particularly enzyme-linked immunosorbent assay (ELISA) and immunodot assays, which have been commercialized or established in-house worldwide. Herein, we introduce the methods and clinical applications of these assays in the diagnosis of autoimmune peripheral neuropathies. Anti-gangliosides antibodies are diagnostic markers of GBS subtypes. We use GBS as an example to explain the role of anti-gangliosides antibodies in the pathogenesis and diagnostic classification of neuropathies.