Complex Neurological Sequelae: Axonal Guillain-Barré Syndrome Post COVID-19 in a Young Patient.

Guillain-Barré syndrome (GBS) encompasses a spectrum of immune-mediated neuropathies, with axonal GBS representing a less common yet often severe subtype. This variant directly damages peripheral nerve axons, resulting in rapid and profound muscle weakness and sensory deficits. Axonal GBS has similar clinical features to the demyelinating form but is generally more severe with a less favorable prognosis. Here, we present a case of axonal GBS in a 46-year-old female following a mild COVID-19 infection, highlighting the diagnostic challenges and the importance of tailored therapeutic approaches and multidisciplinary care in managing this condition.

Guillain-Barré syndrome in a 63-year-old patient possibly triggered by ehrlichiosis. Case report.

Guillain-Barré syndrome is an immune-mediated acute demyelinating polyradiculoneuropathy, characterized by progressive flaccid weakness, triggered mainly by respiratory and gastrointestinal infections. We present the case of a 63-year-old male patient with a history of Ehrlichia infection, who consulted the internal medicine emergency department for lower back pain and progressive lower limb paresthesia, accompanied by decreased lower limb strength and nerve conduction velocity test, with results compatible with acute demyelinating sensorimotor polyradiculoneuropathy. To the best of our knowledge, this is the first documented case in Honduran medical literature; in our research, no other cases were found in Latin America or Europe. The importance of the topic and its dissemination in countries where Ehrlichia infection exists is that when cases of Guillain-Barré syndrome that cannot be associated with previous gastrointestinal or respiratory infection, they could be attributed to Ehrlichia infection as a possible cause; therefore, exhaustive preventive measures can be established regarding the transmitting vector of ehrlichiosis.

Causal effects of systemic inflammatory proteins on Guillain-Barre Syndrome: insights from genome-wide Mendelian randomization, single-cell RNA sequencing analysis, and network pharmacology.

Background: Evidence from observational studies indicates that inflammatory proteins play a vital role in Guillain-Barre Syndrome (GBS). Nevertheless, it is unclear how circulating inflammatory proteins are causally associated with GBS. Herein, we conducted a two-sample Mendelian randomization (MR) analysis to systematically explore the causal links of genetically determined systemic inflammatory proteins on GBS. Methods: A total of 8,293 participants of European ancestry were included in a genome-wide association study of 41 inflammatory proteins as instrumental variables. Five MR approaches, encompassing inverse-variance weighted, weighted median, MR-Egger, simple model, and weighted model were employed to explore the causal links between inflammatory proteins and GBS. MR-Egger regression was utilized to explore the pleiotropy. Cochran's Q statistic was implemented to quantify the heterogeneity. Furthermore, we performed single-cell RNA sequencing analysis and predicted potential drug targets through molecular docking technology. Results: By applying MR analysis, four inflammatory proteins causally associated with GBS were identified, encompassing IFN-γ (OR:1.96, 95%CI: 1.02-3.78, PIVW=0.045), IL-7 (OR:1.86, 95%CI: 1.07-3.23, PIVW=0.029), SCGF-ß (OR:1.56, 95%CI: 1.11-2.19, PIVW=0.011), and Eotaxin (OR:1.99, 95%CI: 1.01-3.90, PIVW=0.046). The sensitivity analysis revealed no evidence of pleiotropy or heterogeneity. Additionally, significant genes were found through single-cell RNA sequencing analysis and several anti-inflammatory or neuroprotective small molecular compounds were identified by utilizing molecular docking technology. Conclusions: Our MR analysis suggested that IFN-γ, IL-7, SCGF-ß, and Eotaxin were causally linked to the occurrence and development of GBS. These findings elucidated potential causal associations and highlighted the significance of these inflammatory proteins in the pathogenesis and prospective therapeutic targets for GBS.

Differential diagnosis of Guillain-Barré syndrome: steroid-responsive radiculopathy in Evans syndrome.

Guillain-Barré syndrome is the most common acute inflammatory demyelinating peripheral nerve condition. Occasionally, other autoimmune conditions can mimic Guillain-Barré syndrome but may require different diagnostic workup and treatment. We report here two patients with Evans syndrome, a rare hematological autoimmune condition who developed a subacute inflammatory radiculopathy. Similarities and distinguishing clinical and diagnostic features are discussed.

Early differential diagnosis between acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy in children: Clinical factors and routine biomarkers.

BACKGROUND: To identify clinical factors and biomarkers that could contribute to early differential diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) in the pediatric population, with limited evidence. METHODS: We conducted an observational retrospective study of children diagnosed with AIDP and A-CIDP between January 2014 and December 2022. Demographic data, clinical features, and routine biomarkers were also analyzed. Statistical analysis was used to identify significant features with high sensitivity and specificity. RESULTS: We included 91 AIDP and 17 A-CIDP patients. The A-CIDP group had an older median age (6.33 vs. 4.33 years, p = 0.017), required more complex immunotherapies (p < 0.001), and showed a longer time to nadir over 2 weeks (76.5 % vs. 7.7 %, p < 0.001). Gastrointestinal dysfunction (29.4 % vs. 6.59 %, p = 0.014) and numbness (35.3 % vs. 12.1 %, p = 0.027) were more prevalent in A-CIDP. The AIDP patients had a longer median hospitalization stays (13 vs. 11 days, p < 0.05), more prodromal events (90.1 % vs. 64.7 %, p = 0.013), and more frequent cranial nerve palsy (61.5 % vs. 5.88 %, p < 0.001). The disability scores on admission, discharge, and peak were worse in the AIDP group (p < 0.001). AIDP patients showed higher cerebrospinal fluid protein (p = 0.039), albumin quotient (p = 0.048), leukocytes (p = 0.03), neutrophils (p = 0.010), platelet count (p = 0.005), systemic inflammatory index (SII) (p = 0.009), and gamma-glutamyl transferase (p = 0.039). Multivariable regression identified two independent predictors of early A-CIDP detection: time from onset to peak beyond 2 weeks (OR = 37.927, 95%CI = 7.081-203.15) and lower modified Rankin Scale score on admission (OR = 0.308, 95%CI = 0.121-0.788). CONCLUSION: Our study found that when the condition continued to deteriorate beyond two weeks with a lower mRS on admission and possibly less cranial nerve involvement, we may favor the diagnosis of pediatric A-CIDP rather than AIDP.

Case Report: AMSAN variant of GBS complicating immediate postpartum period with severe dysautonomia leading to Posterior Reversible Encephalopathy Syndrome.

A 20's primiparous woman, following spontaneous expulsion of intrauterine death of the fetus at 30 weeks of gestation, presented on post-partum day 8 with acute onset flaccid quadriparesis and breathing difficulty, which had rapidly progressed to involve the legs on day 3 up to her upper limbs on post-partum day 5. Following examination, Guillain Barre Syndrome (GBS) with ascending diaphragmatic involvement was diagnosed, and plasma exchange was initiated. She developed raised blood pressure, headache, sudden onset visual loss with 2 episodes of generalized seizures on post-partum day 14. Brain MRI and clinical suspicion helped diagnose Posterior Reversible Encephalopathy Syndrome (PRES). The patient was treated with anticonvulsants and antihypertensive agents. She regained her vision over the next two days, completed the treatment for GBS, and made a good recovery with independence for advanced activities of daily living on follow-up.

Neurolymphomatosis mimicking a Guillain-Barré syndrome triggered by COVID-19 vaccination.

Guillain-Barré syndrome (GBS) is an acute disorder of the peripheral nervous system, causing flaccid paralysis, areflexia, and variable sensory involvement. Proximal as well distal muscles of the limbs can be involved, and in most severe and advanced cases progresses to respiratory failure and death. GBS is considered an autoimmune disease, and at the basis of the attack at the peripheral nervous system different mechanisms have been recognized, in particular viral infections or other immune stimulations. Cranial nerve involvement in patients with diffuse large B-cell lymphoma (DLBCL) and primary central nervous system lymphoma are rare conditions that could present with similar clinical features. Here we present a case of a 36-year-old man hospitalized for acute polyradiculoneuritis of the cranial nerves and lumbar roots that arose a 14 days after severe acute respiratory syndrome COVID-19 2 (Sars-CoV-2) vaccination. Most of the main criteria for the diagnosis of GBS were met, including clinical and electrophysiological criteria. Albuminocytologic dissociation and high protein level in cerebrospinal fluid were also found. Therefore, the patient was treated with a cycle of intravenous immunoglobulin (IVIG) with notable improvement of symptoms and gradual recovery of motility. A five months later, following SARS-CoV-2 infection, the patient presented with worsening of neurological symptoms and was readmitted to the hospital. He underwent instrumental tests again and was treated with repeated cycles of IVIG and then with a cycle of plasmapheresis without any improvement. In the following 10 days he developed very serious conditions; he was transferred to intensive care unit and deceased after 6 days. The cause of the neurological syndrome was determined only after autoptic analysis, which revealed the presence of primary peripheral nervous system (PNS) DLBCL. The reported case highlights that GBS-like presentation always requires a careful differential diagnosis, and physicians should also consider the possibility of an occult cancer.

Concurrent Miller Fisher Syndrome and Immune Thrombocytopenic Purpura: A Case Report and Review of the Literature.

Guillain-Barre syndrome (and its subvariants) and immune thrombocytopenic purpura, while both autoimmune disorders provoked by viral infection, rarely coincide. We present the case of a young man who developed both conditions after URI, review prior cases of comorbidity in the literature, and describe their pathophysiology, diagnosis, and management.

Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain-Barre Syndrome in the United States-2001 to 2018.

INTRODUCTION/AIMS: Randomized controlled trials show that repeat intravenous immunoglobulin (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination therapy) have no additional therapeutic benefit in Guillain-Barre Syndrome (GBS) non-responders. Furthermore, the delineation between GBS and Acute Onset CIDP (A-CIDP) can be particularly challenging and carries therapeutic implications. We aimed to evaluate the presence of repeat IVIG, combination therapy, and diagnostic reclassification from GBS to CIDP. METHODS: We performed a retrospective study of a large healthcare database for patients with GBS in the US from 2001 to 2018. We identified individuals initially diagnosed with GBS and later re-classified as CIDP. Multivariable logistic regression models were developed to determine associations between patient factors and repeat IVIG dosing, combination therapy, and diagnostic re-classification from GBS to CIDP. RESULTS: We identified 2325 patients with GBS. A total of 39.7% received repeat IVIG and 6.1% received combination therapy. The proportion of individuals initially diagnosed with GBS and then re-classified as CIDP was 32.0%. Repeat IVIG, combination therapy, and diagnostic reclassification remained stable over time. Female sex (OR 0.79, 95% CI 0.65-0.96) and medium-high net worth (OR 0.64, 95% CI 0.45-0.90) associated with repeat IVIG therapy, while Asian ethnicity associated with diagnostic re-classification from GBS to CIDP (OR 1.77, 95% CI 1.09-2.86). DISCUSSION: Repeat IVIG dosing was quite common in GBS before newer trials suggesting harm in non-responders, and IVIG/PLEX combination therapy continues to persist despite strong evidence against use in non-responders. Further, nearly one in three patients initially diagnosed with GBS is subsequently diagnosed with CIDP, but the reasons are unclear.

Variation in worldwide incidence of Guillain-Barré syndrome: a population-based study in urban China and existing global evidence.

Background and objectives: Geographical variation existed in the incidences of Guillain-Barré syndrome (GBS), but no national population-based study has evaluated the incidences of GBS in China. This study aimed to estimate the incidence of GBS in urban China and evaluate the worldwide variation in the incidence of GBS. Methods: Firstly, we did a population-based study to calculate the incidence of GBS in urban China based on the National Urban Medical Insurance database from 2013 to 2017. To identify GBS cases, natural language processing was used first for handling the lengthy and unstructured diagnostic information and then checked by prestigious neurologists. Secondly, a systematic review and meta-analysis were performed to analyze the incidence of GBS worldwide. Up to July 4, 2022, Medline, Embase, and Web of Science were retrieved to identify the population-based studies regarding the incidence of GBS. The basic information and the statistics regarding incidence were extracted. Quality assessment considered sample representativeness, condition assessment, and statistical methods. Results: A total of 1.44 billion person-years in insurance data was covered, with 3,534 GBS cases identified. The annual incidences of GBS in urban China between 2013 and 2017 ranged from 0.41 (95% CI: 0.27 to 0.58) to 0.58 (95% CI: 0.38 to 0.82) per 100,000 person-years. The incidence was the highest in Northwest China and the lowest in Northeast China. The meta-analysis included 122 articles. The quality assessment showed that the quality scores of 43.3% of studies were ≥ 0.75 (the total score is 1). The global incidence of GBS was 1.12 (95% CI: 0.98 to 1.27) per 100,000 person-years. The incidences in West Europe, South Asia, and North Europe were higher, while the incidences in Australia and New Zealand, Southeast Asia, and North Africa were lower. The incidence of enteric infections was positively associated with the incidence of GBS (coefficient=0.0000185, P=0.007). The incidence in Europe, Australia, and America rose significantly from 1960 to 2020 (coefficient=0.01, t=2.52, P=0.015). Discussion: There is a clear regional variation of the GBS incidence at both national and global levels. Careful control of enteric infections should be conducted to reduce the disease burden.

Unraveling the neurological intricacies: a rare case of Guillain-Barre syndrome in dengue fever.

Dengue, caused by the dengue virus, presents with various clinical manifestations, including rare neurological complications. Guillain-Barre Syndrome (GBS), an immune-mediated polyradiculoneuropathy, is a rare complication, often triggered by antecedent infections. Herein, we report the case of a 30-year-old male presenting with GBS following dengue fever. His clinical course revealed classic GBS symptoms, including ascending weakness and bulbar involvement, with no noted infection that could plausibly explain a trigger for GBS. Diagnosis entailed cerebrospinal fluid analysis and nerve conduction studies which confirmed acute inflammatory demyelinating polyradiculoneuropathy. Treatment involved plasmapheresis, yielding a positive response. This case underscores the association between dengue and GBS, emphasizing the need for heightened clinical suspicion in endemic regions like Nepal.

Temporal trends and regional variations in mortality related to Guillain-Barré syndrome in the United States: a retrospective study from 1999 to 2020.

AIM: Guillain-Barré syndrome (GBS) is an autoimmune neurological disorder, with an estimated 6.4% increase in cases worldwide from 1990 to 2019. We aim to identify the GBS-related mortality trends in the US stratified by age, sex, race, and region. METHODS: We used data from the CDC-WONDER database to calculate crude (CMR) and age-adjusted mortality rates (AAMRs) per 1,000,000 people. We examined the temporal trends through annual percent change (APC) and the average annual percent change (AAPC) in rates using Joinpoint regression. RESULTS: From 1999 to 2020, a total of 10,097 GBS-related deaths occurred in the US. The AAMR decreased till 2014 (APC: -1.91) but increased back to initial levels by 2020 (APC: 3.77). AAMR was higher in males (1.7) than females (1.1), decreasing till 2015 for females and 2014 for males, but increasing thereafter only for females. Non-Hispanic (NH) American Indians or Alaska Natives displayed the highest AAMR (1.8) while NH Asians or Pacific Islanders displayed the lowest (0.6). AAMRs also varied by region (West: 1.5; South: 1.5; Midwest: 1.4; Northeast: 1.1). Rural regions exhibited a higher AAMR (1.7) than urban regions (1.3). Most deaths occurred in medical facilities (60.99%). The adults aged ≥85 years exhibited an alarmingly high CMR (14.0). CONCLUSIONS: While the mortality rates for GBS initially declined till 2014, they climbed back up afterwards. Highest mortality was exhibited by males and NH American Indians or Alaska Natives, residents of rural regions, and adults ≥85 years. Equitable efforts are needed to reduce the burden on high-risk populations.

Role of Autologous Platelet-Rich Fibrin in Chronic Non-healing Ulcers With Various Etiologies in a Tertiary Care Rehabilitation Centre: A Case Series.

Introduction Chronic non-healing ulcers are defined as a discontinuity or break in the integrity of skin that is not healing in a reasonable period of time due to an underlying systemic etiology. Despite using conventional initial treatment and many other available dressing options, such wounds are difficult to completely heal, thus affecting the progress of rehabilitation measures and compromising functional improvement and quality of life. Materials and methods In this case series, platelet-rich fibrin (PRF) was applied to eight wounds from six patients. The patients included had various etiologies (including spinal cord injury, peripheral vascular disease, Guillain-Barré syndrome, and diabetic foot ulcer) with chronic non-healing wounds over different anatomical locations on the body. Pressure ulcer scale for healing (PUSH) score, surface area, and volume of the wounds were evaluated and monitored weekly after PRF dressing. We have applied PRF every week. On average, two PRF dressings were applied, the maximum being three applications. Results The maximum healing rate in terms of PUSH score was observed to be 3.84% per day, and the minimum was 1.19% per day. The maximum healing rate in terms of surface area was observed to be 5.89% per day, and the minimum was 1.78% per day. Three of the wounds showed complete closure. The maximum follow-up period was 10 weeks. The percentage mean Functional Independence Measure (FIM) improvement was calculated to be 15.87% ± 14.04 during the course of hospitalization after PRF application. Conclusion Based on the results, we can conclude that PRF showed accelerated improvement in the healing of chronic non-healing ulcers of various etiologies at different anatomical locations. It has proven to be a safe and effective method, thereby improving their quality of life and functional independence in performing activities of daily living. To our knowledge till date, no other study in a rehabilitation setting has been done on patients having non-healing ulcers due to various etiologies and at different anatomical locations.

Heterotopic ossification Post-Guillain-Barre syndrome in Saudi Arabia: a case report.

Background: Heterotopic ossification (HO) is the formation of bone within the soft tissues. It can be a complication of Guillain-Barre syndrome (GBS). There are many risk factors for HO, including male sex, mechanical ventilation, and neurogenic trauma. Myelin and axons are the main targets and areas of injury in GBS, an autoimmune-inflammatory neuropathy. Literature shows that this may possibly be associated with the initial administration of the COVID-19 vaccine and GBS. Presentation of the case: A 27-year-old male was diagnosed with bile reflux gastritis. Days later, he presented to the emergency room (ER) with progressive weakness and a critical condition that required ICU. The patient undergoes intubation and remains in the ICU for 4 months. The patient, after extensive rehabilitation, started to complain of left hip pain and limitations of motion. Radiographs confirmed the HO diagnosis. Past drug history showed patients received a single dose of the COVID-19 vaccine 15 days before presentation to the ER. Discussion: There is no clear association between the COVID-19 vaccination and GBS. HO is the formation of abnormal bone within soft tissue. HO post-GBS usually affects large joints like the hips, knees, and shoulders. Researchers poorly understand the pathogenesis of GBS. Conclusion: Despite the absence of a definitive correlation between GSB and the COVID-19 vaccine. Physicians should maintain a state of suspicion while treating patients with a progressive weakness. Additional research is required.

Unraveling Complexity: Acute Intermittent Porphyria Complicated by Rhabdomyolysis and Acute Pancreatitis.

Acute intermittent porphyria (AIP) is a rare autosomal dominant disorder characterized by defective porphyrin metabolism in the blood. It manifests through variable clinical features, among these are abdominal pain, nausea, vomiting, peripheral neuropathy, and seizure. The diverse presentation of AIP poses substantial diagnostic challenges due to its potential to mimic other medical conditions, delaying early recognition and intervention. Management strategies of AIP involve a multifaceted approach, focusing on symptom relief and attack cessation. Early recognition and intervention are pivotal in optimizing patient outcomes, highlighting the importance of heightened clinical suspicion and precise diagnostic pathways. We present a unique case of a 34-year-old female who presented to the emergency department with severe abdominal pain, oliguria, and progressive sensory and motor deficits. Despite exhibiting hallmark symptoms suggestive of AIP, the absence of distinctive "attack periods" added complexity to the diagnostic process, requiring the exclusion of other medical conditions with similar overlapping symptoms.

Unilateral Vocal Cord Paralysis in a Patient with Anti-Galactocerebroside Antibodies: A Case Report.

Anti-galactocerebroside (Gal-C) antibodies are present in patients with conditions such as Guillain-Barré syndrome and mycoplasma pneumonia. We report a rare case of left vocal cord paralysis in a patient with anti-Gal-C IgG antibodies that improved after administeration of antivirals and steroids.

Exploring the "Two-Hit" Phenomenon of Guillain-Barré Syndrome Following Respiratory Syncytial Virus and Influenza Vaccinations.

Guillain-Barré syndrome (GBS) is a neurological disorder characterized by peripheral, autoimmune-mediated demyelinating polyneuropathy, which can cause muscle weakness and paralysis. While most cases are triggered by respiratory or gastrointestinal infections, vaccinations have also been linked to GBS pathogenesis. The association of the influenza vaccine and GBS, notably prevalent during the 1976 United States swine flu pandemic, has significantly decreased with contemporary seasonal influenza vaccines. At the same time, cases of GBS have been reported with newer vaccines, like the recently approved respiratory syncytial virus (RSV) vaccines. However, their exact relationship with autoimmune demyelinating polyneuropathy remains unknown. In this report, we present a case of a 60-year-old man who developed GBS two weeks after receiving the new Pfizer's RSV vaccine in conjunction with the influenza vaccine for the first time.

A Neuropathy Mimic: Statin-Induced Myopathy Presenting as Guillain-Barré Syndrome.

Statins are widely used to manage dyslipidemia and prevent cardiovascular diseases due to their effectiveness and general safety profile. However, they can sometimes cause severe muscle-related adverse effects, presenting diagnostic challenges when symptoms overlap with other conditions. This case report describes a middle-aged woman who presented to the emergency department with bilateral lower limb weakness, initially suggesting Guillain-Barré syndrome (GBS). Despite her history of low-grade fever and diarrhea, primary and secondary surveys, including electrocardiogram, blood gas analysis, and nerve conduction studies, showed no definitive signs of GBS. The patient had a recent history of percutaneous transluminal coronary angioplasty and was on dual antiplatelet therapy and rosuvastatin. Elevated creatine kinase levels and exclusion of other differential diagnoses led to the diagnosis of statin-induced myopathy, a rare but severe adverse effect of statins. The patient was treated with intravenous fluids, cessation of statins, and sessions of hemodialysis and plasmapheresis, resulting in significant improvement and eventual recovery of muscle power and neurological function. This case highlights the importance of recognizing statin-induced myopathy in patients with muscle weakness and emphasizes the need for thorough clinical evaluation to differentiate it from other conditions such as GBS. Further research is warranted to understand the pathophysiology of statin myopathy and identify at-risk populations.

A Case of Type 2 Diabetes Mellitus Revealed Transient Positivity of Glutamic Acid Decarboxylase (GAD) Antibodies Following Immunoglobulin Administrations.

Glutamic acid decarboxylase (GAD) antibodies are frequently measured in diabetes care as islet-associated autoantibodies that are useful in the diagnosis of type 1 diabetes. However, GAD antibodies derived from other persons may contaminate immunoglobulin preparations, and there have been cases of transiently positive GAD antibodies after intravenous immunoglobulin (IVIg) in patients who were originally negative for GAD antibodies. Clinicians may be unaware of such contamination and misdiagnose some cases as type 1 instead of type 2 diabetes mellitus based on positivity for GAD antibodies. Herein, we present a case of type 2 diabetes mellitus that revealed transiently positive GAD antibodies following immunoglobulin administrations. A 68-year-old woman with a medical history of diabetes mellitus was admitted to our hospital for the treatment of Guillain-Barré syndrome, and IVIg was started on the day of admission. Blood tests on admission revealed negative for GAD antibodies but showed weak positivity on day one after IVIg. Afterward, GAD antibodies turned negative on day 72. Immunoglobulin preparations were revealed to have a high concentration of GAD antibodies. Based on changes in GAD antibody titers and all negativity for anti-insulinoma-associated antigen-2 (IA-2), insulin, and zinc transporter 8 (ZnT8) antibodies, the patient was diagnosed with type 2 diabetes mellitus rather than slowly progressive type 1 diabetes mellitus (SPIDDM). This case demonstrates that it is important for the medical clinician to be aware of the possible presence of GAD antibodies in immunoglobulin preparations and to measure antibody titers before and after their use for diagnosing the type of diabetes mellitus.