Mimics of Guillain-Barré Syndrome in Pediatric Patients Admitted to the Neuro-Intensive Care Unit of a Tertiary Care Hospital-A Case Series.

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis-three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics.

Effect of intravenous immunoglobulin and plasmapheresis on nerve conduction parameters compared to the natural course of Guillain-Barré syndrome.

BACKGROUND: Intravenous immunoglobulin (IVIg) and plasmapheresis (PLEX) are recommended in moderate to severe Guillain-Barré Syndrome (GBS), but there is paucity of studies evaluating its effect on nerve conduction studies (NCS). We report the effect of IVIg and PLEX on the NCS parameters and clinical outcomes compared to natural course (NC) of GBS patients. METHOD: Moderate to severe GBS patients were included based on clinical, cerebrospinal fluid, and NCS finding. Six motor and sensory nerves were evaluated at admission, one month and 3 months, and NCS subtyping was done. Axonal and demyelination burden in motor nerves and early reversible conduction block (ERCB) were noted. Patients receiving IVIg, PLEX or on NC were noted. Outcome was defined at 3 months into complete, partial and poor using a 0-6 GBS Disability Scale (GBSDS). RESULT: Seventy-two patients were included, whose median age was 36 years and 22(30.6 %) were females. 44 patients received IVIg, 9 PLEX and 19 were in NC, and they had comparable peak disability. AIDP was the dominant subtype at admission (58.3 %), which remained so at 3 months (50 %). The shift of subtypes was the highest from the equivocal group followed by AMAN and the least from AIDP. IVIg and PLEX group had more reduction in axonal burden and had ERCB compared to NC. 33(44 %) patients had complete recovery, and 40(55.5 %) patients had concordance in clinical and neurophysiological outcome. CONCLUSION: Transition of GBS subtype may occur at follow-up from all the subtypes, the highest from the equivocal and the lowest from the AIDP group. IVIg/PLEX treatment may help in reducing conduction block and axonal burden.

Comparison of Clinical Features, Short-Term Outcome of Guillain-Barré Syndrome Between Adults and Children: A Retrospective Study in Vietnam.

BACKGROUND: Despite extensive research on Guillain-Barré syndrome (GBS) in adults and children, there is a lack of comparison regarding short-term outcomes in various age groups. Our study aims to elucidate the differences in clinical features and short-term outcomes in Vietnam. METHODS: After retrospective data collection, we compared clinical features in patients with GBS aged ≤16 years at Children's Hospital 2 and aged >16 years at University Medical Center Ho Chi Minh City from 2017 to 2021. A positive short-term outcome was recorded if patients had a GBS Disability Score of 0 to 2 at hospital discharge. RESULTS: We analyzed 109 adults (58.7% males; mean age 50.6 ± 17.7) and 111 children (58.6% males; mean age 7.2 ± 4.9). Comparable antecedent infection and immunization incidence in both groups were observed (35.8% vs 45.9%, P > 0.05). Pain and sensory disturbance were the most common onset symptom in adults (57.8%), whereas lower limb weakness predominated in children (61.3%). Ophthalmoplegia (18.3% vs 5.4%), pain, sensory disturbance (85.3% vs 67.6%), ataxia (33.0% vs 15.3%) were more prevalent in adults (P < 0.05). The axonal subtype was prominent in both adults (51.4%) and children (53.2%). Patients were classified into: classic GBS (49.5% and 68.5%), GBS variants (11.0% and 15.3%), classic Miller Fisher syndrome (MFS) (1.8% and 1.8%), MFS variants (2.8% and 0%), and GBS/MFS overlap (34.9% and 14.4%). Short-term outcomes did not significantly differ based on age. CONCLUSIONS: Age-related variations in clinical features were observed, but adults and children exhibited similar short-term functional outcomes.

Guillain-Barré syndrome: immunopathogenesis and therapeutic targets.

INTRODUCTION: Guillain-Barré syndrome (GBS) is a group of acute immune-mediated disorders in the peripheral nervous system. Both infectious and noninfectious factors are associated with GBS, which may act as triggers of autoimmune responses leading to neural damage and dysfunction. AREAS COVERED: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and its vaccines as well as flaviviruses have been associated with GBS, although a robust conclusion has yet to be reached. Immunomodulatory treatments, including intravenous immunoglobulins (IVIg) and plasma exchange (PE), have long been the first-line therapies for GBS. Depending on GBS subtype and severity at initial presentation, the efficacy of IVIg and PE can be variable. Several new therapies showing benefits to experimental animals merit further investigation before translation into clinical practice. We review the state-of-the-art knowledge on the immunopathogenesis of GBS in the context of coronavirus disease 2019 (COVID-19). Immunomodulatory therapies in GBS, including IVIg, PE, corticosteroids, and potential therapies, are summarized. EXPERT OPINION: The association with SARS-CoV-2 remains uncertain, with geographical differences that are difficult to explain. Evidence and guidelines are lacking for the decision-making of initiating immunomodulatory therapies in mildly affected patients or patients with regional subtypes of GBS.

Efgartigimod in the treatment of Guillain-Barré syndrome.

BACKGROUND: Guillain-Barré Syndrome (GBS) is caused by immunoglobulin G (IgG) autoantibodies. Efgartigimod, a human IgG antibody Fc fragment that acts as a natural ligand for the FcRn, can increase IgG degradation, which thus may be a promising therapeutic drug for GBS. CASE PRESENTATION: The two patients presented with postinfectious and acute flaccid paralysis. On admission, they were bedridden. Nerve conduction studies indicated peripheral neuropathy. GBS was suspected and they are treated with two doses of efgartigimod (10 mg/kg) within 5 days. Their muscle strength improved gradually and 4 weeks after the initial dose, they could walk independently. Following the first dose, Patient 1 complaint of muscle soreness, which subsided the next morning. Patient 2 was intubated due to respiratory failure the day after the initial dose, and did not report other adverse effects. DISCUSSION: In GBS patients, two doses of efgartigimod (10 mg/kg) were effective in rapidly improving muscle strength, with a satisfactory safety profile. The findings suggest a potential role for efgartigimod in modifying the disease process in GBS patients. CONCLUSION: Efgartigimod seems effective and safe in the treatment of GBS. This study indicates the potential role of efgartigimod as a novel treatment option for GBS. Well-designed clinical trials should be conducted.

CYBA allelic variants are associated with severity and recovery in Guillain-Barré syndrome.

BACKGROUND AND AIMS: Guillain-Barré syndrome (GBS) is a rare, acute neuropathy characterized by ascending muscle weakness. Age, axonal GBS variants, and antecedent Campylobacter jejuni infection are associated with severe GBS, but the detailed mechanisms of nerve damage are only partly explored. Pro-inflammatory myeloid cells express NADPH oxidases (NOX) that generate tissue-toxic reactive oxygen species (ROS) that are implicated in neurodegenerative diseases. This study analyzed the impact of variants of the gene encoding the functional NOX subunit CYBA (p22phox ) on acute severity, axonal damage, and recovery in adult GBS patients. METHODS: Extracted DNA from 121 patients was genotyped for allelic variation at rs1049254 and rs4673 within CYBA using real-time quantitative polymerase chain reaction. Serum neurofilament light chain was quantified by single molecule array. Patients were followed for severity and motor function recovery for up to 13 years. RESULTS: CYBA genotypes linked to reduced formation of ROS, i.e. rs1049254/G and rs4673/A, were significantly associated with unassisted ventilation, shorter time to normalization of serum neurofilament light chain and shorter time to regained motor function. Residual disability at follow-up was confined to patients carrying CYBA alleles associated with high formation of ROS. INTERPRETATION: These findings implicate NOX-derived ROS in GBS pathophysiology and CYBA alleles as biomarkers of severity.

CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome.

BACKGROUND AND OBJECTIVES: To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study. METHODS: Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/µL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%). RESULTS: In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/µL in 1,005 patients (83%), 5-49 cells/µL in 200 patients (16%), and ≥50 cells/µL in 13 patients (1%). DISCUSSION: ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/µL, is compatible with GBS after a thorough exclusion of alternative diagnoses. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

Elevated blood and cerebrospinal fluid glucose levels affect the severity and short-term prognosis of Guillain-Barré syndrome.

OBJECTIVE: This study aimed to explore the correlation of elevated glucose levels in the blood and cerebrospinal fluid with the progression and short-term prognosis of Guillain-Barré syndrome (GBS). METHODS: The medical records of 982 patients who were diagnosed with GBS in 31 representative tertiary hospitals, located in 14 provinces in southern China, were collected and retrospectively reviewed. Patients were grouped according to the levels of fasting plasma glucose (FPG) and cerebrospinal fluid (CSF) glucose, as well as the concentration of blood hemoglobinAlc (HbA1c). The Hughes grade scale was used to quantify functional outcomes. RESULTS: Compared to patients with normal FPG and CSF glucose levels, those in the high FPG and high CSF glucose groups were characterized by a higher proportion of severe patients (HFGS ≥ 3) at admission (58.8 vs. 73.1, P = 0.000; 57.6 vs. 71.2, P = 0.000), at nadir (67.4 vs. 83.0, P = 0.000; 66.2 vs. 80.4, P = 0.000), and at discharge (29.8 vs. 46.3, P = 0.000; 26.4 vs. 45.0, P = 0.000). Patients in the high HbA1c group also had more severe disability at admission (74.6 vs. 56.1, P = 0.005) and at nadir (80.3 vs. 64.3, P = 0.012) compared to the normal HbA1c group. Moreover, elevated levels of FPG and CSF glucose were significantly correlated with more severe disability at admission, at nadir, and at discharge. CONCLUSIONS: The present study showed that elevated glucose levels in the blood and cerebrospinal fluid were associated with the severity and short-term prognosis of GBS. TRIAL REGISTRATION: chicTR-RRc-17,014,152. ABBREVIATIONS: GBS, Guillain-Barré syndrome; FPG, fasting plasma glucose; CSF, cerebrospinal fluid; HFGS, Hughes Functional Grading Scale; HbA1c, hemoglobin A1c. DM, diabetes mellitus; NCS, nerve conduction study; AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor sensory axonal neuropathy; MV, mechanical ventilation.

iMAX: A new tool for assessment of motor axon excitability. A multicenter prospective study.

OBJECTIVE: This study was undertaken to establish by a multicentric approach the reliability of a new technique evaluating motor axon excitability. METHODS: The minimal threshold, the lowest stimulus intensity allowing a maximal response by 1 mA increments (iUP) and then by 0.1 mA adjustments (iMAX) were prospectively derived from three nerves (median, ulnar, fibular) in four university centers (Liège, Marseille, Fraiture, Nice). iMAX procedure was applied in 28 healthy volunteers (twice) and 32 patients with Charcot-Marie-Tooth (CMT1a), chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barré syndrome (SGB) or axonal neuropathy. RESULTS: Healthy volunteers results were not significantly different between centers. Correlation coefficients between test and retest were moderate (> 0.5). Upper limits of normal were established using the 95th percentile. Comparison of volunteers and patient groups indicated significant increases in iMAX parameters especially for the CMT1a and CIDP groups. In CMT1a, iMAX abnormalities were homogeneous at the three stimulation sites, which was not the case for CIDP. CONCLUSIONS: The iMAX procedure is reliable and allows the monitoring of motor axon excitability disorders. SIGNIFICANCE: The iMAX technique should prove useful to monitor motor axonal excitability in routine clinical practice as it is a fast, non-invasive procedure, easily applicable without specific software or devices.

Covid-19 associated Guillain-Barré syndrome: A series of a relatively uncommon neurological complication.

BACKGROUND AND AIMS: The COVID-19 pandemic has turned the world topsy turvy since its emergence and has claimed innumerable lives worldwide. Neurological manifestations of the disease have raised several eyebrows around the world among which Guillain-Barré syndrome (GBS) deserve special mention. Although majority of the cases of the coronavirus disease 2019 (COVID-19) present with respiratory symptoms, extrapulmonary manifestations are being increasingly reported. We conducted this study to analyze detailed clinical presentations and outcome in a series of eight cases (n = 8) with COVID-19 associated GBS. METHODS: An observational prospective study was conducted among patients with post-infectious/para-infectious GBS. 8 patients were subclassified into acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN) as per electrodiagnostic criteria and were followed up from admission to 6 months post discharge, to obtain a comprehensive clinical profile and outcome in these patients. RESULTS: The diagnosis of GBS was confirmed as per Asbury criteria, supported by electrodiagnostic features in nerve conduction velocity test. Among the series of 8 patients, 3 were diagnosed as AIDP, 3 had AMAN and the remaining 2 patients had AMSAN. 3 patients of GBS were afebrile and were diagnosed as COVID-19 after a positive assay on routine screening. Cerebro-spinal fluid analysis for SARS-Cov-2 RT-PCR and serum anti-ganglioside antibodies were negative in all the patients. CONCLUSION: GBS in patients with COVID-19 should be differentiated from critical illness neuropathy and myopathy. Early diagnosis is important as it is associated with poor outcome and prolonged invasive ventilation.

Guillain-Barre syndrome during COVID-19 pandemic: experience from a referral healthcare center in Mexico. / Síndrome de Guillain-Barré durante la pandemia de COVID-19: experiencia de un centro de referencia en México.

INTRODUCTION: To describe clinical characteristics and electrophysiological variants of GBS cases during the pandemic, we carried out a comparative analysis between SARS-CoV2 related GBS and non-SARS-CoV2 patients and then compared to the 2019 cases. PATIENTS AND METHODS: We carried out a cross-sectional study of GBS patients diagnosed according to Asbury and Cornblath criteria. We collected information on clinical and paraclinical variables. We defined a SARS-CoV-2 related GBS case according to the description of Ellul et al. We used Hadden criteria to classify the electrophysiological variants. We performed a comparative analysis between groups. RESULTS: Fourty-two patients were diagnosed with GBS in 2020, men 64.2%, age 46 ± 17.4 years, patients with obesity/overweight 42.8%, previous diarrhea 31%, history of respiratory tract infection 14.2%. Guillain Barre Disability Scale = 3 points 71.4% and, cranial nerve involvement 69%. The most frequent electrophysiological variant was acute inflammatory demyelinating polyradiculoneuropathy (AIDP) 53.5%. Seven (16.6%) cases were SARS-CoV2 related, four men, age 43.4 ± 13.4 years. When comparing patients with GBS in 2020 vs patients in 2019, we observed a decrease in the previous infection history during 2020 (45.2% vs 73.3%, p-value = 0.005) and a decrease in previous respiratory infection (14.2% vs 33.3%, p = 0.045), as well as a higher frequency of cranial nerve involvement, and albuminocytologic dissociation. CONCLUSIONS: SARS-CoV2 virus infection preventive measures may be impacting the presentation of post-infectious diseases such as GBS. We did not observe an increase in GBS cases during 2020. Also, the AIDP variant were more frequent in our population in the COVID-19 pandemic.

TITLE: Síndrome de Guillain-Barré durante la pandemia de COVID-19: experiencia de un centro de referencia en México.Introducción. Se trata de describir las características clínicas y variantes electrofisiológicas de los casos de síndrome de Guillain-Barré (SGB) durante la pandemia. Llevamos a cabo un análisis comparativo entre pacientes con SGB relacionado con el SARS-CoV-2 y sin antecedente del virus, y posteriormente realizamos una comparación con los casos de 2019. Pacientes y métodos. Se llevó a cabo un estudio transversal de los pacientes con diagnóstico de SGB según los criterios de Asbury y Cornblath. Se recolectaron información clínica y variables paraclínicas. Definimos el SGB relacionado con el SARS-CoV-2 conforme a la descripción de Ellul et al. Se utilizaron los criterios de Hadden para la clasificación de las variantes electrofisiológicas. Por último, realizamos un análisis comparativo entre grupos. Resultados. Se diagnosticó a 42 pacientes con SGB en 2020, un 64,2% hombres, con una edad de 46 ± 17,4 años, un 42,8% con obesidad/sobrepeso, un 31% con historia de diarrea previa y un 14,2% con infección respiratoria previa. El 71,4% tuvo una puntuación en la Guillain-Barré Disability Score igual o mayor que 3 puntos y el 69% tenía afectados los nervios del cráneo. La variante electrofisiológica más común fue la polirradiculoneuropatía desmielinizante inflamatoria aguda (PDIA; 53,5%). Siete (16,6%) casos tuvieron relación con el SARS-CoV-2, cuatro hombres, con edad de 43,4 ± 13,4 años. Al realizar la comparación entre pacientes con SGB de 2020 frente a los de 2019, observamos un decremento en el antecedente de infección previa en 2020 (45,2 frente a 73,3%; p = 0,005) y un decremento específico en la historia de infección respiratoria (14,2 frente a 33,3%; p = 0,045), así como una mayor frecuencia de afectación de los nervios del cráneo y de disociación albuminocitológica. Conclusiones. Las maniobras preventivas para la infección por el SARS-CoV-2 impactan directamente en la presentación de enfermedades postinfecciosas como el SGB. No observamos un incremento en los casos de SGB durante 2020. Asimismo, la variante de PDIA fue la más frecuente en nuestra población durante la pandemia de COVID-19.

Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome: a case report of a rare complication following Dengue-Chikungunya co-infection.

Pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré Syndrome (GBS) is characterized by weakness in cervicobrachial and oropharyngeal region, together with areflexia of upper limbs. Being an uncommon variant, it is often misdiagnosed as other neurological conditions resembling GBS. Although most of the cases occur as a post-infectious complication, no reports describing its development following dengue-chikungunya co-infection have been documented. A young female presented with a progressive history of swallowing difficulty, bilateral arm weakness and neck weakness. Three weeks earlier, she was presented with clinical features corresponding to dengue and was symptomatically treated. Currently, hypotonia and decreased muscle strength were observed in both upper limbs and neck. Detailed investigation revealed the presence of Immunoglobulin M (IgM) antibodies against dengue antigen (NS 1) and Chikungunya virus (CHIKV), confirming the possibility of previous dengue-chikungunya co-infection. Nerve conduction studies and electromyography of upper limbs pointed towards findings consistent with the early stages of acute motor demyelinating and possible axonal neuropathy. The detection of antiganglioside antibodies (anti-GT1a antibodies), confirmed the diagnosis of the pharyngeal-cervical-brachial variant of GBS. A five days treatment of intravenous immunoglobulin (IVIG) along with physical rehabilitation was started which led to significant improvement and the patient was discharged after 15 days. PCB is an unfamiliar variant of GBS for many clinicians. Diagnosis can be made by a thorough history, clinical examination and investigations that can rule out other potential causes of cervicobrachial and oropharyngeal weakness. It also necessitates careful monitoring and followups after mono- and co-arboviral infections to prevent any debilitating neurological complications.

Guillain-Barré Syndrome-Like Polyneuropathy Associated with Immune Checkpoint Inhibitors: A Systematic Review of 33 Cases.

Immune checkpoint inhibitors (ICIs) have been increasingly used in the treatment of various types of tumors with favorable results. But these treatments also led to a variety of immune-related adverse events (irAEs). Neurological irAEs such as Guillain-Barré Syndrome are rare and may have serious consequences once they occur. A systematic literature search was performed in PubMed and Embase for all case reports of GBS associated with ICIs published in English reporting on human beings from 1990 up to date. A total of 30 case reports (total patients = 33) were used for final analysis. The included cases were from 11 countries, covering 10 tumor types, with melanoma accounting for the largest number. The mean age was 62.2 ± 11.1 years old, and males were dominant (male: 26 and female: 7). The median time of initial symptoms was 8.2 weeks after the 1st dose of ICIs. The most common manifestations of GBS associated with ICIs were weakness, hyporeflexia or areflexia, and paresthesia in order. The GBS subtypes suggested by electrophysiological results were acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and Miller Fisher syndrome (MFS). The protein level of CSF in patients with GBS related to ICIs was 180.68 ± 152.51 mg/dl. Immediate termination of ICIs followed by intravenous immunoglobulin was the preferred treatment option. 72.7% of patients recovered or had residual mild dysfunction after treatment. Elderly male patients with melanoma were most likely to develop ICI-related GBS. The specific neurological symptoms, CSF analysis, and electrophysiological examination were important means of diagnosis.

Electrophysiological characteristics of patients with nitrous oxide abuse.

Objective: In the young generations with nitrous oxide abuse (N2O), featured electrophysiological response of the peripheral neuropathy caused by nitrous oxide remains to be defined.Methods: Patients with nitrous oxide abuse (20 cases), two variants of Guillain-Barré syndrome (GBS), that is, acute inflammatory demyelinating polyradiculoneuropathy (GBS-AIDP, 19 cases) and acute motor axonal neuropathy (GBS-AMAN, 18 cases), as well as diabetic peripheral neuropathy (DPN, 20 cases) were enrolled into this study. Electrophysiological parameters including distal motor latency (DML), motor nerve conduction velocity (MNCV), sensory nerve conduction velocity (SNCV), amplitudes of compound muscle action potential (CMAP), and sensory nerve action potential (SNAP) were measured and analyzed by comparing the parameters between the aforementioned patients groups as well as normal control group (20 subjects).Results: Compared to normal control subjects, patients with nitrous oxide abuse showed prolonged DML, slower MNCV and SNCV in the limbs, lower amplitudes of CMAP in the median, tibial and peroneal nerves, and lower SNAP in median and ulnar nerves. Abnormalities of MNCV and amplitudes of CMAP in the lower limbs were significantly higher than that in the upper limbs . Abnormal electrophysiological features of patients with nitrous oxide abuse were dramatically different from those in GBS-AIDP or DPN patients, but similar to those in GBS-AMAN patients.Conclusions: Nitrous oxide abuse could cause abnormal electrophysiological response in the limbs. Some of the parameters (DML, MNCV, SNCV, CMAP and SNAP) appeared significantly different between the patients with nitrous oxide abuse, GBS with AIDP or AMAN, and DPN patients.Significance: Electrophysiological examination could be considered as an important supporting factor in differential diagnosis for nitrous oxide abuse, GBS with AIDP or AMAN, and DPN.

Risk factors for the severity of Guillain-Barré syndrome and predictors of short-term prognosis of severe Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is a neurological disorder characterized by paralysis. Identifying the severity, appropriate therapeutic method, and prognosis of GBS at an early stage is highly important. This study aimed to investigate the modifiable risk factors for the severity of GBS and consequent need for mechanical ventilation (MV) and to identify clinical predictive factors for poor short-term outcomes of severe GBS. 155 GBS patients who were admitted to the Affiliated Yantai Yuhuangding Hospital of Qingdao University during 2014-2020 were enrolled. Demographic, clinical, therapeutic and evolutionary data were collected and were then analyzed using univariate and multivariate regression analyses. Our analytic data demonstrated that the significant clinical predictors of severe GBS were recent history of surgery, older age, cranial nerve impairment, and elevated levels of liver enzymes (p < 0.05). Furthermore, autonomic dysfunction, lower Medical Research Council (MRC) score at nadir, and elevated levels of liver enzymes were significantly associated with MV for severe GBS (p < 0.05), and lower MRC score at nadir and autonomic dysfunction remained significant predictors of MV in severe GBS (p < 0.05). Lastly, recent history of surgery, lower MRC score at admission and at nadir, requirement for MV, and pneumonia during hospitalization were significantly associated with the short-term outcome of severe GBS and that lower MRC score at admission and need for MV were confirmed to be predictors of poor short-term prognosis (p < 0.05). Of note, this study suggested that recent history of surgery is a predictor of severity in GBS patients and is associated with the poor short-term prognosis of severe GBS.

Recurrent Miller-Fisher syndrome overlapping Guillain-Barrè syndrome and Bickerstaff brainstem encephalitis: A case report.

Miller-Fisher syndrome (MFS) together with Guillan-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) are considered to form a continuous clinical spectrum of the same disease, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. The frequency of overlapping clinical signs and the risk of recurrence are independent and very low, but no cases of GQ1b-seropositive recurrent MFS overlapping with GBS and BBE have been described so far. Here, we describe for the first time an atypical case of recurrent GQ1b-seropositive MFS overlapping GBS and BBE, 12 years after a previous GQ1b-seronegative typical MFS episode. Our case expands the clinical spectrum of recurrent MFS, and it should prompt clinicians to investigate the presence of anti-ganglioside antibodies in recurrent MFS even when these were negative in the previous episode, especially in those presenting with overlapping spectrum symptoms and a critically ill picture during the second episode.