Establishment of a single-center-based early prognostic scoring system for Guillain-Barré syndrome.

BACKGROUND: Previous studies have developed clinical prognostic models for Guillain-Barré syndrome including EGOS and mEGOS, they have good reliability and accuracy, but individual entries are poor. This study aims to establish a scoring system to predict the early prognosis, in order to provide additional treatment for patients with poor prognosis and shorten the length of hospital stay. METHODS: We retrospectively analyzed risk factors affecting the short-term prognosis of Guillain-Barré syndrome, and developed a scoring system for early determination of disease prognosis. Sixty two patients were divided into two groups based on the Hughes GBS disability score at discharge. Groups were compared for differences in gender, age at onset, antecedent infection, cranial nerve involvement, pulmonary infection, mechanical ventilation support, hyponatremia, hypoproteinemia, impaired fasting glucose, and peripheral blood neutrophil-to-lymphocyte ratio. Statistically significant factors were included in a multivariate logistic regression analysis, and a scoring system to predict the short-term prognosis was established based on the regression coefficients. The receiver operating characteristic curve of this scoring system was plotted, and the area under the ROC curve was calculated to assess the accuracy of the prediction model. RESULTS: Univariate analysis revealed that age at onset, antecedent infection, pneumonia, mechanical ventilation support, hypoalbuminemia, hyponatremia, impaired fasting glucose, and elevated peripheral blood neutrophil-to-lymphocyte ratio were risk factors for poor short-term prognosis. The above factors were included in the multivariate logistic regression analysis, and pneumonia, hypoalbuminemia, and hyponatremia could be used as independent predictors. The receiver operating characteristic curve was plotted with a calculated area under the ROC curve of 82.2% (95% CI 0.775-0.950, P < 0.0001). The best cut-off value for the model score was 2, with a sensitivity of 0.9091, a specificity of 0.7255, and a Youden index of 0.6346. CONCLUSION: Pneumonia, hyponatremia, and hypoalbuminemia were independent risk factors for poorer short-term prognosis in patients with Guillain-Barré syndrome. The short-term prognosis scoring system of Guillain-Barré syndrome we constructed using these variables had some predictive value, and the short-term prognosis with quantitative scores of 2 or more was worse.

Reference values for trans-laminar cribrosa pressure difference and its association with systemic biometric factors.

OBJECTIVES: To provide reference values of trans-laminar cribrosa pressure difference (TLCPD) and reveal the association of TLCPD with systemic biometric factors. METHODS: In this cross-sectional study, 526 quasi-healthy subjects (including 776 eyes) who required lumbar puncture for medical reasons were selected from 4915 neurology inpatients from 2019 to 2022. Patients with any diseases affecting intraocular pressure (IOP) or intracranial pressure (ICP) were excluded. The ICPs of all subjects were obtained by lumbar puncture in the left lateral decubitus position. IOP was measured in the seated position by a handheld iCare tonometer prior to lumbar puncture. TLCPD was calculated by subtracting ICP from IOP. Systemic biometric factors were assessed within 1 h prior to TLCPD measurement. RESULTS: The TLCPD (mean ± standard deviation) was 4.4 ± 3.6 mmHg, and the 95% reference interval (defined as the 2.5th-97.5th percentiles) of TLCPD was -2.27 to 11.94 mmHg. The 95% reference intervals for IOP and ICP were 10-21 and 6.25-15.44 mmHg, respectively. IOP was correlated with ICP (r = 0.126, p < 0.001). TLCPD was significantly negatively correlated with body mass index (r = -0.086, p = 0.049), whereas it was not associated with age, gender, height, weight, blood pressure, pulse, or waist and hip circumference. CONCLUSIONS: This study provides reference values of TLCPD and establishes clinically applicable reference intervals for normal TLCPD. Based on association analysis, TLCPD is higher in people with lower BMI.

Clinical Manifestation, Auxiliary Examination Features, and Prognosis of GFAP Autoimmunity: A Chinese Cohort Study.

OBJECTIVE: This paper reports the clinical manifestation and auxiliary examination features of 15 Chinese patients with glial fibrillary acidic protein (GFAP) autoimmunity. METHODS: From June 2016 to December 2019, patients suspected to have neurological autoimmune disease after having their serum and cerebrospinal fluid (CSF) tested for conventional neural antibodies were scanned for additional autoantibodies by immunohistochemistry. Samples that showed a characteristic immunoreactive pattern reminiscent of the GFAP of astrocytes were selected and confirmed by cell-based assay using cells-expressing human GFAPα. RESULTS: A total of 15 patients (eight male and seven female) with a median age at onset of 53 years (range 28-72) were identified as GFAP-IgG-positive. Fourteen cases had GFAP-IgG detected in the CSF, while serum GFAP-IgG was detected in 11 cases. Eleven of the fifteen patients (73.3%) presented with an acute monophasic course, of which 10 (90.9%) had antecedent flu-like symptoms. The predominant phenotype was meningoencephalitis (46.7%), followed by meningoencephalomyelitis in 40% of the cases. The most common clinical features included long tract signs, brainstem symptoms, tremors, headaches, and psychiatric symptoms. Magnetic resonance imaging (MRI) revealed the enhancement of the meninges, the surface of the brainstem, the cerebellum, and the spinal cord as predominant. Inflammatory CSF showed mild lymphocyte-predominant pleocytosis with a median of 51/µL and elevated protein with a median of 87.5 mg/dL. Five patients had coexisting antibodies, including NMDAR-IgG in three patients and Yo and MOG-IgG in one patient each. One patient underwent a stereotactic brain biopsy, and the neuropathology diagnosis was diffuse large B-cell lymphoma. One patient had ovarian teratoma. Eleven of the fifteen (73.3%) patients received both intravenous immunoglobulin and steroids. Among them, three patients also received immunosuppressive agents later. During a two-year follow-up, 9 of the 15 (60%) patients achieved complete clinical remission. CONCLUSIONS: The clinical presentation of GFAP astrocytopathy is heterogeneous. It can be characterized by an acute monophasic course and a chronic relapsing course. Tremors are a prominent clinical manifestation in patients with an acute monophasic course with GFAP-IgG antibodies only. Most patients responded well to immunotherapy. In patients with GFAP autoimmunity, presenting with a chronic relapsing course, one should actively search for immunogenic factors and the culprit antibodies. In the case of primary central nervous system lymphoma, GFAP autoimmunity does not always equate to autoimmune GFAP astrocytopathy.