Jiing-Feng Lirng

Objectives: The aim of this study was to investigate the functional networks in subjects with reversible cerebral vasoconstriction syndrome (RCVS) using resting-state functional magnetic resonance imaging (rs-fMRI).Methods: We prospectively recruited patients with RCVS and healthy controls (HCs) between February 2017 and April 2021. The rs-fMRI data were analyzed using graph theory methods. We compared node-based global and regional topological metrics (Bundle 1) and network-based intranetwork and internetwork connectivity (Bundle 2) between RCVS patients and HCs. We also explored the associations of clinical and vascular (ie, the Lindegaard index, LI) parameters with significant rs-fMRI metrics.Results: A total of 104 RCVS patients and 93 HCs were included in the final analysis. We identified significantly decreased local efficiency of the left dorsal anterior insula (dAI; p = 0.0005) in RCVS patients within 30 days after disease onset as compared to HCs, which improved 1 month later. RCVS patients also had increased global efficiency (p = 0.009) and decreased average degree centrality (p = 0.045), clustering coefficient (p = 0.033), and assortativity values (p = 0.003) in node-based analysis. In addition, patients with RCVS had increased internetwork connectivity of the default mode network (DMN) with the salience (p = 0.027) and dorsal attention (p = 0.016) networks. Significant correlations between LI and regional local efficiency in left dAI (r(s) = -0.418, p = 0.042) was demonstrated.Interpretation: The significantly lower local efficiency of the left dAI, suggestive of impaired central autonomic modulation, was negatively correlated with vasoconstriction severity, which is highly plausible for the pathogenesis of RCVS. ANN NEUROL 2023

Background/Aims: To avoid the negative impacts of the COVID-19 pandemic on clinical clerkship, supplemental teachings such as digital materials in the scenario-based distal simulations were implemented. This study utilized the OSCE (objective-structured clinical examination) to evaluate the impact of COVID-19 pandemic on the learning outcome of medical students from the regular group (class of 2020) and pandemic-impacted group (class of 2021). Methods: All medical students serially took, firstly, the mock-OSCE, secondly, the mock-OSCE, and the national OSCE. Then, the serial OSCE scores were compared between groups. Results: Although with similar scores in the first mock OSCE, the regular group (n = 78) had a higher average score in the national OSCE than the pandemic-impacted group (n = 80) (872.18 vs. 834.96, p = 0.003). In terms of improvement, the performances of the regular group were also better than the pandemic-impacted group between the second mock OSCE and the national OSCE (79.10 vs. 38.14, p = 0.014), and between the second mock OSCE and the national OSCE (125.11 vs. 77.52, p = 0.003). While separating distinct genres, the regular group had more of a score increment in standardized patient-based stations between the second mock OSCE and the national OSCE (regular vs. pandemic-impacted: 57.03 vs. 18.95, p = 0.003), as well as between the first mock OSCE and the national OSCE (75.97 vs. 26.36, p < 0.001), but there was no significant difference among the skill-based stations. In particular, the scores of the emergency medicine associated station in the national OSCE of the pandemic-impacted group was lower. Conclusions: Our study implies that the pandemic significantly hampered the learning outcomes of final year medical students in their clinical participation. Especially facing the COVID-19 pandemic, more supplemental teachings are needed to compensate the decreasing emergency medicine exposure.

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

Degenerative spinal deformity (DSD) has become a prevalent cause of disability and pain among the aging population worldwide. Though surgery has emerged as a promising option for DSD, the natural course, outcomes, and effects of surgery on DSD have remained elusive. This cohort study used a national database to comprehensively follow up patients of DSD for all-cause mortality, respiratory problems, and hip fracture-related hospitalizations. All patients were grouped into an operation or a non-operation group for comparison. An adjustment of demographics, comorbidities, and propensity-score matching was conducted to ameliorate confounders. A Cox regression hazard ratio (HR) model and Kaplan-Meier analysis were also applied. The study comprised 21,810 DSD patients, including 12,544 of the operation group and 9266 of the non-operation group. During the 14 years (total 109,591.2 person-years) of follow-up, the operation group had lower mortality (crude hazard ratio = 0.40), lower respiratory problems (cHR = 0.45), and lower hip fractures (cHR = 0.63) than the non-operation group (all p < 0.001). After adjustment, the risks for mortality and respiratory problems remained lower (adjusted HR = 0.60 and 0.65, both p < 0.001) in the operation than the non-operation group, while hip fractures were indifferent (aHR = 1.08, p > 0.05). Therefore, surgery for DSD is invaluable since it could reduce the risks of mortality and of hospitalization for respiratory problems.

Limited histopathology studies have suggested that reversible cerebral vasoconstriction syndromes (RCVS) does not present with vascular wall inflammation. Previous vascular imaging studies have had inconsistent vascular wall enhancement findings in RCVS patients. The aim of this study was to determine whether absence of arterial wall pathology on imaging is a universal finding in patients with RCVS. We recruited patients with RCVS from Taipei Veterans General Hospital prospectively from 2010 to 2012, with follow-up until 2017 (n = 48). We analyzed the characteristics of vascular wall enhancement in these patients without comparisons to a control group. All participants received vascular wall imaging by contrasted T1 fluid-attenuated inversion recovery with a 3-T magnetic resonance machine. The vascular wall enhancement was rated as marked, mild or absent. Of 48 patients with RCVS, 22 (45.8%) had vascular wall enhancement (5 marked and 17 mild). Demographics, clinical profiles, and cerebral artery flow velocities were similar across patients with versus without vascular wall enhancement, except that patients with vascular wall enhancement had fewer headache attacks than those without (p = 0.04). Follow-up imaging completed in 14 patients (median interval, 7 months) showed reduced enhancement in 9 patients, but persistent enhancement in 5. Almost half of our RCVS patients exhibited imaging enhancement of diseased vessels, and it was persistent for approximately a third of those patients with follow-up imaging. Both acute and persistent vascular wall enhancement may be unhelpful for differentiating RCVS from central nervous system vasculitis or subclinical atherosclerosis.

Granular cell tumors are rare neoplasms which can occur in any part of the body. Granular cell tumors of the orbit account for only 3 % of all granular cell tumor cases. Computed tomography and magnetic resonance imaging of the orbit have proven useful for diagnosing orbital tumors. However, the rarity of intraorbital granular cell tumors poses a significant diagnostic challenge for both clinicians and radiologists. We report a case of a 37-year-old Chinese woman with a rare intraocular granular cell tumor of her right eye presenting with diplopia, proptosis, and restriction of ocular movement. Preoperative orbital computed tomography and magnetic resonance imaging with contrast enhancement revealed an enhancing solid, ovoid, well-demarcated, retrobulbar nodule. In addition, magnetic resonance imaging features included an intraorbital tumor which was isointense relative to gray matter on T1-weighted imaging and hypointense on T2-weighted imaging. No diffusion restriction of water was noted on either axial diffusion-weighted images or apparent diffusion coefficient maps. Both computed tomography and magnetic resonance imaging features suggested an intraorbital hemangioma. However, postoperative pathology (together with immunohistochemistry) identified an intraorbital granular cell tumor. When intraorbital T2 hypointensity and free diffusion of water are observed on magnetic resonance imaging, a granular cell tumor should be included in the differential diagnosis of an intraocular tumor.

To report the development of estrapontine myelinolysis (EPM) in a patient with adrenal insufficiency and review similar in the literature. A 49-year-old female with insufficiency presented with acute dysarthria, stuttering, and parkinsonism. She received isotonic saline hydration for adrenal crisis and hyponatremia 18 days before the onset of symtoms. The brain MRI and MRS showed demyelination at bilateral basal ganglia and the thalamus, which was compatible with EPM and resolved within 3 months after steroid treatment. Development of acute parkinsonism after rapid correction of hyponatremia may indicate the occurrence of EPM and underlying adrenal insufficiency should be excluded in these patients.

Spinocerebellar ataxia (SCA) and multiple system atrophy-cerebellar type (MSA-C) often present with similar clinical manifestations in the beginning. Magnetic resonance spectroscopy (MRS) has been proved to be a useful tool to help differentiate different types of SCA and MSA-C on cross-sectional studies. However, longitudinal changes of the MRS metabolites in these subjects have never been reported. The purpose of this study was to track the longitudinal evolution of the MRS metabolites in these patients and to ascertain the correlation between clinical severity measured by Scale of the Assessment and Rating of Ataxia (SARA) and MRS metabolites. Significant reductions of NAA/Cr and NAA/Cho in the cerebellar hemispheres in all patients and lower Cho/Cr in the cerebellar hemispheres in patients with SCA2 or MSA-C were found at all times. At initial assessments, patients with MSA-C or SCA2 tended to have lower NAA/Cr and Cho/Cr in the cerebellar hemispheres than those with SCA3 or SCA6. At follow-ups, patients with SCA2 or MSA-C had a lower NAA/Cr in cerebellar hemispheres than those with SCA3 or SCA6. Patients with MSA-C had a lower NAA/Cr in the vermis and Cho/Cr in the cerebellar hemispheres than those with SCA2 at the start, and had a lower NAA/Cr in cerebellar hemispheres than those with SCA2 at follow-ups. Characteristic patterns of neurodegenerative evolution were observed in patients with disparate SCAs and MSA-C using MRS and SARA. A continual impairment of neuronal integrity was observed in all groups of patients. The longitudinal changes of MRS metabolites and SARA scores were most striking in patients with SCA2 and MSA-C. Although the changes in the metabolites on MRS may still be used to help understand the pathophysiology of ataxia disorders, they are short of being a good biomarker.