Transient hypoglycorrhachia with paroxysmal abnormal eye movement in early infancy

Paroxysmal abnormal eye movement in early infancy is one of the initial symptoms of glucose transporter 1 deficiency syndrome (GLUT1DS). We describe four early infants with transient hypoglycorrhachia presenting with abnormal eye movements. Their symptoms disappeared after the introduction of a ketogenic diet (KD), and their development was normal. Since no variants in SLC2A1 were detected, the CSF-to-blood glucose ratios (C/B) were re-examined, and within normal range. None of the four patients displayed recurrent symptoms after withdrawal from the KD. Because long-term KD has potential adverse effects and could affect the quality of life of patients and their families, re-examination of CSF glucose during late infancy should be considered in the case of absence of the SLC2A1 pathogenic variant.     

ARCO Consensus on the Pathogenesis of Non-traumatic Osteonecrosis of the Femoral Head

Osteonecrosis of the femoral head (ONFH) is a devastating disease frequently leading to femoral head collapse and hip arthritis. Specifically, non-traumatic ONFH primarily affects young and middle-aged adults. Although compromised local circulation of the femoral head seems to be pathognomonic for the disease, the pathogenesis is perplexing and continues to be an area of scrutiny and research. Comprehension of the pathogenesis is of crucial importance for developing and guiding treatments for the disease. Therefore, we provide an up-to-date consensus on the pathogenesis of non-traumatic ONFH.     

Vascular Wall Imaging of Unruptured Cerebral Aneurysms with a Hybrid of Opposite-Contrast MR Angiography

BACKGROUND AND PURPOSE:Inflammation and degeneration of the intracranial saccular aneurysm wall play a major role in aneurysm formation, development and subsequent rupture. The aim of this study was to characterize the walls of unruptured intracranial aneurysms by using a hybrid of opposite-contrast MRA at 3T.MATERIALS AND METHODS:Fourteen consecutive patients with 17 unruptured intracranial aneurysms who initially underwent clipping surgery were prospectively evaluated. All aneurysms were scanned preoperatively by using a hybrid of opposite-contrast MRA in 3T high-resolution MR imaging. We classified intraoperative findings of atherosclerotic plaques in the aneurysms into 3 grades: grade A (major plaques), grade B (minor plaques), and grade C (no plaques). The contrast ratio of the high-intensity area was also measured relative to the background low-intensity area inside the carotid artery.RESULTS:Findings from preoperative plaque imaging of the aneurysm corresponded to the intraoperative findings in 15 of 16 aneurysms (excluding 1 that was impossible to visualize in its entirety due to anatomic reasons). Overall sensitivity and specificity of the hybrid of opposite-contrast MRA were 88.9% and 100%, respectively. During the operation, 4 aneurysms were classified as grade A; 5, as grade B; and 7, as grade C. The means of the contrast ratio for grades A, B, and C were 0.72 ± 0.03, 0.34 ± 0.30, and −0.02 ± 0.09, respectively.CONCLUSIONS:The hybrid of opposite-contrast MRA can detect visible atherosclerotic plaques in the unruptured aneurysm wall, and the contrast ratio in intracranial aneurysms correlated with their presence and extent. A study including a larger series is needed to validate the diagnostic potential of this imaging technique.

Intracranial aneurysms are common vascular lesions, often consisting of a saccular dilation of a cerebral artery vessel. The prevalence of intracranial aneurysms in the general population is estimated between 2.5% and 5%.^1,2 Aneurysmal rupture occurs with a 1% risk per year, depending on the size, location, and morphometry of the aneurysm, and leads to subarachnoid hemorrhage with associated high morbidity and mortality rates.^1,2 Intracranial aneurysms with an estimated high risk of rupture undergo management via a surgical or endovascular approach, depending on the specific risks of treatment.^3,4 Therefore, it is important to accurately assess the risk of aneurysmal rupture.The pathogenesis of intracranial aneurysms and their natural history are not well-understood. Histopathologic studies have shown that the infiltration of inflammatory cells and the degeneration of the aneurysm wall with atherosclerosis correlates with the formation, development, and rupture risk of cerebral aneurysms.^5–9 However, characterization of the aneurysm wall is limited by imaging data quality and the need to harvest surgical specimens.In this regard, the characteristics of high-field-strength MR imaging, which has a favorable SNR and changes in relaxation time and susceptibility, can depict the intracranial vessel walls and their pathologies, including small vessels with atherosclerosis.^10,11The hybrid of opposite-contrast MR angiography (HOP-MRA) used in this study is a modern technique that combines the advantages of 3D TOF MRA and flow-sensitive black-blood (FSBB) MRA.¹² The clinical efficacy of this technique was established to improve the visualization of peripheral vessels.^13,14 Theoretically, tissue with shorter T1 and T2* introduces high signal in FSBB of HOP-MRA, which demonstrates atherosclerotic plaques, including fat, as high-signal-intensity areas and demonstrates the blood space as low-signal-intensity areas in intracranial aneurysms.¹² The strength of this technique is the dual-echo 3D gradient-echo sequence, which enables a shorter imaging time and minimization of misregistration. The present study investigated the utility of HOP-MRA at 3T for the characterization of visible atherosclerotic plaques in intracranial aneurysms by using subtraction between TOF and FSBB imaging.     

Effect of ethanol on endothelin-1 release from gastric vasculature

The effects of ethanol on gastric vasculature in isolated vascularly perfused rabbit stomach was investigated. The isolated stomach was perfused with Krebs-Henseleit solution containing 3% dextran bubbled with 95% O₂ and 5% CO₂ at a rate of 12 ml/min. After mixture and perfusion of 10 mM to 400 mM of ethanol, perfusion pressure and endothelin-1 concentration in effluent from gastric vasculature were measured. Perfusion pressure and endothelin-1 concentration in effluent increased in a dose-dependent manner with increasing ethanol concentrations. In conclusion, the data suggest that ethanol may stimulate the release of endothelin from gastric vasculature and may cause gastric ischemia due to vasoconstriction resulting in acute gastric mucosal injury.     

The utility of three-dimensional computed tomography for prediction of tumor invasiveness in clinical stage IA lung adenocarcinoma

BackgroundIt is critical to have an accurate measurement of solid tumor size in order to predict the invasiveness of small lung adenocarcinomas. Some lesions cannot be measured accurately via High-resolution computed tomography (HRCT) due to their irregular shape and unclear borders. For this reason, we evaluated the relative efficacy of three-dimensional (3D) CT for predicting invasive adenocarcinoma.MethodsWe evaluated 195 patients with clinical stage IA adenocarcinomas, including 109 with lesions documented as invasive that were surgically resected at our institute during 2017. All lesions were categorized as either (I) lesions that were difficult to evaluate (i.e., hazy lesions; HL) or (II) more typical lesions (TL). The relationships between solid tumor size as determined by HRCT, solid tumor volume as determined by 3D CT and pathologic diagnosis were evaluated.ResultsFifty-seven patients (29%) were diagnosed with HL. We set the cut-off value for the solid volume at 225 mm³ as predictive for invasive adenocarcinoma. When evaluating all 195 patients as a group, the accuracy, sensitivity, and specificity based on the solid tumor volume were similar to those based on the solid tumor size. When we limit our analysis to the HL group, the specificity based on solid tumor volume (65.5%) was higher than that based on solid tumor size (44.8%) with a difference that approached statistical significance (P=0.070).Conclusions3D CT was equivalent to HRCT for predicting invasive adenocarcinoma and may be particularly useful for diagnosing lesions that are difficult to evaluate on HRCT.