An anaplastic pleomorphic xanthoastrocytoma with periventricular extension: An autopsy case report and review of the literature.

Pleomorphic xanthoastrocytomas (PXAs) are rare low-grade astrocytic tumors that typically present as superficial nodular cystic tumors of the cerebrum attached to the leptomeninx. Histologically, they are pleomorphic, hypercellular glial neoplasms. Despite the presence of microscopic pleomorphism, patients' postoperative prognosis is generally good. Anaplastic PXAs (APXAs) have a high mitotic index and patients with APXAs have a worse prognosis than patients with PXAs. Here, we report an autopsy case of APXA initially diagnosed as PXA. After gross total resection, the tumor recurred and was diagnosed as an APXA; thereafter, the patient died. An autopsy revealed that the tumor had relapsed at the primary site and had spread to the leptomeningeal space while concurrently invading the cerebrum including the periventricular area forming multifocal lesions. The histological findings of the autopsy were similar to those for epithelioid glioblastoma (EGBM) and small cell glioblastoma (SCGBM). In particular, the periventricular area with multifocal lesions was composed of SCGBM-like cells. It has been shown that multifocal lesions are frequently identified in patients with SCGBM. This is the first histopathologically confirmed case of APXA-related tumor presenting with periventricular extension and multifocal lesion formation. The periventricular extension might be a feature of PXAs and APXAs. However, suspected periventricular spread on imaging in past cases of PXAs and APXAs might instead represent the malignant transformation of these tumors to glioblastoma-like high-grade tumors, which often show SCGBM-like histological patterns.

Ovarian function after allogeneic hematopoietic stem cell transplantation in children and young adults given 8-Gy total body irradiation-based reduced-toxicity myeloablative conditioning.

BACKGROUND: The spectrum of late sequelae after hematopoietic stem cell transplantation (HSCT) includes infertility, which is the most frequent complication. Some reports suggested that ovarian function may be better preserved in females undergoing HSCT with reduced-intensity conditioning (RIC) than with conventional myeloablative conditioning (MAC). However, the impact of HSCT after 8-Gy TBI-based reduced-toxicity MAC (RTMAC), whose efficacy is between those of conventional MAC and RIC, on ovarian function remains unclear. PROCEDURE: A single-center retrospective analysis of data derived from patient information for all the children who underwent transplantation at the Shinshu University Hospital was carried out. Patients who underwent 8-Gy total body irradiation (TBI)-based RTMAC before HSCT were analyzed. RESULTS: A total of 36% (five of 14) of the patients developed primary ovarian insufficiency (POI) during the observation period, but serum follicle-stimulating hormone levels reduced to normal range with spontaneous menstruation in two, implying the reversal of POI. Furthermore, only one (10%) of the 10 prepubertal patients (71%; 10/14) at the time of HSCT suffered from POI at the last observation, but all three post-pubertal patients developed POI (100%), and two (67%) continued to suffer from POI at the last observation. CONCLUSIONS: Taken together, 8-Gy TBI-based RTMAC before HSCT may decrease the possibility of POI compared with conventional MAC, especially in prepubertal patients. A longer follow-up will be required to ascertain whether a normal pregnancy and delivery can occur in such patients.

Self-Assembly of Metal-Organic Frameworks into Monolithic Materials with Highly Controlled Trimodal Pore Structures.

We present a two-step template-free approach toward monolithic materials with controlled trimodal porous structures with macro-, meso-, and micropores. Our method relies on two ordering processes in discrete length scales: 1) Spontaneous formation of macroporous structures in monolithic materials by the sol-gel process through the short-range ordered self-assembly of metal-organic frameworks (MOFs), and 2) reorganization of the framework structures in a mediator solution. The Zr-terephthalate-based MOF (UiO-66-NH2 ) was adopted as a proof of concept. The self-assembly-induced phase separation process offered interconnected macropores with diameters ranging from 0.9 to 1.8 µm. The subsequent reorganization process converted the microporous structure from low crystalline framework to crystalline UiO-66. The resultant mesopore size within the skeletons was controlled in the range from 9 to 21 nm. This approach provides a novel way of designing spaces from nano- to micrometer scale in network-forming materials.

Giant cavernous malformation in the ventrolateral midbrain with extension into the thalamus: a case report of a paramedian supracerebellar transtentorial approach.

Cavernous malformations (CMs) of the midbrain and thalamus are relatively rare and particularly difficult to be resected given their location in eloquent tissues. Here, we report a case of a 14-year-old boy who experienced repeated and progressive right hemiparesis. Image examinations showed a gradually enlarged CM originated in the left ventrolateral midbrain extending to the left thalamus with repeated hemorrhage. By performing a paramedian supracerebellar transtentorial approach, the CM was totally removed, and the patient recovered without any new neurological deficit. The authors' experience suggests that this approach is eminent in treating giant lesions involving the ventrolateral midbrain and thalamus.

Unusual flexibility of transparent poly(methylsilsesquioxane) aerogels by surfactant-induced mesoscopic fiber-like assembly.

High-performance thermal insulators represented by aerogels are regarded as one of the most promising materials for energy savings. However, significantly low mechanical strength has been a barrier for aerogels to be utilized in various social domains such as houses, buildings, and industrial plants. Here, we report a synthetic strategy to realize highly transparent aerogels with unusually high bending flexibility based on poly(methylsilsesquioxane) (PMSQ) network. We have constructed mesoscopic fine fiber-like structures of various sizes in PMSQ gels by the combination of phase separation suppression by tetramethylammonium hydroxide (TMAOH) and mesoscopic fiber-like assembly by nonionic poly(ethylene oxide)-b-poly(propylene oxide)-b-poly(ethylene oxide) (PEO-b-PPO-b-PEO) type surfactant. The optimized mesoscale structures of PMSQ gels have realized highly transparent and resilient monolithic aerogels with much high bendability compared to those reported in previous works. This work will provide a way to highly insulating materials with glasslike transparency and high mechanical flexibility.

Identification and characterization of a novel kaempferol sulfotransferase from Arabidopsis thaliana.

In plants, flavonoids have been shown to be subjected to conjugation modifications such as glycosylation, methylation, and sulfation. Among these modifications, sulfation is known as an important pathway in the regulation of the levels of endogenous compounds such as steroids. Although a large variety of flavonoid sulfates also exist in plants, the detailed biochemical characterization of Arabidopsis thaliana sulfotransferases (AtSULTs) remains to be fully clarified. We report here that uncharacterized AtSULT202E1 (AGI code: At2g03770), a SULT202E subfamily member, shows the sulfating activity toward flavonoids. The general characteristics of the enzyme were studied on the optimum temperature and pH, the effect of divalent cations, and the thermal stability with kaempferol as substrate. A comparative analysis of the sulfation of flavonoids by AtSULT202E1, AtSULT202B1 and AtSULT202A1 revealed that three AtSULTs have differential substrate specificities. Surprisingly, 3-hydroxyflavone was sulfated only by AtSULT202A1 while 7-hydroxyflavone was highly sulfated by AtSULT202E1 and AtSULT202B1. These results indicate that flavonols might be sulfated in a position specific manner. In conclusion, our studies indicate that a novel AtSULT202E1 has the sulfating activity toward flavonoids together with AtSULT202B1 and AtSULT202A1. The existence of three flavonoid sulfotransferases in A. thaliana suggests that sulfation of flavonoids have an important role in regulation of their functions.

Oxide-on-Oxide Porous Electrodes Revealing Superior Reversible Li+-Coupled Electron-Transfer Properties by Unconventional Heterojunction Effects.

Internal spacing of electrodes is a key point for controlling electron-transfer (ET)-related phenomena. However, their disordered porous structures often prevent the observation of microscopic effects. It hampers the development of modern electrochemical theories. The development of model porous electrodes therefore provides an ideal platform to discover intriguing fundamental principles of electrode processes. We developed a new synthetic strategy for all-oxide monolithic ruthenium dioxide (RuO2)/antimony-doped tin oxide (ATO) electrodes with a controlled hierarchically porous structure and oxide-oxide heterojunction. The use of the obtained RuO2/ATO electrodes as model electrodes suppressed influences related to different mass diffusion efficiencies between electrodes with heterojunctions of different types. Then, we showed unconventional oxide-oxide heterojunction effects, improving reversible Li+-coupled electron-transfer properties using model electrodes constituted of various nanostructured (nano-) RuO2 on porous ATO. In addition to the superior electrochemical properties of the nano-RuO2/ATO heterojunction, the quasi-two-dimensional (2D) RuO2/ATO heterojunction led to improved specific capacity at a high rate and longer cycle life. We anticipate that this oxide-oxide heterojunction effect and developed all-oxide model porous electrodes can provide a path to develop advanced reversible energy storage devices.

Hypercalcemic crisis caused by primary hyperparathyroidism in a 11-year-old boy: a rare case report and review of the literature.

Background: Hypercalcemic crisis caused by primary hyperparathyroidism (PHPT) in pediatric patients is very rare, and appropriate treatment approach for this condition has not been well demonstrated. Here, we report a case of PHPT-induced hypercalcemic crisis in a boy. Case Description: An 11-year-old boy visited the clinic with abdominal pain and nausea that lasted for 3 months, but the cause of his symptoms could not be identified. As these symptoms worsened after 1 month, he was referred to a nearby hospital. The boy's albumin-corrected serum calcium level was very high (14.3 mg/dL). Treatment was immediately started with the administration of normal saline, furosemide, and calcitonin to lower his serum calcium levels. Based on elevated intact-parathyroid hormone (i-PTH) (405 pg/mL) level and enlargement of the right superior parathyroid on diagnostic imaging, he was diagnosed with hypercalcemic crisis due to PHPT. As his albumin-corrected serum calcium level increased to 16.5 mg/dL and he could not take almost any foods due to severe nausea, he was transferred to our hospital and treated with pamidronate. Although his albumin-corrected serum calcium level decreased to 14.0 mg/dL, his symptoms did not improve completely. Therefore, 2 days after transfer to our hospital, he underwent emergency surgery to resect the enlarged right superior parathyroid gland. Fifteen minutes after removal of the enlarged parathyroid gland, the serum intact-PTH level decreased to 41.7 pg/mL. The histopathological diagnosis of the enlarged parathyroid gland was adenoma. The boy became asymptomatic, and his albumin-corrected serum calcium level was maintained within the normal limits for 6 months post operatively. Genetic testing performed after the surgery did not detect any pathogenic mutations in the MEN1 and CDC73 genes, and no genetic predisposition has been identified to date. Conclusions: Emergency focused parathyroidectomy prior to genetic testing might be an appropriate strategy when the pediatric patient presents with a PHPT-induced hypercalcemic crisis.

[Acute neck pain caused by calcium pyrophosphate dehydrate deposition in the transverse ligament of the atlas: a case report].

We report a case of pseudogout manifested by severe posterior neck pain. Pseudogout of the neck, also known as the crowned dens syndrome, causes acute neck pain characterized by calcium pyrophosphate dehydrate deposition around the odontoid process. Crowned dens syndrome is typified clinically by severe cervical pain and stiffness, often in conjunction with raised inflammatory markers. A 71-year-old man presented with severe neck pain. On admission, elevation of serum CRP level was confirmed. Magnetic resonance images showed no responsible abnormalities except for degenerating change of the spine. The patient was diagnosed as having pseudogout caused by calcium pyrophosphate dehydrate deposition based on cervical computed tomographic imaging, which showed linear calcification in the transverse ligament of the axis. After administration of non-steroidal anti-inflammatory drugs, the fever and neck pain disappeared and the CRP level returned to within the normal range. Pseudogout of the cervical spine should be considered as a differential diagnosis when we examine patients with acute neck pain. Cervical spinal computed tomographic scan is a more sensitive and useful examination method to diagnose this disease rather than magnetic resonance images.

Emergent electrochemical functions and future opportunities of hierarchically constructed metal-organic frameworks and covalent organic frameworks.

Designing spatial and architectural features across from the molecular to bulk scale is one of the most important topics in materials science which has received a lot of attention in recent years. Looking back to the past research, findings on the influences of spatial features denoted as porous structures on the applications related to mass transport phenomena have been widely studied in traditional inorganic materials, such as ceramics over the past two decades. However, due to the difficulties in precise control of the porous structures at the molecular level in this class of materials, the mechanistic understanding of the effects of spatial and architectural features across from the molecular level to meso-/macroscopic scale is still lacking, especially in electrochemical reactions. Further understanding of fundamental electrochemical functions in well-defined architectures is indispensable for the further advancement of key next-generation energy devices. Furthermore, creating periodic porosity in reticular structures is starting to be recognized as an emerging approach to control the electronic structure of materials. In this review, we focus on the investigations on preparing well-defined molecular-level crystalline porous materials known as metal-organic frameworks (MOFs) and covalent organic frameworks (COFs) into hierarchically constructed architectures from molecular structures lower than the reticular frameworks to meso-/macroscopic scale structures. By connecting well-defined nanosized porous structures in MOFs/COFs and additional length-scale space or shapes, emergent electrochemical functions towards emerging devices, such as beyond Li-ion batteries including all-solid-state rechargeable batteries, are expected to be obtained. By summarizing recent advancements in synthetic strategies of hierarchically constructed MOF/COF based materials and fundamental investigation of their structural effect in a wide spectrum of electrochemical applications, we highlight the importance and future direction of this developing field of hierarchically constructed MOFs/COFs, while emphasizing the required chemical stability of the MOFs/COFs which meet the use in the game-changing electrochemical devices.

Underwater Endoscopic Ear Surgery for Closure of Cholesteatomatous Labyrinthine Fistula With Preservation of Auditory Function.

OBJECTIVE: To analyze the outcomes of the underwater endoscopic ear surgery (UWEES) technique for closure of cholesteatomatous labyrinthine fistula (LF) with preservation of auditory function. STUDY DESIGN: Retrospective case review study. SETTING: Tertiary referral center. PATIENTS: A total of 12 patients with cholesteatomatous LF. INTERVENTION: Surgical method of closure using UWEES for cholesteatomatous LF to minimize inner ear damage. Artificial cerebrospinal fluid (CSF) was used as the perfusate, except for earlier cases when saline was employed. MAIN OUTCOME MEASURES: Comparison of bone conductance hearing level (BCHL) before and after surgery. A change of BCHL less than 10 dB was defined as successful preservation of bone conductance hearing. RESULTS: All cases of LF were treated successfully by closure using the UWEES technique. Seven cases were type I, one was type IIa, and four were type III according to the Milewski and Dornhoffer classification of LF. The average LF size was 3.1 mm (1-7 mm). Eleven patients were evaluated and their bone conductance hearing was well preserved in all of them (11/11). One patient was too young for preoperative evaluation of BCHL, but hearing preservation was verified 2 years later at the age of 6 years. Remarkably, none of the patients complained of vertigo, except for only a slight manifestation on postoperative day 1. CONCLUSION: The UWEES technique was effective for closure of cholesteatomatous LF with preservation of auditory function.

Intraoperative Lumbar Muscle Motor Evoked Potential Monitoring With Transcortical Stimulation.

BACKGROUND: Stimulating electrodes for lower extremity motor-evoked potential (LE-MEP) monitoring with transcortical stimulation are usually placed on the medial side of motor cortex convexity, which is not lower extremity but lumbar motor area. Lumbar MEP may be elicited with lower stimulation intensity than LE-MEP through this location, and it is useful to monitor lower extremity motor function intraoperatively. METHODS: Intraoperative lumbar and LE-MEP monitoring with transcortical stimulation during surgery of 12 patients with lesions involving the motor cortex from January 2012 to February 2019 at Shinshu University Hospital were reviewed retrospectively. Stimulations were delivered by a train of 5 pulses of anodal constant current stimulation. Stimulating electrode position was determined by motor cortex mapping. Recording needle electrodes were placed on bilateral lumbar muscles and contralateral leg muscles. The threshold-level stimulation method was used for MEP monitoring. The thresholds, monitoring result, and postoperative motor function of lumbar and lower extremities were compared. RESULTS: The mean baseline thresholds were 19.9 ± 8.9 mA for lumbar MEP and 26.5 ± 11.5 mA for LE-MEP (P = 0.02). Patterns of intraoperative monitoring changes were the same between lumbar and LE-MEP monitoring. CONCLUSIONS: Lumbar MEP was stimulated with lower stimulation intensity than the LE-MEP with the same intraoperative pattern of waveform changes in 12 patients. Lumbar MEP monitoring may be useful for preserving the corticospinal tract of lower extremities intraoperatively.

Microscopic Navigation-Guided Fence Post Technique for Maximal Tumor Resection During Glioma Surgery.

BACKGROUND: The fence post technique, which involves insertion of catheters as fence posts around a tumor, has been widely used to demarcate the tumor border for maximal resection of intraparenchymal tumors, such as gliomas. However, a standard procedure for fence post insertion has not been established, and there are some limitations. To overcome this problem, a simple microscopic navigation-guided fence post technique was developed. The feasibility and efficacy of this novel technique during glioma surgery were assessed. METHODS: The microscopic navigation-guided fence post technique was used in 46 glioma surgeries performed in 42 patients. Intraoperatively, the preplanned trajectory was overlaid on the microscopic surgical field, and the microscope angle was changed until the entry and target points of the trajectory overlapped. A fence post catheter was inserted as planned under microscopic view, and the tumor was resected with fence post guidance. Preoperative tumor characteristics and surgical outcomes were evaluated. RESULTS: Mean age of patients was 50 years (range, 16-78 years), and 19 (45%) of 42 patients were women. Maximal safe resection was successfully achieved in 45 surgeries (97.8%), which was planned preoperatively with identification of the tumor border with fence posts without adverse effects of brain shift. No surgical complications attributable to fence post insertion occurred. CONCLUSIONS: Clinical experience indicated that the microscopic navigation-guided fence post technique, in which fence posts can be placed without requiring the surgeon to take their eyes off the microscope, is safe and useful in glioma surgery.

Multiscale structural control of linked metal-organic polyhedra gel by aging-induced linkage-reorganization.

Assembly of permanently porous metal-organic polyhedra/cages (MOPs) with bifunctional linkers leads to soft supramolecular networks featuring both porosity and processability. However, the amorphous nature of such soft materials complicates their characterization and thus limits rational structural control. Here we demonstrate that aging is an effective strategy to control the hierarchical network of supramolecular gels, which are assembled from organic ligands as linkers and MOPs as junctions. Normally, the initial gel formation by rapid gelation leads to a kinetically trapped structure with low controllability. Through a controlled post-synthetic aging process, we show that it is possible to tune the network of the linked MOP gel over multiple length scales. This process allows control on the molecular-scale rearrangement of interlinking MOPs, mesoscale fusion of colloidal particles and macroscale densification of the whole colloidal network. In this work we elucidate the relationships between the gel properties, such as porosity and rheology, and their hierarchical structures, which suggest that porosity measurement of the dried gels can be used as a powerful tool to characterize the microscale structural transition of their corresponding gels. This aging strategy can be applied in other supramolecular polymer systems particularly containing kinetically controlled structures and shows an opportunity to engineer the structure and the permanent porosity of amorphous materials for further applications.

Investigation of TSH receptor blocking antibodies in childhood-onset atrophic autoimmune thyroiditis.

Atrophic autoimmune thyroiditis (AAT) is a type of autoimmune hypothyroidism without goiter. TSH receptor-blocking antibodies (TSBAb) are involved in its etiology in adults. Reportedly, this disease is extremely rare in children. In this study, we aimed to investigate the prevalence of TSBAb during AAT onset in children using a commercially available cell-based bioassay TSAb kit. We conducted a multicenter retrospective observational study. We collected data of patients with AAT who were < 15 yr old, enrolled in a collaborative research group, and diagnosed since July 2003. AAT was defined as acquired autoimmune hypothyroidism without thyroid enlargement. Eighteen patients (including 15 females) whose TSH receptor antibody (TRAb) or TSBAb levels were measured within a year from the initial visit were included. The median age at diagnosis was 9.3 years, and the estimated time between onset and diagnosis was 2.6 yr. The positive rate for either TSBAb or TRAb was 38.8% (95% confidence interval: 18.3-59.5%). There were no significant differences in age, the estimated time between onset and diagnosis, and FT4 levels at diagnosis between the TSBAb-positive and -negative groups. Unlike previous reports, we showed that the prevalence of TSBAb-positivity in childhood-onset AATs is not rare, as in adults.

Transient Retinal Ischemia During Carotid Endarterectomy Estimated by Intraoperative Visual Evoked Potential Monitoring: Technical Note.

OBJECTIVE: Carotid revascularization surgery is known to carry a risk of postoperative visual deterioration associated with retinal ischemia. We checked intraoperative visual evoked potential (VEP) monitoring in carotid endarterectomy (CEA). METHODS: Ten consecutive patients who underwent CEA in Shinshu University Hospital under total intravenous anesthesia were checked by intraoperative VEP and electroretinogram (ERG) recording in addition to somatosensory evoked potential monitoring. RESULTS: Two of 10 patients presented decreased amplitude of VEP and ERG on the ipsilateral affected side by clamping the common carotid artery and persistent attenuation of VEP and ERG during external carotid artery occlusion, using an internal carotid shunt. These findings disappeared immediately after releasing the cervical carotid artery clamping. In the other 8 patients, VEP and ERG did not change throughout the surgery. CONCLUSIONS: Transient retinal ischemia during even brief carotid artery occlusion in the CEA procedure could be estimated by intraoperative VEP and ERG monitoring.

Staple electrodes: an innovative alternative for intraoperative electrophysiological monitoring.

Intraoperative electrophysiological monitoring is essential for minimally invasive neurosurgery. The authors developed an innovative recording method using a staple electrode, consisting of a surgical skin staple and an integrated circuit (IC) test clip with a cable. The staple is put on the patient's skin after the induction of general anesthesia. After head fixation, the IC test clip is simply hooked to the staple. The authors used this method for recording in 158 consecutive cases. It took only a few minutes to set up 4-18 staple electrodes in each case. None of the staple electrodes became disconnected unintentionally, and the initial impedance was kept throughout the procedures. The authors conclude that the staple electrode is superior to conventional disc or needle electrodes in speed of setup, electrical stability, and cost-effectiveness and recommend its routine use for intraoperative electrophysiological monitoring.

Real-Time Navigation-Guided Drilling Technique for Skull Base Surgery in the Middle and Posterior Fossae.

Objective The usefulness of the bony surface registration method for navigation system image-guided surgery in the lateral or prone position has been reported. This study was performed to evaluate the efficacy of our new real-time navigation-guided drilling technique with bony surface registration for skull base surgery in the middle and posterior fossae. Methods The study included 29 surgeries for skull base tumors that required drilling of the petrous bone between January 2015 and December 2017 in Shinshu University Hospital. A navigation system was used for drilling of the petrous bone as follows: (1) some labyrinthine structures were marked by color in the source image and superimposed on the navigation image on the workstation preoperatively; (2) bony surface registration was performed with a three-dimensional (3D) skull reconstruction model in the operating room; (3) the petrous bone was drilled under navigation guidance with real-time view-through confirmation of 3D color-marked labyrinthine structures with observation under a microscopic operative view. Results Real-time identification of some structures in the petrous bone was performed, and adequate and precise drilling of the petrous bone was achieved without the risk of labyrinthine perforation or stress. Using this method, surgeons do not need to alternate their gaze between the surgical field and the navigation screen. Conclusions Due to the development of bony surface registration, this new technique is useful for drilling petrous bone in the middle and posterior fossa skull base surgeries.

An infant case of diffuse cerebrospinal lesions and cardiomyopathy caused by a BOLA3 mutation.

INTRODUCTION: Mitochondrial dysfunction results in a wide range of organ disorders through diverse genetic abnormalities. We herein present the detailed clinical course of an infant admitted for extensive, rapidly progressing white matter lesions and hypertrophic cardiomyopathy due to a BOLA3 gene mutation. CASE: A 6-month-old girl with no remarkable family or past medical history until 1 month prior presented with developmental regression and feeding impairment. Ultrasound cardiography and brain magnetic resonance imaging (MRI) respectively disclosed the presence of hypertrophic cardiomyopathy and symmetrical deep white matter lesions. She was transferred to our hospital at age 6 months. High lactate levels in her cerebrospinal fluid suggested mitochondrial dysfunction. Despite vitamin supplementation therapy followed by a ketogenic diet, the patient began exhibiting clusters of myoclonic seizures and respiratory failure. Brain and spinal cord MRI revealed rapid progression of the white matter lesions. She died at 10 months of age. Fibroblasts obtained pre-mortem displayed low mitochondrial respiratory chain complex I and II activity. A homozygous H96R (c. 287 A > G) mutation was identified in the BOLA3 gene. DISCUSSION: No reported case of a homozygous BOLA3 gene mutation has survived past 1 year of life. BOLA3 appears to play a critical role in the electron transport system and production of iron-sulfur clusters that are related to lipid metabolism and enzyme biosynthesis.