Systemic inflammation caused by an intracranial mesenchymal tumor with a EWSR1::CREM fusion presenting associated with IL-6/STAT3 signaling.

Pediatric neoplastic diseases account for about 10% of cases of fever of unknown origin (FUO), and most neoplastic disease cases are leukemia, lymphoma, and neuroblastoma. Brain tumors are rarely reported as the cause of FUO, although craniopharyngioma, metastatic brain tumor, and Castleman's disease have been reported. We report a case of intracranial mesenchymal tumor (IMT) with a FET:CREB fusion gene, which had inflammatory phenotype without neurological signs. A 10-year-old girl was admitted with a 2-month history of intermittent fever and headache, whereas her past history as well as her family history lacked special events. Sepsis work-up showed no pathological organism, and empirical antibiotic therapy was not effective. Bone marrow examination showed a negative result. Cerebrospinal fluid examination showed elevated protein as well as cell counts, and head magnaetic resonance imaging showed a hypervascular mass lesion with contrast enhancement in the left cerebellar hemisphere. The patient underwent tumor excision, which made the intermittent fever disappear. Pathological examinations resembled those of classic angiomatoid fibrous histiocytoma (AFH), but the morphological features were distinct from the AFH myxoid variant; then we performed break-apart fluorescence in situ hybridization and confirmed the tumor harbored the rare EWSR1::CREM fusion gene (Ewing sarcoma breakpoint region 1 gene (EWSR1) and cAMP response element binding (CREB) family gene). Consequently, we diagnosed the condition as IMT with EWSR1::CREM fusion. Elevated serum concentration of interleukin 6 (IL-6) was normalized after tumor resection, which suggested the fever could be caused by tumor-derived IL-6. This is the first case of IMT with EWSR1::CREM fusion that showed paraneoplastic symptoms associated with the IL-6/signal transducer and activator of transcription 3 (STAT3) signaling pathway. Although brain tumors are rarely diagnosed as a responsible disease for FUO, they should be considered as a cause of unknown fever even in the absence of abnormal neurological findings.

A Ruptured Aneurysm of the Transdural Anastomotic Artery Occurring at the Temporal Base in Moyamoya Disease: Case Report and Literature Review.

A 24-year-old woman with hemorrhagic onset moyamoya disease received bilateral indirect bypass surgery 11 years ago. She presented with a life-threatening atraumatic acute right subdural hematoma with temporal lobe intracerebral hemorrhage due to a transdural anastomosis (TDA) aneurysm rupture. We reviewed six cases of rare TDA aneurysms, all of which occurred around the temporal base. TDA aneurysms may occur near the main trunk of the middle meningeal artery (MMA) where hemodynamic stress is high. Therefore, we must pay attention to skull base aneurysms that form near the MMA; otherwise, the prognosis would be poor if such an aneurysm ruptured.

Leakage sign is a reliable predictor of hematoma expansion in acute epidural hematoma.

BACKGROUND: Hematoma expansion in small/medium-sized acute epidural hematoma (AEDH) cases upon emergency admission is critical. Predicting hematoma expansion can lead to early surgical interventions, improving outcomes and eliminating the need to check for expansion via computed tomography (CT). This study aimed to identify the most reliable predictors of AEDH expansion. METHODS: We retrospectively collected data from patients with pure AEDH not requiring surgical treatment upon emergency admission from 2012-2022. We assessed clinical and laboratory data, time from injury to the first CT, and time to follow-up CT. Factors predictive of hematoma expansion on the second follow-up CT, including the leakage sign (LS), were analyzed. RESULTS: A total of 23 patients with pure AEDH without surgery at admission were included, and LS was positive in 18. Thirteen patients showed hematoma expansion. The hematoma expansion group showed a significantly higher rate of positive LS and lower mean platelet count than the group without hematoma expansion. LS's predictive value for AEDH expansion showed 100% sensitivity and 50% specificity. All patients with negative LS and normal platelet counts showed no hematoma expansion. Analyzing the time from injury to the first CT suggested that LS (+) within 120 min strongly predicted hematoma expansion. Reconstructed three-dimensional images of the leakage point on the skull revealed multiple mottled bleeding points on the dural surface. CONCLUSION: LS can predict hematoma expansion in patients with pure AEDH for whom emergency surgery is unnecessary at admission. The time from injury and platelet counts must also be considered.

Brain Hypothermia Therapy and Targeted Temperature Management for Acute Encephalopathy in Children: Status and Prospects.

In adult intensive care, brain hypothermia therapy (BHT) was reported to be effective in neuroprotection after resuscitation and cardiac arrest. By contrast, in neonatal intensive care, the pathophysiology of brain damage caused by hypoxic-ischemic encephalopathy (HIE) is attributed to circulatory disturbances resulting from ischemia/reperfusion, for which neonatal brain cryotherapy is used. The International Liaison Committee on Resuscitation, 2010, recommends cerebral cryotherapy for HIE associated with severe neonatal pseudoparenchyma death. The usefulness of BHT for neuroprotection in infants and children, especially in pediatric acute encephalopathy, is expected. Theoretically, BHT could be useful in basic medical science and animal experiments. However, there are limitations in clinical planning for treating pediatric acute encephalopathy. No international collaborative study has been conducted, and no clinical evidence exists for neuroprotection using BHT. In this review, we will discuss the pathogenesis of neuronal damage in hypoxic and hypoperfused brains; the history of BHT, its effects, and mechanisms of action; the success of BHT; cooling and monitoring methods of BHT; adverse reactions to BHT; literature on BHT. We will review the latest literature on targeted temperature management, which is used for maintaining and controlling body temperature in adults in intensive care. Finally, we will discuss the development of BHT and targeted temperature management as treatments for pediatric acute encephalopathy.

Preoperative Novel Magnetic Resonance Fusion Imaging for Carotid Endarterectomy Patients with Contrast Contraindications: A Pilot Study.

OBJECTIVE: For carotid endarterectomy (CEA) patients with renal dysfunction and allergies to contrast media, we developed a preoperative novel method of noncontrast 3-dimensional magnetic resonance fusion imaging (NC-3DMRFI) which could describe well blood vessels, plaques, and bony structures even in surgical position. In this study, we examined the usefulness of this method. METHODS: We extracted noncontrast magnetic resonance images of bones, blood vessels, and plaques to create a 3-dimensionalusion image. An image acquired in the normal position and another in the surgical position during CEA were used to create a fusion image. We compared the fusion imaging results with the intraoperative findings of 6 patients with contrast contraindications received CEA. RESULTS: Preoperative NC-3DMRFI could clearly show the positions of the carotid bifurcation, the distal end of plaque, and the bony structure in 5 of the 6 cases. Intraoperative findings and preoperative fusion imaging results were comparable in all cases where fusion images could be created. The fusion imaging in the surgical position during CEA was useful for preoperative examination, and the surgical space could be secured in the case of a high cervical location. CONCLUSIONS: This pilot study showed our novel NC-3DMRFI method is useful for preoperative simulation in CEA, especially in patients with renal dysfunction and allergies to contrast media.

Visual Outcomes and Surgical Approach Selection Focusing on Active Optic Canal Decompression and Maximum Safe Resection for Suprasellar Meningiomas.

The goal of treating patients with suprasellar meningioma is improving or preserving visual function while achieving long-term tumor control. We retrospectively examined patient and tumor characteristics and surgical and visual outcomes in 30 patients with a suprasellar meningioma who underwent resection via an endoscopic endonasal (15 patients), sub-frontal (8 patients), or anterior interhemispheric (7 patients) approach. Approach selection was based on the presence of optic canal invasion, vascular encasement, and tumor extension. Optic canal decompression and exploration were performed as key surgical procedures. Simpson grade 1 to 3 resection was achieved in 80% of cases. Among the 26 patients with pre-existing visual dysfunction, vision at discharge improved in 18 patients (69.2%), remained unchanged in six (23.1%), and deteriorated in two (7.7%). Further gradual visual recovery and/or maintenance of useful vision were also observed during follow-up. We propose an algorithm for selecting the appropriate surgical approach to a suprasellar meningioma based on preoperative radiologic tumor characteristics. The algorithm focuses on effective optic canal decompression and maximum safe resection, possibly contributing to favorable visual outcomes.

Tiger attack at a Japanese safari park: a case report.

BACKGROUND: Big cat bites are highly lethal due to the enormous bite force of these animals. This article reviews the morphology of these types of injuries and key points of management through a survival case at a Japanese safari park. CASE PRESENTATION: We report the case of a 26-year-old female keeper who was attacked by a tiger. She was quickly transported to our university hospital by ambulance helicopter. The keeper was severely bitten on the head and face and had wounds all over her body. Craniofacial repair was performed by emergency surgery. She suffered mild facial nerve paralysis and trismus because of being bitten by the tiger and is currently recovering. CONCLUSIONS: A multidisciplinary approach of the severe tiger bites successfully treated a young woman cosmetically and mentally. Animal farms and zoos that keep tigers should take strict measures to avoid direct confrontation with tigers.

Neurosurgical Treatment of Patients with Posterior Fossa Acute Subdural Hematoma Right after Cardiac Surgery.

As posterior fossa acute subdural hematoma (ASDH) right after cardiac surgery is extremely rare, the clinical course and optimal treatment strategy remain undetermined. We performed a retrospective analysis of patients with posterior fossa ASDH right after cardiac surgery requiring neurosurgical treatment at our institution over a 7-year period and, in this study, discussed the neurosurgical strategy and clinical course. Collected data included clinical history, laboratory results, time course, symptoms, neurosurgical treatment, outcome at discharge, and imaging studies. All six patients were women who had no history of head trauma and had received antithrombotic therapy during the perioperative period of cardiac surgery. All patients showed lower platelets count and were diagnosed with ASDH within 3 days (longest time 64 h) right after cardiac surgery. After discontinuation of anticoagulation therapy and administration of reversal agents, they underwent emergency hematoma evacuation craniotomy (n = 5) or burr hole drainage surgery (n = 1), which were performed in the prone (n = 4) or lateral (n = 2) positions. Four of these patients showed favorable outcomes, and two showed poor outcomes. One of the poor-outcome patients received three antithrombotic therapies, and another developed rapidly progressive ASDH. Posterior fossa ASDH associated with antithrombotic therapy right after cardiac surgery is frequently found in women, and emergent neurosurgical treatment with anticoagulation discontinuation and reversal agent administration can be performed safely. Burr hole drainage surgery might be acceptable in nonsevere cases. By contrast, we must pay attention to cases receiving both anticoagulant and antiplatelet drugs and rapid progression cases.

A case of mid-thoracic osteoporotic vertebral fracture with the inability to belch syndrome.

Background: Osteoporotic vertebral fractures (OVF) commonly occur at the thoracolumbar junction, but are less frequently encountered in the mid-thoracic region. Here, a 69-year-old female presented with back pain and the new onset of symptoms characterized by the inability to belch. Case Description: A 69-year-old female presented with back pain. 2 months later, she developed anorexia and difficulty belching. The thoracic magnetic resonance (MR) demonstrated a T7 OVF. As she ultimately underwent a balloon kyphoplasty (BKP), as conservative treatment was unsuccessful. Conclusion: OVF should be suspected in elderly females with the inability to belch accompanied by chest and back pain. The diagnosis is best established with a spinal MR imaging and should be followed by BKP.

Cerebrospinal fluid shunt for normal pressure hydrocephalus patient exacerbates cord symptoms due to spinal tumor.

Background: Normal-pressure hydrocephalus (NPH) and spinal intradural extramedullary benign tumors rarely exist together. Here, a 72-year-old female who presented with NPH symptoms (i.e., gait disturbance and dementia) newly developed symptoms of spinal cord compression attributed to a previously undiagnosed schwannoma. Case Description: A 72-year-old female was diagnosed with NPH without disproportionately enlarged subarachnoid space hydrocephalus. The lumbar puncture revealed an elevated cerebrospinal fluid (CSF) protein level of 0.141 g/dl, but with normal pressure. The patient's NPH symptoms improved after lumbar-peritoneal shunt placement. However, a year later, she subacutely developed a progressive Brown-Sequard syndrome. On the cervical magnetic resonance (MR), an intradural extramedullary lesion was found at the C5-C6 level which at surgery, proved to be a schwannoma. A review of this patient and three others with NPH and intradural extramedullary benign tumors revealed that 4.3 months following CSF shunting for NPH, they developed rapidly progressive cord deficits, attributed to their benign spinal tumors. Conclusion: Before the placement of shunts for NPH, patients should undergo holospinal MR imaging studies to rule out attendant spinal intradural extramedullary tumors.

Characteristics of patients who received helicopter emergency medical services in Japan from 2012 to 2019: a retrospective analysis of data from Tochigi Prefecture.

BACKGROUND: Helicopter Emergency Medical Services (HEMS) has been in operation in Japan since 2001, allowing patients almost anywhere in the nation to receive on-scene emergency treatment from physicians. However, there is insufficient literature on the characteristics of the patients who use Japanese HEMS. Thus, this study aimed to investigate the overall characteristics of patients receiving HEMS care within a single prefecture in Japan. METHODS: We retrospectively analyzed the data of 5163 patients-in Tochigi Prefecture-who received HEMS care from 2012 to 2019. Descriptive statistics were used to analyze the following aspects of care: diagnosis, severity, background characteristics, geographical and environmental variables, immediate pre-hospital intervention, transportation type, and short-term clinical outcomes. RESULTS: Among 7370 HEMS requests received during the study period, treatment was provided to 5163 patients (1.8 cases per day; 3489 men [67.6%]). Nearly 55% (n = 2856) of patients were aged above 60 years. Age peaks were observed at 0-9 years and 60-69 years. The median distance from the base hospital to the site was 26.7 km. The age-standardized rate of HEMS treatment was 30.3 patients per 100,000 people. Cases of trauma and cardiovascular diseases were the most common (65.3%). Most individuals aged 0-9 years and 60-69 years had neurological disease (seizures accounted for 80.5% of this group) and cardiovascular disease, respectively. The number of patients was similar across all four seasons. After immediate pre-hospital intervention, 81.6% of patients receiving HEMS care were transferred by the helicopter ambulance (53.4% and 28.2% to the base hospital and to other hospitals, respectively). Overall, 56.6% of patients receiving HEMS care were transferred to the base hospital, and the short-term recovery rate was above 75%. Intravenous drip and oxygen administration were the most common pre-hospital interventions (93.1% and 72.7%, respectively). CONCLUSIONS: This study is the first to describe the overall characteristics of HEMS patients using comprehensive data of all HEMS patients in one prefecture in Japan. Further research using both local- and national-level data is needed to accelerate the understanding of the benefits of HEMS.

Primary Pituitary Adenoid Cystic Carcinoma: A Rare Salivary Gland-Like Tumor in the Sella.

Adenoid cystic carcinoma (ACC) is a tumor of the exocrine glands that originates primarily from the minor and major salivary glands, nasopharynx, and lacrimal glands. ACC grows slowly but is locally aggressive and prone to recurrence. It is uncommon for ACCs to develop in the pituitary gland as a primary tumor. We present a case of primary pituitary ACC extending to the sphenoid sinus resembling an invasive adenoma in a 71-year-old woman with a history of nasal epistaxis. We reviewed other reported cases of pituitary ACCs with retrospective validation of whether the tumor was primary or not. The intrasellar tumor exhibited MYB rearrangement with enlargement and destruction of the sella turcica and dural tears toward the sphenoid sinus, which would be consistent for a tumor originating from the pituitary gland. Including our case, only four intrasellar and one suprasellar ACC have been confirmed as primary tumors. All intrasellar ACCs had the characteristic of some form of invasion of neighboring structures with evidence of hyperprolactinemia. ACC could develop in the pituitary gland as a form of salivary gland-like tumor derived from the ectopic salivary gland rests. ACCs rarely arise from the pituitary gland; however, the accurate determination of primary occurrence is difficult because of the invasive nature of these tumors, and the anatomical relationship with the sphenoid sinus and the cavernous sinus. Excessive bleeding from the tumor or unexpected internal carotid artery injury should be avoided during surgery for primary and secondary pituitary ACC.

Transmission of toxigenic Corynebacterium ulcerans infection with airway obstruction from cats to a human.

BACKGROUND: Infections caused by Corynebacterium ulcerans, a zoonotic pathogen, have been reported worldwide. This microorganism is known to produce the diphtheria toxin and cause diphtheria-like illness. CASE PRESENTATION: A 63-year-old woman with a history of diabetes and hypertension developed cold and flu-like symptoms, which gradually progressed into respiratory distress. Therefore, the patient was intubated for dyspnea with pseudomembrane formation. A toxin-producing strain of C. ulcerans was identified, also detected in the patient's domestic cats. Multilocus sequence typing confirmed all strains, including the patient's isolate, as ST337. CONCLUSION: Multilocus sequence typing revealed zoonotic transmission of C. ulcerans from domestic cats to a human.

Early postoperative prediction of both disease remission and long-term disease control in acromegaly using the oral glucose tolerance test.

PURPOSE: Transsphenoidal surgery (TSS) is the cornerstone of acromegaly treatment. Two biochemical parameters, growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels, sometimes diverge postoperatively; however, it is important to maintain disease control without further treatment, regardless of whether these parameters converge. This study investigated whether remission and long-term disease control could be predicted using early postoperative GH and IGF-1 levels. METHODS: We reviewed 36 consecutive surgically treated patients with acromegaly. IGF-1 levels and minimum GH levels during an oral glucose tolerance test (OGTT) were evaluated at 2 weeks, as well as at 3 months postoperatively. After comparison between the remission and nonremission groups, we analyzed whether early postoperative parameters could predict remission and long-term disease control. RESULTS: Twenty-five patients (69.4%, Group A) achieved remission within 1 year postoperatively. Of the remaining patients (median follow-up period, 53 months), seven (19.5%, Group B) maintained normal IGF-1 levels without treatment, whereas four (11.1%, Group C) required additional treatment. GH levels <1.5 ng/mL measured on the morning after surgery and nadir GH levels <0.7 ng/mL during the OGTT conducted at 2 weeks postoperatively were predictive of remission, with the latter demonstrating 95.2% sensitivity and 100% specificity. All group C patients had nadir GH levels ≥0.7 ng/mL during the OGTT and IGF-1 levels ≥SD +3 at 2 weeks postoperatively. CONCLUSION: Early postoperative nadir GH levels during the OGTT and IGF-1 levels at 2 weeks postoperatively demonstrated excellent predictive value for both endocrinological remission and the necessity for additional treatment.

Gamma knife surgery-induced aneurysm rupture associated with tissue plasminogen activator injection: A case report and literature review.

BACKGROUND: Cases involving delayed development of intracranial aneurysms related to gamma knife surgery (GKS) have been recently reported. Here, we present a rare case of GKS-induced aneurysm rupture after intravenous injection of tissue plasminogen activator (t-PA) for occlusion of the middle cerebral artery (MCA). To the best of our knowledge, this is the first case in which t-PA-induced rupture of a GKS-related unruptured aneurysm. CASE DESCRIPTION: A 56-year-old woman underwent GKS for left trigeminal neuralgia. Eighteen years later, she suddenly experienced MCA occlusion with consciousness disturbance and right hemiparesis. She received an intravenous injection of t-PA and then was transferred to our hospital. We confirmed residual thrombus, and she underwent mechanical thrombectomy successfully. A postthrombectomy brain computed tomography scan revealed subarachnoid hemorrhage with a hematoma in the left cerebellar hemisphere. Cerebral angiography revealed a small irregular-shaped aneurysm at the branching site of the left circumflex branch at the distal position of the anterior inferior cerebellar artery, which was not detected on initial imaging. Coil embolization was performed. One month after the ischemic attack, she was transferred to a rehabilitation hospital, with a modified Rankin Scale score of 5. CONCLUSIONS: The tendency to rupture is greater for GKS-induced aneurysms than for intrinsic unruptured aneurysms, according to previous reports. When performing acute treatment for cerebral infarction in patients with a history of GKS, the presence of aneurysms should be evaluated and we should keep in mind that GKS aneurysms are very small and tend to rupture.