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Moyamoya disease (MMD) is a cerebrovascular disorder that is predominantly observed in women of East Asian descent, and is characterized by progressive stenosis of the internal carotid artery, beginning in early childhood, and a distinctive network of collateral vessels known as "moyamoya vessels" in the basal ganglia. Additionally, a prevalent genetic variant found in most MMD cases is the p.R4810K polymorphism of RNF213 on chromosome 17q25.3. Recent studies have revealed that RNF213 mutations are associated not only with MMD, but also with other systemic vascular disorders, including intracranial atherosclerosis and systemic vascular abnormalities such as pulmonary artery stenosis and coronary artery diseases. Therefore, the concept of "RNF213-related vasculopathy" has been proposed. This review focuses on polymorphisms in the RNF213 gene and describes a wide range of clinical and genetic phenotypes associated with RNF213-related vasculopathy. The RNF213 gene has been suggested to play an important role in the pathogenesis of vascular diseases and developing new therapies. Therefore, further research and knowledge sharing through collaboration between clinicians and researchers are required.

A patient with trigeminal neuralgia due to venous compression was successfully treated by transposition achieved by drilling the suprameatal tubercle. A 53-year-old woman presented with classical trigeminal neuralgia affecting the maxillary division of the right trigeminal nerve. MRI and CT revealed a bony prominence, called the suprameatal tubercle, above the opening of the internal acoustic meatus. MRI showed a thick venous flow void interposed between the suprameatal tubercle and trigeminal nerve. The patient underwent retrosigmoid craniotomy. The bony prominence restricted transposition of the compressed vein, so the compressed vein was successfully transposed after drilling the prominence. The patient's symptoms resolved completely. Drilling the suprameatal tubercle is useful for transposing sandwiched compression vessels.

Background: Few studies have used simulation models to examine long-term improvement in microsurgical technique. We investigated whether improvement in surgical technique could be assessed by continuous, objective, contest-format evaluation of the same microsurgical task., Methods: Since 2014, neurosurgeons with 1-10 years of experience participated in a biannual competition-format test. The task involved creating as many sutures as possible during the 5-minute interval after arteriotomy of a 1-mm artificial vessel. A modified version of the Objective Structured Assessment of Technical Skills examination was created and used. Changes and differences in scores over time were examined for each evaluator., Results: Overall, 103 neurosurgeons participated in the study at least once, and those who participated more than once were divided into two groups: those who had the highest score in each contest and those who had the lowest score. The linear regression equations for the highest and lowest scorers were y=7.62x+81.56 (R 2 =0.628) and y=1.94x+67.93 (R 2 =0.0433), respectively. High scorers had high scores from the first time they participated, and their scores tended to increase further, while scores for low scorers tended not to increase with additional experience. Scores for the four evaluators did not significantly differ., Conclusions: Our results suggest that technical improvement in surgery can be assessed by long-term, continuous evaluation of microsurgical technique and that the present evaluation system might help increase surgical safety.

Background: Initial management of severe traumatic brain injury is important and includes treatment decision-making and prediction of prognosis. We examined whether biomarkers at admission could be useful prognostic predictors. We focused on electrolytes and blood glucose, which can be measured easily at any facility and for which results can be obtained promptly, before those of other biomarkers, such as D-dimer., Methods: All trauma patients with head injuries treated at Chiba Hokusoh Hospital between 2014 and 2017 were investigated. Cases of multiple trauma accompanied by fatal trauma, hemorrhagic shock, or cardiopulmonary arrest, and pediatric cases, were excluded from this study. Blood gas data at the initial hospital visit were reviewed retrospectively. A poor outcome was defined as death during hospitalization or a vegetative state due to head injury. Factors related to poor outcomes were analyzed., Results: Of the 185 male and 79 female patients studied, 34 had poor outcomes. Poor outcome was significantly correlated with potassium (P = 0.003), glucose (P < 0.001), and glucose-to-potassium ratio (P < 0.001) at arrival. The odds ratio was 4.079 for a glucose-to-potassium ratio of ≥50., Conclusions: We evaluated blood gas data at the initial hospital visit, as these results can be obtained more quickly than those of other biomarkers assessed previously. Serum glucose-to-potassium ratio at admission may be a potential predictor of prognosis for severe traumatic brain injury.

Objective: Petrous internal carotid aneurysm (PA) concomitant with a mass lesion and cranial nerve palsy is relatively rare. Flow-diverter stent implantation is now widely used as an alternative treatment for PA. However, alternative treatments sometimes cannot be used because of tortuosity of the carotid artery, allergies to contrast material, and high costs. The outcomes of different treatment methods should therefore be assessed. Here, we review the available literature on treatments for PA., Methods: In a search using the terms "aneurysm", "carotid artery", and "petrous" on PubMed, MEDLINE, and databases such as OvidSP, 221 articles were identified. We also performed a literature review and discuss and compare the causes, symptoms, treatment methods, and clinical outcomes of PA., Results and Conclusions: Onset of secondary aneurysm was generally heralded by bleeding (p<0.001), while onset of primary aneurysm was heralded by cranial nerve deficit (p= 0.0014). Outcomes after treatment of 34 cranial nerve palsies in 25 patients are reported.

Tumors of the skull base, such as meningiomas, tend to recur. With progress in free vascularized flap surgery, an increasing number of studies are investigating skull base reconstruction with free flaps after tumor removal. In this report, we discuss the results of second free flap surgery after skull base reconstructive surgery. We retrospectively analyzed data from patients treated at our center during the period from 2013 through 2017. All four patients identified had skull base anaplastic meningioma and had undergone radiotherapy. In all cases, the flap and donor blood vessel were sourced from sites that differed from those used in the previous surgeries. No complications developed, such as cerebrospinal fluid leakage, meningitis, wound infection, wound hemorrhage, or flap necrosis. Because the first flap was found to be unviable, it was difficult to preserve and was removed. Essential points in preventing complications are anchoring at the appropriate site, pinprick testing of the created flap, and use of multilayered countermeasures to prevent cerebrospinal fluid leakage.

Background: Infundibular dilatation (ID) is a funnel-shaped enlargement of the origin of cerebral arteries. The coexistence of an aneurysm and ID is relatively rare. Patients with IDs are rarely followed up. However, some IDs have been reported to develop into aneurysms with subsequent rupture. Here we report on a case of an aneurysm that coexisted with ID of the posterior communicating artery., Case Presentation: A 51-year-old woman underwent magnetic resonance imaging (MRI) to check for aneurysms and other problems. MRI revealed an unruptured aneurysm of the right internal carotid artery, for which the patient was admitted to our hospital. Three-dimensional computed tomographic angiography revealed an aneurysm, which protruded outward, and ID of the posterior communicating artery, which protruded inward. A right pterional craniotomy was performed with aneurysm clipping. The postoperative course was uneventful. In this report, we demonstrate operative views of the aneurysm and ID with the use of neuroendoscopy., Conclusion: ID can develop into a true arterial aneurysm and potentially rupture. Therefore, we need to observe the patients with IDs carefully, particularly in young women.

Acute intracerebral hemorrhage (ICH) associated with mild anemia is commonly observed on radiological examination, and there are several reports of ruptured aneurysms occurring with ICH but without accompanying subarachnoid hemorrhage. However, the relationship among computed tomography (CT), magnetic resonance imaging (MRI), and intraoperative findings of ICH caused by ruptured cerebral aneurysm in patients with severe chronic anemia has been rarely reported and is poorly understood. Here, we report atypical radiological and intraoperative findings of acute ICH caused by ruptured cerebral aneurysm in a patient with severe chronic anemia. A 64-year-old man with anemia was admitted to our hospital after he experienced left hemiparesis and a disturbance of consciousness. At a referring institution, he showed evidence of macrocytic anemia (white blood cell count, 9,000/μL; red blood cell count, 104×10(4)/μL; hemoglobin, 4.0 g/dL; hematocrit, 12.2%; and platelet count, 26.6×10(4)/μL). Both CT and MRI showed a right frontal ICH. The outer ring of the hematoma appeared as low-density area on CT, a low-intensity area on T1-weighted MRI, and a high-intensity area on T2-weighted MRI with a serous component. The patient received a blood transfusion and underwent surgical removal of the hematoma the following day. The white serous effusion visualized with CT and MRI was identified as a blood clot in the hematoma cavity. The blood that leaks from blood vessels appears as a high-intensity area on CT because it undergoes plasma absorption in a solidification shrinkage process, and is, therefore, concentrated. Although we did not examine the white effusion to determine if serous components were present, we speculated that the effusion may have contained serous components. Therefore, we removed the part of the effusion that appeared as a low-density area on CT. The presence of ICH without subarachnoid hemorrhage suggested the possible adhesion and rupture of a previous aneurysm. Therefore, ICH appeared as a mixed density area on CT because bleeding may have occurred several times. Because radiological findings of ICH caused by ruptured cerebral aneurysm in patients with severe chronic anemia are similar to those of ICH and cerebral edema, we suggest that the atypical radiological findings of ICH caused by ruptured cerebral aneurysm in patients with severe chronic anemia should be carefully evaluated, especially when surgery is indicated.