Role of RNF213 polymorphism in defining quasi-moyamoya disease and definitive moyamoya disease.

OBJECTIVE: Quasi-moyamoya disease (QMMD) is moyamoya disease (MMD) associated with additional underlying diseases. Although the ring finger protein 213 (RNF213) c.14576G>A mutation is highly correlated with MMD in the Asian population, its relationship to QMMD is unclear. Therefore, in this study the authors sought to investigate the RNF213 c.14576G>A mutation in the genetic diagnosis and classification of QMMD. METHODS: This case-control study was conducted among four core hospitals. A screening system for the RNF213 c.14576G>A mutation based on high-resolution melting curve analysis was designed. The prevalence of RNF213 c.14576G>A was investigated in 76 patients with MMD and 10 patients with QMMD. RESULTS: There were no significant differences in age, sex, family history, and mode of onset between the two groups. Underlying diseases presenting in patients with QMMD were hyperthyroidism (n = 6), neurofibromatosis type 1 (n = 2), Sjögren's syndrome (n = 1), and meningitis (n =1). The RNF213 c.14576G>A mutation was found in 64 patients (84.2%) with MMD and 8 patients (80%) with QMMD; no significant difference in mutation frequency was observed between cohorts. CONCLUSIONS: There are two forms of QMMD, one in which the vascular abnormality is associated with an underlying disease, and the other in which MMD is coincidentally complicated by an unrelated underlying disease. It has been suggested that the presence or absence of the RNF213 c.14576G>A mutation may be useful in distinguishing between these disease types.

Bacterial Flora in the Sphenoid Sinus Changes with Perioperative Prophylactic Antibiotic Administration.

The complications of endonasal transsphenoidal surgery (ETSS) include meningitis and sinusitis, and these complications are troublesome. Some reports have investigated the type of bacteria and the susceptibility of sphenoid sinus mucosal flora to drugs. However, most specimens can be collected after perioperative antibiotic administration. In this study, 95 and 103 sphenoid sinus mucosal samples collected during ETSS from September 2013 to February 2015 and from June 2017 to January 2019, respectively, were examined for bacterial culture. Sphenoid sinus mucosal samples were collected after antibiotic administration in the first period, whereas samples were collected before antibiotic administration in the second period. Hence, the specimens in the second period were not affected by antibiotics. Moreover, drug susceptibility tests for the detected bacteria were performed. Overall, 52 and 51 bacterial isolates were collected during both periods. Gram-positive cocci (GPCs), including Staphylococcus aureus and Staphylococcus epidermidis, were more common in the non-antibiotic group than in the antibiotic group (p <0.01). However, the proportion of gram-negative rods (GNRs) did not significantly differ between the two groups (p = 0.54). The antibiotic group had a significantly higher proportion of bacteria resistant to ampicillin (p <0.01) and first-generation cephalosporin (p = 0.01) than the non-antibiotic group. In conclusion, there was a difference in bacterial flora in the sphenoid sinus mucosal samples collected before and after intraoperative antibiotic administration.

Incision of the anterior petroclinoidal fold during clipping for securing the proximal space of an internal carotid artery-posterior communicating artery aneurysm: a technical note.

Surgical clipping of an internal carotid artery (ICA)-posterior communicating artery (ICPC) aneurysm is often difficult in cases involving limited space to insert a clip at the proximal aneurysm neck hidden by the tent. In such cases, we perform incision of the anterior petroclinoidal fold to secure the proximal space for clip insertion. Between April 2013 and March 2018, we treated 89 ICPC aneurysm cases by clipping. Incision of the anterior petroclinoidal fold was performed in 15 of the 89 cases (16.8%). Fast imaging employing steady-state acquisition (FIESTA) magnetic resonance imaging (MRI) can indicate the locations of the aneurysm and tent and can help assess the need for tent incision. We widely dissected the distal sylvian fissure and sufficiently exposed around the aneurysmal space. We coagulated and cut the anterior petroclinoidal fold supra between the ICA and proximal neck of the aneurysm using a low-power bipolar system and needles or micro-scissors with care to avoid injury around structures such as the ICA, aneurysm, and oculomotor nerve. When using this strategy, we often select the Yasargil FT717 clip that has a curve along the skull base because of easy insertion. All cases showed complete aneurysm exclusion on three-dimensional computed tomography angiography, and there was no cerebral infarction, neurological deficit (such as hemiparesis), or oculomotor nerve palsy. Therefore, our strategy of incision of the anterior petroclinoidal fold during clipping for securing the proximal space of an ICA aneurysm is effective and safe.

Bilateral Chronic Subdural Hematoma Presenting with Pseudo-Subarachnoid Hemorrhage Sign on Computed Tomography.

BACKGROUND: On rare occasions, cisterns are demonstrated as high-density areas on computed tomography (CT) and misdiagnosed with subarachnoid hemorrhage (SAH). This false-positive finding is called pseudo-SAH. PATIENTS AND METHODS: From April 2014 to August 2018, a total of 161 patients with chronic subdural hematoma (CSDH) were treated in our hospital. For these cases, the existence of a pseudo-SAH sign on CT was retrospectively examined. RESULTS: One patient with bilateral CSDH showed pseudo-SAH and a further examination to evaluate vascular abnormalities causing true SAH was necessary. In three patients, the Sylvian fissures were demonstrated as high-density areas due to an atherosclerotic middle cerebral artery; however, the condition was not misdiagnosed with SAH. CONCLUSION: In cases of CSDH, there is a possibility that CT demonstrates a pseudo-SAH sign. In such cases, close examinations to exclude true SAH are mandatory.

De novo Aneurysm Formation on Internal Carotid Artery at Origin of Thick Posterior Communicating Artery: 7 Years after Transient Occlusion of Contralateral Internal Carotid Artery.

The incidence of de novo intracranial aneurysm formation has been reported to be 0.84% per year. It is rare for de novo aneurysm formation to be observed on serial radiological examinations. A 64-year-old male with a history of right internal carotid artery (ICA) occlusion 7 years ago had subarachnoid hemorrhage (SAH) due to a ruptured left ICA aneurysm at the bifurcation of the posterior communicating artery (PComA). At the time of ICA occlusion, the left PComA was thick, about 3.0 mm in diameter, and no aneurysm was detected on radiological examinations. Thirty-eight months later, a small aneurysm was detected on the left ICA on magnetic resonance angiography (MRA). At the onset of SAH, the aneurysm was larger than that observed on the previous MRA. Left frontotemporal craniotomy was performed, and the aneurysm was clipped. A thick PComA might contribute to the development of an aneurysm at its origin due to hemodynamic stress. Persistent hemodynamic stress may cause enlargement of an aneurysm in 4 years and its subsequent rupture. In patient with a thick PComA, close observation is necessary to screen for de novo formation of a cerebral aneurysm.

Persistent Primitive Hypoglossal Artery Associated With Multiple Cerebral Aneurysms.

Persistent primitive hypoglossal artery is a rare anastomosis between the carotid and basilar arteries, and sometimes associated with cerebral aneurysms. However, association of persistent primitive hypoglossal artery with aneurysms located on arteries other than persistent primitive hypoglossal artery itself or posterior circulation is very rare. An 80-year-old woman suffered from subarachnoid hemorrhage, whose angiography demonstrated aneurysms on the left middle cerebral artery and anterior communicating artery, and the left persistent primitive hypoglossal artery. The middle cerebral artery aneurysm was the origin of hemorrhage. Although repeated craniotomy was necessary for the left middle cerebral artery aneurysm, both aneurysms were successfully clipped. In our case, neither aneurysm was located on an artery related to the persistent primitive hypoglossal artery. There is a possibility that cases of persistent primitive hypoglossal artery are accompanied by cerebral aneurysms on arteries other than the persistent primitive hypoglossal artery or in the posterior circulation.

Aneurysm of lenticulostriate artery in a patient presenting with hemorrhage in the caudate nucleus and lateral ventricle-delayed appearance and spontaneous resolution.

BACKGROUND: An aneurysm of distal lenticulostriate artery is very rare. The natural course and management of this rare aneurysm are not clear. CASE DESCRIPTION: An 81-year-old woman developed consciousness disturbance. Computed tomography revealed hemorrhage in the right caudate nucleus and lateral ventricles. Three-dimensional computed tomographic angiography demonstrated only an aneurysm at the basilar artery. On angiography, on the sixth day, an aneurysm at the right lenticulostriate artery was demonstrated. Then, the aneurysm disappeared on three-dimensional computed tomographic angiography on the 15th day. Subsequent radiological examinations revealed no vascular anomaly in the right lenticulostriate artery. CONCLUSION: An aneurysm at this location can show dynamic changes based on radiological findings. Close radiological observation is necessary.

Dissecting Vertebral Artery Aneurysm Presenting Regrowth After Stent-Assisted Coil Embolization in Acute Stage.

For a case of dissecting vertebral artery aneurysm (DVAA) in a dominant vertebral artery (VA) or posterior inferior cerebellar artery (PICA)-involving lesion, stent-assisted coil embolization (SACE) is an effective technique to preserve blood flow of the VA. A 41-year-old man presented with subarachnoid hemorrhage. Angiography demonstrated DVAA on the left VA just distal to the PICA, and the right VA was thinner than the left. For this case, SACE was performed to preserve the left VA and PICA. On the 10th day, angiography showed recurrence of the dissection. The dissected portion had thickened and extended to both distal and proximal sides involving the PICA origin and proximal portion to the PICA. A second endovascular embolization was performed and the recurrent dissecting aneurysm was embolized including the main VA cavity. In cases of DVAA, there is a possibility of recurrence after SACE, if a dissecting cavity remains unembolized. Therefore, total embolization is necessary under close observation from multiple angles, including the down-the-barrel view.

Thromboembolic complications during endovascular treatment of ruptured cerebral aneurysms.

Background In cases of subarachnoid hemorrhage due to aneurysm rupture, the administration of an anticoagulant or antiplatelet agent involves the risk of rebleeding from the aneurysm. There is a possibility of inducing thromboembolic events during the endovascular embolization of ruptured cerebral aneurysms. Patients and methods From April 2006 to March 2017, we treated a total of 70 patients with ruptured cerebral aneurysms with an endovascular technique. Among them, five patients (7.1%) showed intra-arterial thrombus formation. The aneurysms were located at the anterior communicating artery and basilar artery in two patients each, and on the internal carotid artery at the bifurcation of the anterior choroidal artery (AChoA) in one. In these patients, the clinical course, radiological findings, and management were retrospectively reviewed. Results Thrombus formation was observed in the posterior cerebral artery, anterior cerebral artery (A2), AChoA, and middle cerebral artery. The timing of thrombus formation was during coil delivery in four cases, and guiding catheter advancement in one. As for thrombus management, for all patients, administrations of heparin and antiplatelet agents were performed. For four patients, urokinase injection into the affected arteries was added after the completion of embolization. Cerebral infarction was postoperatively identified in two patients, but no hemorrhage was noted. Conclusion Administrations of heparin and antiplatelet drugs should be performed appropriately during procedures, and close observation of the arterial condition on angiography is necessary. Once thromboembolism occurs during the endovascular embolization of ruptured cerebral aneurysms, adequate heparinization, and antiplatelet therapy should first be performed.

Radiological findings of transorbital penetrating intracranial injury in a child.

In penetrating injuries, woods are known to be difficult to detect with radiological imaging studies, because the wood density are known to be extremely close to the value of air on CT. Adjustment of CT window and reconstruction of a 3D image from CT images allowed us to more accurately distinguish wood from air and to find the fragment of the wooden chopstick. It is particularly useful in transorbital penetrating injury.