Postoperative disappearance of leptomeningeal enhancement around the brainstem in glioblastoma.

PURPOSE: Leptomeningeal enhancement (LME) suggests leptomeningeal dissemination (LMD) of tumor cells, which is a complication of end-stage glioblastoma, and is associated with a poor prognosis. However, magnetic resonance imaging (MRI) occasionally indicates the disappearance of peri-brainstem LME after surgical resection of glioblastoma. Since preoperative LMD may affect treatment indications, we aimed to analyze the clinical significance of preoperative LME of the brainstem in glioblastoma. METHODS: We retrospectively collected clinical and radiological data from consecutive patients with glioblastoma and preoperative LME of the brainstem, who were treated at our hospital between 2017 and 2020. RESULTS: Among 112 patients with glioblastoma, nine (8%) showed preoperative LME of the brainstem. In comparison with tumors without LME, tumor size was significantly associated with the preoperative LME of the brainstem (p = 0.016). In addition, there was a trend toward significance for a relationship between deep tumor location and preoperative LME of the brainstem (p = 0.058). Notably, among six patients who underwent surgical resection for glioblastoma with LME of the brainstem, four showed significant radiological disappearance of the LME on postoperative MRI. This suggests that the LME did not result from LMD in these cases. Moreover, these four patients lived longer than would be expected from the presence of LMD. However, this LME disappearance was not observed after biopsy or chemoradiotherapy. CONCLUSIONS: These findings suggest that preoperative LME does not necessarily indicate the presence of untreatable LMD; moreover, LME may disappear after surgical tumor resection. Thus, transient preoperative LME could be attributed to other mechanisms, including impaired venous flow due to intratumoral arteriovenous shunts, which can be resolved by reducing the tumor burden.

Intracranial aneurysms treated with stent-assisted coil embolization: evaluation with four-dimensional ultrashort-TE MR angiography.

OBJECTIVES: As a novel follow-up method for intracranial aneurysms treated with stent-assisted coil embolization (SACE), we developed four-dimensional magnetic resonance angiography (MRA) with minimized acoustic noise utilizing ultrashort-echo time (4D mUTE-MRA). We aimed to assess whether 4D mUTE-MRA is useful for the evaluation of intracranial aneurysms treated with SACE. METHODS: This study included 31 consecutive patients with intracranial aneurysm treated with SACE who underwent 4D mUTE-MRA at 3 T and digital subtraction angiography (DSA). For 4D mUTE-MRA, five dynamic MRA images with a spatial resolution of 0.5 × 0.5 × 0.5 mm3 were obtained every 200 ms. Two readers independently reviewed the 4D mUTE-MRA images to evaluate the aneurysm occlusion status (total occlusion, residual neck, and residual aneurysm) and the flow in the stent using a 4-point scale (from 1 [not visible] to 4 [excellent]). The interobserver and intermodality agreement was assessed using κ statistics. RESULTS: On DSA images, 10 aneurysms were classified as total occlusion, 14 as residual neck, and 7 as residual aneurysm. In terms of aneurysm occlusion status, the intermodality and interobserver agreement was excellent (κ = 0.92 and κ = 0.96, respectively). For the flow in the stents on 4D mUTE-MRA, the mean score was significantly higher for single stents than multiple stents (p < .001) and for open-cell type stents than closed-cell type (p < .01). CONCLUSIONS: 4D mUTE-MRA is a useful tool with a high spatial and temporal resolution for the evaluation of intracranial aneurysms treated with SACE. KEY POINTS: • In the evaluation of intracranial aneurysms treated with SACE on 4D mUTE-MRA and DSA, the intermodality and interobserver agreement in aneurysm occlusion status was excellent. • 4D mUTE-MRA shows good to excellent visualization of flow in the stents, especially for cases treated with a single or open-cell stent. • 4D mUTE-MRA can provide hemodynamic information related to embolized aneurysms and the distal arteries to stented parent arteries.

Variations in the branching patterns of the anterior choroidal artery: an angiographic study with special reference to temporal lobe epilepsy surgery.

BACKGROUND: The preservation of the anterior choroidal artery (AChA) is essential for avoiding neurological sequelae after mesial temporal lobe epilepsy (mTLE) surgery. The purpose of this study is to reveal the anatomical variation in which the perforating branches arise from the plexal segment of the AChA by using a modern neuroimaging modality. METHODS: This study analyzed 3D rotational angiography (3DRA) images from 56 subjects. The AChA and perforating branches were visualized using slab MIP. We analyzed branching patterns, courses of the perforating arteries arising from the plexal segment of the AChA, and the anastomosis of the AChA with other cerebral arteries. RESULTS: The slab MIP applied to 3DRA visualized one or more perforating branches from the AChA in 92.9% of cases. The presence of perforating branches arising from the AChA plexal segment was 17.3%. Most of the branching points of plexal perforators were likely located in the operative field during hippocampal resection. The course of the AChA plexal perforators included the posterior limb of the internal capsule. Anastomosis with other cerebral arteries was visualized in 25% of the AChA with plexal perforators. CONCLUSIONS: 3DRA slab MIP was useful for visualizing the perforating branches of the AChA. Our results showed the possibility that surgical manipulation of the choroid plexus may cause infarction in the AChA territory. We suggest that the existence of the AChA plexal perforators should be recognized to further enhance the safety of hippocampal resection for mTLE.

Multiple Cerebral Aneurysms Associated With Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1) is a rare disease with an incidence of approximately one in every 3000 births. NF1 is mainly recognized as a tumor suppressor. Vasculopathy in NF1 is well described in the literature, but the association between NF1 and cerebral aneurysm has not been determined. We report a case of a 67-year-old female with NF1 accompanied by 8 cerebral aneurysms. Two of the patient's unruptured aneurysms, the large distal anterior cerebral artery (ACA) aneurysm and anterior communicating artery aneurysm, were initially treated with microsurgical clipping. The peripheral ACA aneurysm gradually increased in size and ruptured after 5 years. Coil embolization was performed for the ruptured aneurysm. Four of the 5 remaining unruptured aneurysms were treated surgically. The patient is currently well, without neurological deficit, and coil embolization is scheduled for the last remaining aneurysm. NF1 is a probable risk factor for multiple cerebral aneurysms due to vessel wall vulnerability. Therapeutic indications for patients with NF1 who show multiple cerebral aneurysms include strict follow-up and aggressive treatment to avoid subarachnoid hemorrhage.

Prolonged Mean Transit Time Detected by Dynamic Susceptibility Contrast Magnetic Resonance Imaging Predicts Cerebrovascular Reserve Impairment in Patients with Moyamoya Disease.

BACKGROUND: Evaluating cerebrovascular reserve (CVR) is important for patients with moyamoya disease (MMD). 123I-iodoamphetamine single-photon emission CT (SPECT) with acetazolamide (ACZ) challenge is widely carried out, but using ACZ becomes problematic owing to its off-label use and its adverse effects. Here, we report the efficacy of dynamic susceptibility contrast MRI (DSC-MRI) for the evaluation of CVR in MMD patients. METHODS: All 33 MMD patients underwent both SPECT and DSC-MRI at an interval of <10 days from each other (mean age 38.3 years). The region of interest (ROI) was the anterior cerebral artery (ACA) territory, middle cerebral artery (MCA) territory, basal ganglia and cerebellum hemisphere for cerebral blood flow (CBF), cerebral blood volume (CBV) and mean transit time (MTT) images. The ratios of the ROIs to the ipsilateral cerebellum were calculated for each parameter and evaluated. The CVR was calculated using images acquired by SPECT before and after ACZ administration. The ratios of DSC-MRI parameters and CVR were compared and evaluated for each ROI. RESULTS: The MTT of the ACA and MCA territories significantly correlated with CVR (p < 0.0001). However, CBF and CBV had no correlation with CVR. The MTT ratio had a threshold of 1.966, with a sensitivity of 68.4% and a specificity of 91.5% for predicting decreased CVR (<10%). CONCLUSION: MTT had a negative correlation with CVR. DSC-MRI is easy, safe and useful for detecting decreased CVR and can be used as a standard examination in MMD patient's care.

Evaluation of Intracranial Arteriovenous Malformations With Four-Dimensional Arterial-Spin Labeling-Based 3-T Magnetic Resonance Angiography.

OBJECTIVE: We aimed to assess the usefulness of 3-T 4-dimensional (4D) arterial spin-labeling (ASL)-based magnetic resonance angiography (MRA) with color-coded time-of-arrival (TOA) maps for the evaluation of cerebral arteriovenous malformations (AVMs). METHODS: Our study included 6 patients with cerebral AVMs. They underwent 4D-ASL MRA at 3T and digital subtraction angiography. A pseudocontinuous arterial spin labeling protocol with look-locker sampling was used for spin labeling. Two independent readers reviewed the 4D-ASL MRA images with color-coded TOA maps for the nidus size, arterial feeders, and venous drainage. Two other readers consensually reviewed the digital subtraction angiography images. RESULTS: The cerebral AVMs were demonstrated on all 4D-ASL MRA images. In 5 high-flow AVMs, the color-coded TOA maps were especially useful for identifying the feeder/drainer. Intermodality agreement was excellent for the nidus size (κ = 1.0), very good for arterial feeders (κ = 0.88), and good for venous drainage (κ = 0.80). CONCLUSIONS: The 4D-ASL 3-T MRA with color-coded TOA maps is useful for assessing the gross angiographic characteristics of intracranial AVMs.

The leptomeningeal ivy sign on fluid-attenuated inversion recovery images in moyamoya disease: positron emission tomography study.

BACKGROUND: The ivy sign is sometimes seen on fluid-attenuated inversion recovery (FLAIR) images in moyamoya disease (MMD). In recent studies using single-photon emission computed tomography, ivy sign proliferation correlated with decreases in cerebrovascular reserve. However, a decreased vascular reserve is not concrete. The purpose of this study was to evaluate the correlation between ivy sign proliferation and the findings of 15O gas positron emission tomography (PET). METHODS: In 19 MMD patients (12 women, age 31-69 years) with ischemic symptoms, FLAIR magnetic resonance imaging and 15O gas PET were performed. We classified the middle cerebral artery (MCA) territory into 2 regions in each hemisphere, and the degree of the ivy sign (ivy sign score) in each region was classified into 3 grades (0-2), where grade 0 indicated an absence of the ivy sign, grade 1 indicated that the ivy sign was seen on less than half of the cortical surface in each region, and grade 2 indicated that the ivy sign was seen on more than half of the cortical surface. We examined the relationship among the ivy sign score, the severity of ischemic symptoms and PET parameters in 76 MCA regions of 19 patients. RESULTS: Ivy sign scores of the regions were 0 (n = 19), 1 (n = 40), and 2 (n = 17). Total ivy sign score of a hemisphere increased as clinical symptoms became more severe. Cerebral blood flow (CBF) values were lower, cerebral blood volume (CBV) values were higher, and CBF/CBV values were lower than those of controls as symptoms became severe (p < 0.05). CBF and CBF/CBV values decreased and CBV values increased as the ivy sign score increased, and were significantly higher and lower, respectively, than control values (p < 0.05). No significant differences in cerebral metabolic rate of oxygen and oxygen extraction fraction were found between the 3 ivy sign scores. A positive correlation was found between ivy sign score and increases in CBV (p < 0.01), and a more obvious negative correlation was found between ivy sign score and decreases in CBF/CBV (p < 0.001). CONCLUSIONS: We evaluated the correlation between ivy sign proliferation and the findings of 15O gas PET. We suggested that ivy sign proliferation was associated with both dilated pial vasculature and the slow flow of developed leptomeningeal collaterals in patients with MMD.

Haptoglobin phenotype predicts cerebral vasospasm and clinical deterioration after aneurysmal subarachnoid hemorrhage.

Vasospasm (VS) and delayed cerebral ischemia (DCI) after subarachnoid hemorrhage (SAH) are thought to greatly affect prognosis. Haptoglobin (Hp) is a hemoglobin-binding protein expressed by a genetic polymorphism (1-1, 2-1, and 2-2). Our objects were to investigate whether the Hp phenotype could predict the incidence of cerebral infarction, favorable outcome, clinical deterioration by DCI, and angiographical VS after aneurysmal SAH. Ninety-five consecutive patients who underwent clipping or coil embolization were studied. Favorable functional outcome was defined as a modified Rankin Scale score of 0-2 at 3 months. Angiographical VS was diagnosed based on cerebral angiography findings performed between days 7 and 10 after SAH. The Hp 2-2 group had a significantly greater risk of angiographical VS than that of Hp 2-1 and 1-1 groups combined on univariate (odds ratio [OR]: 3.60, confidence interval [CI]: 1.49-8.67, P = .003) and multivariate logistic regression analyses after being adjusted for age, sex, Fisher groups, and other risk factors (OR: 3.75, CI: 1.54-9.16, P = .004). The Hp 2-2 group also showed the tendency of a greater risk of clinical deterioration by DCI with marginal significance on univariate and age- and sex-adjusted analyses (univariate OR: 2.46, CI: .90-6.74, P = .080; age- and sex-adjusted OR: 2.46, CI: .89-6.82, P = .080) but not after being adjusted for other multiple risk factors. The Hp 2-2 group was not associated with the favorable 3-month outcome and cerebral infarction (univariate: P = .867, P = .209; multivariate: P = .905, P = .292). The Hp phenotype seems to be associated with a higher rate of angiographical VS and clinical deterioration by DCI but does not affect the incidence of cerebral infarction and favorable outcome.

Case Report: RNF213 variant and choroidal anastomosis as potential risk factors for early stroke in moyamoya syndrome associated with Down syndrome.

Introduction: Recent studies have suggested associations between RNF213 variants and the formation of periventricular anastomosis among patients with moyamoya disease, leading to early onset of cerebral hemorrhage and rebleeding. Case description: We report herein the case of a boy with Down syndrome and moyamoya syndrome. Exome sequencing identified a heterozygous RNF213 R4810K variant. After ischemic stroke occurred at 9 years old, indirect surgical revascularization was performed for the left cerebral hemisphere and improved ischemic symptoms and cerebral hypoperfusion, while the left choroidal anastomosis remained. At 13 years old, he presented with left thalamic hemorrhage attributed to the anterior choroidal artery, with rebleeding observed four days after the initial hemorrhage under strict blood pressure control. The patient was discharged without neurological deficits 20 days after the hemorrhagic stroke. Conclusion: Presence of an RNF213 variant and choroidal anastomosis may represent risk factors for cerebral hemorrhage in patients with Down syndrome and moyamoya syndrome, as well as in patients with moyamoya disease.

Olfactory hallucinations caused by an unruptured posterior communicating artery aneurysm improved by clipping: A case report with literature review.

Background: Unruptured cerebral aneurysms that lead to epilepsy are rare and olfactory hallucinations caused by such an aneurysm are extremely rare. Various treatments have been proposed, including wrapping, clipping with or without cortical resection, and coil embolization, but there is no consensus on the best approach. Case Description: We present a case of a 69-year-old female who experienced olfactory hallucinations caused by a posterior communicating artery aneurysm and was treated with clipping without cortical resection, with a positive outcome. Conclusion: According to our knowledge, there has been only one report of a posterior communicating artery aneurysm presenting with olfactory hallucinations has been reported, where clipping and cortical resection were performed. This is the first report of a posterior communicating artery aneurysm with olfactory hallucinations that was effectively treated with clipping alone. There have been a few similar reports of large middle cerebral artery aneurysms, most of which are believed to be caused by entorhinal cortex compression. Although a definitive treatment protocol for this condition remains elusive, we suggest that elimination of the pulsatile compressive stress exerted on the cerebral cortex through surgical clipping or coil embolization is crucial for achieving efficacious seizure management.

A Case of Cavernous Sinus Dural Arteriovenous Fistula Draining Solely to the Superior Ophthalmic Vein with Normal Cerebral Venous Flow from the Superficial Middle Cerebral Vein to the Inferior Petrosal Sinus due to a Septum in the Cavernous Sinus.

Objective: We report here an atypical case of cavernous sinus dural arteriovenous fistula (CSDAVF) with a septation that separates the cavernous sinus (CS) into two components, namely, normal cerebral venous drainage and shunted blood drainage into the superior ophthalmic vein (SOV) alone. The CSDAVF was successfully treated by selective transvenous embolization (TVE) through the septum with the trans-inferior petrosal sinus (IPS) approach. Case Presentation: A 74-year-old woman presented with right exophthalmos and tinnitus on the right side. Neuroradiological examination showed CSDAVF mainly supplied by multiple feeders from the bilateral ascending pharyngeal artery and meningohypophyseal trunk with a shunted pouch located medial-dorsally to the right CS. Blood from the CSDAVF drained via the anterior component of the CS to the right SOV only. Normal cerebral venous blood from the right superficial middle cerebral vein drained through the dorsolateral component of the right CS into the right IPS. These findings suggest that a septal barrier exists between the outflow tract of the dural arteriovenous fistula and the normal cerebral venous outflow tract within the CS. The CSDAVF was successfully treated by selective TVE through the septum with the trans-IPS approach after detailed evaluation of 3D rotational angiography (3DRA) and MRA/MR venography (MRV) cross-sectional images. The patient's symptoms improved, and she was discharged uneventfully. Conclusion: Septation within the CS can completely separate the drainage route of the CSDAVF from the normal cerebral drainage route. Successful catheterization to the shunted pouch through the septum with the IPS approach and selective embolization were possible with detailed evaluation of anatomy on MRA/MRV cross-sectional images and 3DRA images.

Inhalational Anesthesia Reduced Transient Neurological Events After Revascularization Surgery for Moyamoya Disease.

BACKGROUND AND OBJECTIVES: The choice between inhalational and total intravenous anesthesia (TIVA) in revascularization surgery for Moyamoya disease (MMD) remains a topic of debate. Anesthesia methods have changed with the advent of new anesthetics. This study investigated whether modern anesthesia methods affected the development of neurological symptoms after revascularization surgery for MMD. METHODS: This single-center retrospective study included 63 adult patients (82 hemispheres) with MMD treated with direct and indirect bypass surgeries at our hospital between 2013 and 2022. Patients were divided into inhalational anesthesia (IA) and TIVA groups based on the anesthesia maintenance method. Baseline patient characteristics; postoperative neurological symptoms, including hyperperfusion syndrome, cerebral infarction, and transient neurological events (TNEs); and cortical hyperintensity belt (CHB) sign scores (5-point scale from 0 to 4) on postoperative magnetic resonance imaging were compared between the two groups. The operation methods, anesthetics, and intraoperative hemodynamic and ventilatory parameters were compared between patients with and without TNEs. RESULTS: The IA and TIVA groups comprised 39 and 43 hemispheres, respectively. The frequency of postoperative hyperperfusion syndrome and cerebral infarction did not differ between the groups, but the number of TNEs in the IA group (5/39; 13%) was significantly lower than that in the TIVA group (16/43; 37%). Multivariate logistic regression analysis revealed that TNEs were associated with TIVA (odds ratio, 3.91; 95% CI, 1.24-12.35; P = .02). The median [IQR] postoperative CHB sign score in the IA group (2 [1-3]) was significantly lower than that in the TIVA group (4 [3-4]). CONCLUSION: The IA group had fewer postoperative TNEs and lower CHB sign scores than the TIVA group. Although further studies are needed, this study provides insights into the prevention of TNEs with IA and reconsideration of the optimal anesthesia for MMD.

Outer-diameter narrowing of the internal carotid and middle cerebral arteries in moyamoya disease detected on 3D constructive interference in steady-state MR image: is arterial constrictive remodeling a major pathogenesis?

BACKGROUND: To obtain information on affected vessels in moyamoya disease (MMD), we analyzed the vascular morphological characteristics of MMD using three-dimensional (3D) constructive interference in steady-state (CISS) magnetic resonance imaging (MRI). METHODS: The population of this 3D-CISS MRI study consisted of 51 patients with MMD: 16 patients with atherosclerotic middle cerebral artery (MCA) stenosis or occlusion, 42 MRI control patients, and 28 control digital subtraction angiography (DSA) patients. We measured the outer diameters of the terminal portion of the internal carotid artery (ICA) and the proximal portion of the MCA (M1 portion). We evaluated the inner diameter as the relative value (%) obtained from magnified DSA images and analyzed these data. RESULTS: The outer diameters of the ICA and M1 portions were significantly smaller in the MMD group than in the other two groups, while the M1 outer diameter of the atherosclerosis group was not significantly different compared to the control (ICA: MMD, 2.61 ± 0.46 mm vs. control, 4.04 ± 0.50 mm and M1: MMD, 1.92 ± 0.43 mm vs. control, 3.34 ± 0.54 mm vs. atherosclerosis, 3.45 ± 0.56 mm). Furthermore, in MMD patients, the outer diameter was unrelated to the progression of the luminal stenosis grade estimated by DSA. CONCLUSIONS: This is the first report that the outer diameters of both the ICA and M1 decrease in MMD patients. Our findings suggest that the vascular constrictive changes of the affected arteries are an important phenomenon reflecting MMD pathology.

Factors Associated with Rapidly Deteriorating Myelopathy in Patients with Spinal Arteriovenous Shunts.

Spinal arteriovenous (AV) shunts are rare conditions that sometimes present with myelopathy symptoms. The progression of the symptoms is usually gradual; however, some cases show rapid deterioration. We retrospectively investigated the factors that induced the rapid deterioration of myelopathy symptoms in patients with spinal AV shunts. We treated 33 patients with myelopathy with spinal AV shunts at our institutions, eight of whom experienced rapid deterioration (within 24 hours: 24.2%). Of these, three were related to the body movement or particular postures associated with playing golf, 30 minutes of Japanese straight sitting, and massage care. One patient showed deterioration after embolization for a tracheal aneurysm. The remaining four patients received steroid pulse therapy (high-dose steroid infusion) shortly before the rapid deterioration. These symptoms stopped progressing after cessation of steroid use. While positional or physical factors contributing to myelopathy deterioration might exist, we could not identify specific factors in this study. Nevertheless, rapid deterioration was frequently observed after high-dose steroid use. We must take care not to administer high-dose steroids for myelopathy caused by spinal AV shunt disease.

Minimally invasive treatment strategy for partially thrombosed anterior inferior cerebellar artery aneurysm: A case report.

BACKGROUND: Partially thrombosed anterior inferior cerebellar artery (AICA) aneurysms are extremely rare; thus, no established therapeutic approach exists. CASE DESCRIPTION: We report a large, partially thrombosed AICA aneurysm and discuss its therapeutic nuances. The aneurysm was asymptomatic; therefore, we aimed to treat it through a minimally invasive procedure. The aneurysm was of fusiform type and the proximal neck of the aneurysm was positioned at midline in front of the brainstem. To approach the neck, posterior transpetrosal approach is recommended. However, this approach can be invasive; thus, we performed distal clipping of the aneurysm using transcondylar fossa approach with occipital artery-AICA bypass to avoid ischemia of the AICA territory. Although the size of the aneurysm initially increased, it subsequently decreased. CONCLUSION: This is a rare case report describing the long-term clinical course after distal clipping in detail. We showed that traditional microsurgical techniques can be applied to treat patients with new, minimally invasive treatment strategies.

Intracranial and extracranial multiple arterial dissecting aneurysms in rheumatoid arthritis: A case report.

OBJECTIVE: We describe a case of intracranial and extracranial multiple arterial dissecting aneurysms in rheumatoid arthritis (RA). CASE PRESENTATION: A 29-year-old man with a medical history of RA since 18 years of age was admitted to our hospital for vomiting, dysarthria, and conscious disturbance. At 23, he underwent ligation of the left internal carotid artery (ICA) with superficial temporal artery to middle cerebral artery anastomosis because of acute infarct of the left hemisphere caused by arterial dissection of the left ICA. During the current admission, computed tomography (CT) revealed subarachnoid hemorrhage, and digital subtraction angiography (DSA) demonstrated dissecting aneurysms of the left intracranial vertebral artery (VA) and right extracranial VA. We diagnosed him with a ruptured dissecting aneurysm of the left intracranial VA and performed endovascular parent artery occlusion on the left VA. For the right unruptured VA aneurysm, we performed coil embolization simultaneously. At 2 weeks after the endovascular treatment, follow-up DSA revealed that multiple de novo dissecting aneurysms developed on the origin of the left VA and left and right internal thoracic arteries. Those aneurysms were treated with coil embolization. Other remaining aneurysms on the left thyrocervical trunk, right transverse cervical artery, and both common iliac arteries were treated by conservative therapy. While continuing medical treatment for RA, the patient recovered and was discharged to a rehabilitation hospital. CONCLUSION: Considering that RA-induced vasculitis can be a potential risk of vascular complications including multiple arterial dissections, physicians should carefully perform endovascular interventional procedures for patients with long-term RA.

Hemodynamic study about cortical hyperintensity belt sign after direct bypass surgery for moyamoya disease.

Transient neurological events (TNEs) are observed after direct bypass surgery in patients with moyamoya disease (MMD). Although a correlation between cortical hyperintensity belt signs (CHBs) and TNEs has been reported, the pathophysiology of CHBs is still unknown. The purpose of this study was to reveal the pathophysiology of CHBs by using dynamic susceptibility contrast-magnetic resonance imaging. Thirty patients with MMD were included in this study. We provided scores (0-2) for the existence of CHBs on postoperative FLAIR images. We placed the ROI for the presented area of CHBs in the images of cerebral blood flow, CBV, and MTT. We calculated the change of the hemodynamic parameters (increase ratio, IR) and analyzed the relationship between IRs, CHB scores, and TNEs. TNEs were observed in 15 cases (50%) and CHBs were detected in 28 cases (93%). TNEs showed significantly higher CHB scores than those without (p < 0.05). The group of CHB score 2 showed a significantly higher CBV IR than the group with of score 0 (p < 0.05). Patients with TNEs showed a significantly higher CBV IR than those without (p < 0.05). As for the cut-off level to predict an appearance of TNEs, the CBV IR was 1.36 by the Receiver Operating Characteristic analysis, and the sensitivity and specificity were 80% respectively. We hypothesize that the pathophysiology of the CHBs are vasogenic edemas because the postoperative CBV increase correlated with the CHBs.

Remote Lesions of Synchronous Sacral and Cervical Dural Arteriovenous Fistulas: A Case Report.

BACKGROUND: Spinal dural arteriovenous fistula (DAVF) is a rare disease, and multiple concurrent lesions are extremely rare. Such multiple spinal DAVFs have similar outflow routes at neighboring levels. However, there have been no reported cases of remote lesions of multiple spinal DAVFs. The authors herein report a case of double synchronous cervical and sacral DAVFs. CASE DESCRIPTION: A 56-year-old man with no previous history of disease presented with progressive myelopathy, and spinal magnetic resonance imaging showed extensive edema of the spinal cord from the conus to T7. The initial angiography incidentally revealed a C1 DAVF, but the sacral DAVF was not diagnosed. The C1 DAVF was treated surgically, because of the risk of subarachnoid hemorrhage. A second angiography was performed 2 months after the first surgery because of high clinical suspicion, and the sacral DAVF was diagnosed at that time. The sacral DAVF was treated surgically, and the symptoms gradually resolved within 6 months after surgery. CONCLUSIONS: Spinal DAVF is a rare disease, but clinicians should be aware of the possibility of the concurrence of multiple spinal fistulas located at different levels in the same patient.

High prevalence of small vessel disease long after cranial irradiation.

Accelerated atherosclerosis of intra- or extracranial large arteries is a major cause of radiation-induced strokes. Recent development of MRI techniques has enabled detection of another important vasculopathy: microinfarcts or microbleeds after cranial irradiation. The purpose of this study was to investigate the incidence and characteristics of small vessel disease after cranial irradiation. MR images and clinical records of 102 outpatients who had undergone cranial irradiation to brain tumors were retrospectively reviewed. Only those who had undergone T1WI, T2WI, FLAIR, and T2∗WI during follow-up were included. Small vessel disease including small subcortical infracts, microbleeds, and volume of white matter lesions were assessed on the latest MR images of each patient. Ischemic or hemorrhagic stroke during the follow-up period was also reviewed. Twelve patients (mean age at the latest MRI, 38.5years; 8men) were assessed. The total radiation dose was 52.3±9.7Gy in these patients, 9 of whom received whole brain irradiation. Small subcortical infarcts (mean 2.3) were detected in 8 patients, microbleeds (mean 19.4) were detected in 11, and white matter lesion volume was 38.3±11.6ml. During the follow-up period of 19.8±9.7years, 5 patients experienced stroke (4 lacunar, 1 hemorrhagic). These strokes occurred as long as a median 21.9 (range, 10.4-30.2) years after cranial irradiation. In conclusion, small vessel disease is not a rare complication after cranial irradiation, even in young patients. Patients after cranial irradiation should be followed up with MR imaging including a hemosiderin-sensitive sequence.

Subarachnoid Hemorrhage Because of Distal Superior Cerebellar Artery Dissection in Neurofibromatosis Type 1.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a rare disease with an incidence of 1 in every 3000 births. Numerous studies have focused on the main function of NF1 as a tumor suppressor, whereas few have examined the cerebrovascular abnormalities observed in patients with NF1. It is worth noting that intracranial aneurysms are uncommon in this condition. CASE DESCRIPTION: We report a case of NF1 with a dissection of the distal segment of the superior cerebellar artery. A 36-year-old woman presented with a distal superior cerebellar artery (SCA) dissection causing subarachnoid hemorrhage. Subsequently, because of the rich collateral blood flow distal to the dissection, N-butyl cyanoacrylate (NBCA) glue embolization was unsuccessful. Therefore, direct trapping of the artery was necessary. The patient was discharged after an uneventful postoperative period, and has remained without complications. CONCLUSIONS: In the treatment of subarachnoid hemorrhage because of a distal SCA dissection in patients with NF1, NBCA glue embolization may be a safer option than microsurgery or coil embolization, in the acute phase, considering the possible vulnerability of the vessel wall, accessibility, morphology of the lesions, and the risk of developing unpredictable infarcts in the case of parent artery occlusion. However, regular reevaluation of the blood flow is necessary to monitor recurrence, given the rich collateral circulation.