Nationwide survey on quasi-moyamoya disease in Japan

Objectives: Moyamoya disease (MMD) is a unique occlusive disease of the bilateral internal carotid arteries with moyamoya vessels. Inherited or acquired disorders and conditions may present in conjunction with MMD. This condition is known as quasi-moyamoya disease. To identify the clinical features of quasi-MMD in Japan, a nationwide survey was conducted. Patients and methods: A questionnaire was mailed directly to 241 departments that had acknowledged treating quasi-MMD patients in the primary survey. We ascertained the sex, age, family history, clinical manifestation, radiological findings, treatments, course of the disease, and daily activity of the patients. Results: A total of 114 departments replied to the questionnaire. The data of 108 patients (66 female and 42 male; female to male ratio 1.57) were registered and analyzed. Mean age was 30.6 years old, with a peak in children. Seven patients (7 %) exhibited familial MMD. The initial clinical manifestation was motor weakness, followed by transient ischemic attack and headache. Their imaging study type included ischemic type in 64 patients (63.4 %), bleeding type in seven (6.9 %), and normal in 27 (26.7 %). Stenoocclusive lesion was seen in the internal carotid artery in more than half of the patients. Development of moyamoya vessels was mild in approximately 40 % of the patients. Almost all cases were accompanied by cerebral hypoperfusion. About half of them were unilateral lesion. Vascular reconstruction was employed for the approximately 60 % patients. The prognosis did not change significantly. Conclusion: Clinical features of quasi-MMD were revealed in the nationwide study. In quasi-MMD, unilateral lesion is dominant, and the development of moyamoya vessels and intracranial hemorrhage are less dominant

Impact of Clipping versus Coiling on Postoperative Hemodynamics and Pulmonary Edema after Subarachnoid Hemorrhage

Volume management is critical for assessment of cerebral vasospasm after aneurysmal subarachnoid hemorrhage (SAH). This multicenter prospective cohort study compared the impact of surgical clipping versus endovascular coiling on postoperative hemodynamics and pulmonary edema in patients with SAH. Hemodynamic parameters were measured for 14 days using a transpulmonary thermodilution system. The study included 202 patients, including 160 who underwent clipping and 42 who underwent coiling. There were no differences in global ejection fraction (GEF), cardiac index, systemic vascular resistance index, or global end-diastolic volume index between the clipping and coiling groups in the early period. However, extravascular lung water index (EVLWI) and pulmonary vascular permeability index (PVPI) were significantly higher in the clipping group in the vasospasm period. Postoperative C-reactive protein (CRP) level was higher in the clipping group and was significantly correlated with postoperative brain natriuretic peptide level. Multivariate analysis found that PVPI and GEF were independently associated with high EVLWI in the early period, suggesting cardiogenic edema, and that CRP and PVPI, but not GEF, were independently associated with high EVLWI in the vasospasm period, suggesting noncardiogenic edema. In conclusion, clipping affects postoperative CRP level and may thereby increase noncardiogenic pulmonary edema in the vasospasm period. His trial is registered with University Hospital Medical Information Network UMIN000003794

Clinical Features and Long-Term Follow-Up of Quasi-Moyamoya Disease in Children.

Background: Inherited or acquired disorders and conditions may present in conjunction with moyamoya disease. This condition is known as quasi-moyamoya disease. Methods: A retrospective review of 69 moyamoya disease patients treated for the past 20 years identified 7 patients with quasi-moyamoya disease and 5 of them were pediatric patients. Results: The mean age at initial diagnosis was 6.4 years (range 5-9). Associated disorders were: craniosynostosis, dwarfism with coarctation of aorta, Proteus syndrome, and cranial irradiation for brain tumor. Their clinical type included cerebral ischemia in 3 patients, cerebral bleeding with ischemia in 1 and epilepsy in 1. The 3 patients with cerebral ischemia underwent bypass surgery and their ischemia was improved. One patient died of brain tumor recurrence and the activities of daily living in the remaining patients were affected by mental retardation. Conclusions: The clinical course and radiological finding of quasi-moyamoya disease are diverse because of associated disorders, distinguishing definite moyamoya disease

Quadruple coaxial catheter system on transvenous embolization for dural arteriovenous fistula

Background: Although transvenous embolization (TVE) is an effective method for treating dural arteriovenous fistula (AVF), directing the catheter to the lesion site is difficult.Objective: We report on the utility of a quadruple coaxial catheter system for TVE.Materials and methods: The quadruple catheter system comprised a 6 Fr guiding sheath, 6 Fr guiding catheter, 4 Fr intermediate catheter, and a regular microcatheter. The system was utilized in 27 consecutive dural AVF cases treated with TVE. In this study, we reviewed our experience with this system, including the theory, method of use, and complications.Results: Stenosis or obstruction of the vascular access was identified in 12 cases. The catheter could not reach to the lesion in three cases of cavernous sinus (7.4%); therefore, transarterial embolization was employed. Angiographic results revealed that the cases consist of total occlusion (n = 16, 59.5%), subtotal (n = 10, 37.0%), and partial occlusion (n = 1, 3.7%). Complete resolution or improvement of symptoms was observed in 23 patients (85.2%), no improvement of symptoms was observed in three patients (7.4%), and deterioration of symptoms was observed in one patient (3.7%). Venous perforation occurred in one patient without any neurological deficit. The catheter system provided access to the lesion and provided stability during the mechanically demanding process navigating the catheter and placing the coils.Conclusion: We determined that the quadruple coaxial system was safe and efficient for TVE for dural AVF

Three-dimensional 4K resolution video microscope in an orbitozygomatic approach for skull base tumor

Background: The authors propose that the newly developed three-dimensional 4K resolution (3D-4K) video microscope, Orbeye™, can be a user-friendly alternative tool for performing orbitozygomatic craniotomy and tumor removal. It was officially approved in Japan in October 2017.Case description: A 38-year-old, otherwise healthy, woman presented with left impaired visual acuity, motor aphasia, headache, and vomiting. Magnetic resonance imaging (MRI) revealed the presence of a large left sphenoid ridge meningioma with marked perifocal edema and mass effect. Using Orbeye™, en bloc orbitozygomatic craniotomy and skull base tumor removal were safely performed. It enabled us to perform the procedure, and to share the operative image owing to the realistic 3D perception of the operative field, its excellent illumination, and viewing angle. Especially, the lower viewing angle appeared to be extremely difficult to obtain using conventional methods. The patient resumed her daily life, and a postoperative MRI showed total removal of the tumor.Conclusions: Orbeye™ has overcome shortcomings of the operative microscope. It has a user-friendly design, and surgeons’ intraoperative fatigue and stress appear to be decreased. It is useful for observers to understand the skull base technical nuances using the 3D-4K image

Extremely Rare Breakdown of the Moyamoya Vessels Resulting in Intraventricular Hemorrhage after Direct Bypass Surgery in a Pediatric Patient with Moyamoya Disease

Revascularization surgery is established for both ischemic and hemorrhagic moyamoya disease (MMD), although hemorrhagic complication is a serious problem especially in adult MMD patients showing postoperative hyperperfusion. Herein, we present an extremely rare case with pediatric MMD showing intraventricular hemorrhage the day following direct bypass surgery, possibly due to a breakdown of the terminal branch of well-developed perforators working as moyamoya vessels. Clinicians should consider this rare complication after bypass surgery for MMD regardless of preoperative hemodynamics or patient age

Efficacy of DynaCT digital angiography in the detection of the fistulous point of dural arteriovenous fistulas.

BACKGROUND AND PURPOSE: Identifying the precise hemodynamic features, including the fistulous point, is essential for treatments of dural arteriovenous fistulas (DAVFs). This study illustrates the efficacy of DynaCT digital angiograms obtained from a 3D C-arm CT to directly visualize the location of the fistulous points in DAVFs. MATERIALS AND METHODS: This retrospective study observed 14 consecutive patients with DAVFs, which included 7 cavernous sinuses, 4 transverse-sigmoid sinuses, 2 convexity-superior sagittal sinuses, and 1 tentorial sinus. In the assessment of the practical applicability for the diagnosis of DAVFs, images obtained from 2D digital subtraction angiography (DSA) and DynaCT were comparatively evaluated. RESULTS: In all patients, DynaCT digital angiography could clearly demonstrate the feeding arteries, the fistulous points, and the draining veins. Significant anatomic landmarks for the fistulous points with relationships to osseous structures were also provided. Compared with 2D DSA, DynaCT digital angiograms demonstrated 12 additional findings in 8 patients (57%), including the detection of the fistulous points (n = 7), the feeders (n = 1), the retrograde leptomeningeal drainage (n = 1), the draining veins (n = 1), and the venous anomaly (n = 2). CONCLUSIONS: In comparison with 2D DSA, DynaCT may provide more detailed information to evaluate DAVFs. DynaCT digital angiograms have a high contrast and isotropic spatial resolution, allowing a reliable visualization of small vessels and fine osseous structures. Such detailed information, especially for the location of the fistulous points, could be very useful for either the endovascular or the surgical treatments of DAVFs

Long-term follow-up of endovascular coil embolization for cerebral aneurysms using three-dimensional time-of-flight magnetic resonance angiography.

OBJECTIVES: As endovascular treatment becomes more prevalent, aneurysm recurrence from neck remnants, recanalization, incomplete obliteration and bleeding remain major concerns. In the current analysis, we attempted to identify factors related to disease progression and clinical outcome in patients treated with coil embolization. METHODS: This study included 58 patients who underwent endovascular coil embolization for treatment of intracranial aneurysm. The result of embolization was evaluated with three-dimensional time-of-flight magnetic resonance angiography (TOF MRA) and classified as a complete occlusion, a residual neck (minor, central and marginal types), a residual dome (central and marginal types). Patients were followed up clinically and radiologically. Statistical analyses were performed to establish factors that influenced the occurrence of adverse events such as recurrence of aneurysm. RESULTS: Overall, the complete occlusion rate was 18.8%, the occurrence of a residual neck was 67.2%, and the residual dome rate was 14.1%. The mean clinical follow-up was 31.2 months. Recurrences were found in 18 aneurysms, and major recurrences were retreated with coiling or surgery. The post-treatment study revealed that the marginal-type aneurysm filling has a significant impact on outcome. Thus, perianeurysmal edema was correlated with recurrence of the aneurysm. CONCLUSIONS: Three-dimensional TOF MRA was a sensitive tool for visualizing residual filling of embolized aneurysm and is useful for long-term follow-up of patients

Recurrence of the cavernous sinus dural arteriovenous fistula at adjacent sinuses following repeated transvenous embolizations: case report and literature review.

We present a unique case of a cavernous sinus (CS) dural arteriovenous fistula (DAVF), which recurred at adjacent sinuses following repeated transvenous embolizations (TVEs). A 68-year-old woman presented with progressive left conjunctival chemosis and diplopia. Cerebral angiography revealed a left CS DAVF, which was completely obliterated by TVE via the left inferior petrosal sinus (IPS). Two years later, the DAVF recurred in the left IPS, and again in the left sigmoid sinus (SS) 3 years after the initial treatment in spite of a second TVE. Moreover, the left SS and the left internal jugular vein, which had been previously stenotic, had been occluded. The third TVE resulted in the complete obliteration of the SS DAVF. CS DAVFs may recur at adjacent sinuses even after complete obliteration by TVE. Careful follow-up is necessary to check for the recurrence of DAVFs, especially in cases with venous flow changes, such as sinus occlusion, following endovascular treatment.The original publication is available at www.springerlink.co

Human neural stem cells enhance structural plasticity and axonal transport in the ischaemic brain

Stem cell transplantation promises new hope for the treatment of stroke although significant questions remain about how the grafted cells elicit their effects. One hypothesis is that transplanted stem cells enhance endogenous repair mechanisms activated after cerebral ischaemia. Recognizing that bilateral reorganization of surviving circuits is associated with recovery after stroke, we investigated the ability of transplanted human neural progenitor cells to enhance this structural plasticity. Our results show the first evidence that human neural progenitor cell treatment can significantly increase dendritic plasticity in both the ipsi- and contralesional cortex and this coincides with stem cell-induced functional recovery. Moreover, stem cell-grafted rats demonstrated increased corticocortical, corticostriatal, corticothalamic and corticospinal axonal rewiring from the contralesional side; with the transcallosal and corticospinal axonal sprouting correlating with functional recovery. Furthermore, we demonstrate that axonal transport, which is critical for both proper axonal function and axonal sprouting, is inhibited by stroke and that this is rescued by the stem cell treatment, thus identifying another novel potential mechanism of action of transplanted cells. Finally, we established in vitro co-culture assays in which these stem cells mimicked the effects observed in vivo. Through immunodepletion studies, we identified vascular endothelial growth factor, thrombospondins 1 and 2, and slit as mediators partially responsible for stem cell-induced effects on dendritic sprouting, axonal plasticity and axonal transport in vitro. Thus, we postulate that human neural progenitor cells aid recovery after stroke through secretion of factors that enhance brain repair and plasticity