Subarachnoid iodine leakage on dual-energy computed tomography after mechanical thrombectomy is associated with malignant brain edema.

BACKGROUND: Dual-energy computed tomography (DE-CT) can differentiate between hemorrhage and iodine contrast medium leakage following mechanical thrombectomy (MT) for acute ischemic stroke (AIS). We determined whether subarachnoid hemorrhage (SAH) and subarachnoid iodine leakage (SAIL) on DE-CT following MT were associated with malignant brain edema (MBE). METHODS: We analyzed the medical records of 81 consecutive anterior circulation AIS patients who underwent MT. SAH or SAIL was diagnosed via DE-CT performed immediately after MT. We compared the procedural data, infarct volumes, MBE, and modified Rankin scale 0-2 at 90 days between patients with and without SAH and between patients with and without SAIL. Furthermore, we evaluated the association between patient characteristics and MBE. RESULTS: A total of 20 (25%) patients had SAH and 51 (63%) had SAIL. No difference in diffusion-weighted imaging (DWI)-infarct volume before MT was observed between patients with and without SAH or patients with and without SAIL. However, patients with SAIL had larger DWI-infarct volumes 1 day following MT than patients without SAIL (95 mL vs 29 mL; p=0.003). MBE occurred in 12 of 81 patients (15%); more patients with SAIL had MBE than patients without SAIL (22% vs 3%; p=0.027). Severe SAIL was significantly associated with MBE (OR, 12.5; 95% CI, 1.20-131; p=0.006), whereas SAH was not associated with MBE. CONCLUSION: This study demonstrated that SAIL on DE-CT immediately after MT was associated with infarct volume expansion and MBE.

Three-Dimensional (3D) Microcatheter Shaping Using Touch Screen Devices for Cerebral Aneurysm Coil Embolization.

OBJECTIVE: In cerebral aneurysm coil embolization, proper microcatheter shaping is crucial to reduce complications and achieve sufficient embolization. Shaping a microcatheter in 3 dimensions (3D) is often required but can be challenging. We assessed the usefulness of a novel shaping on screen (SOS) method that displays real-size 3D rotational angiography (RA) images on a touch screen device during cerebral aneurysm embolization to facilitate 3D microcatheter shaping. METHODS: In this study, 18 patients with cerebral aneurysm treated with this technique were included. Real-size 3D-RA images obtained during the embolization procedure were displayed on the touch screen device, which allowed for real-time manipulation. The shape of the microcatheter was adjusted to conform to the curvature of the vessel by swiping the touch screen device and bending the mandrel accordingly. We assessed the clinical and angiographic results, along with the accuracy and stability of the microcatheter. RESULTS: No procedure-related complications were observed. The mean packing density was 41% ± 12%. In all but 1 case, microcatheters were inserted into the aneurysms without guidewire assistance. After coiling, all microcatheter forms were stable. CONCLUSIONS: Three-dimensional (3D) microcatheter shaping using touch screen devices during cerebral aneurysm coil embolization may be simple and safe and can achieve high packing density of aneurysms.

Pituitary adenoma presenting as isolated oculomotor nerve palsy.

Background: Isolated oculomotor nerve palsy is a relatively uncommon symptom of pituitary adenoma that usually occurs in association with pituitary apoplexy or cavernous sinus (CS) invasion. Case Description: We report two cases of relatively small pituitary adenomas with neither apoplexy nor CS invasion presenting as isolated oculomotor nerve palsy. Both patients presented with gradually worsening diplopia, without headache or visual field defects. Magnetic resonance imaging (MRI) showed a pituitary tumor with no evidence of intratumoral hemorrhage. Computed tomography revealed a lateroposterior extension of the tumor with the erosion of the posterior clinoid process. Constructive interference in steady-state MRI revealed compression of the oculomotor nerve by the tumor at the oculomotor triangle. The patients underwent endoscopic transsphenoidal surgery, and the intraoperative findings showed that the tumors did not invade the CS. The tumors were completely resected, and the oculomotor palsies resolved fully. Conclusion: These cases illustrate the need to consider isolated oculomotor nerve palsy as an initial manifestation of a relatively small pituitary adenoma with neither apoplexy nor CS invasion. Based on the characteristic radiological findings, early surgical treatment is recommended to preserve oculomotor function.

Two differential cavities in syringomyelia of pediatric Chiari I malformation presenting with unilateral foot drop.

INTRODUCTION: Patients with Chiari I malformation (CM1) may have chronic symptoms of syringomyelia, including numbness and weakness of the upper limbs, typically during young adulthood. Acute or subacute presentation of unilateral foot drop has been rarely reported as a first symptom of CM1-associated syringomyelia exclusively in childhood or adolescence. Why these patients do not show any symptoms of the upper limbs although holocord syringomyelia is always observed on magnetic resonance imaging (MRI) is unclear. CASE PRESENTATION: A four-year-old girl presented rapidly with isolated left foot drop. Conventional MRI revealed holocord syringomyelia associated with CM1. Three-dimensional constructive interference in steady state (3D-CISS) imaging further demonstrated that the syringomyelia was comprised of two differential cavities that communicated with each other via a small pore: a centrally positioned upper cavity and a left-deviated lower one. Surgical decompression of the foramen magnum resolved the symptom with radiological improvement of the two cavities. CONCLUSION: In contrast to a centrally enlarged syrinx that is often asymptomatic, a paracentrally extended syrinx usually produces segmental signs related to its levels. Thus, the left foot drop in this case would have been due to the ipsilaterally deviated lower cavity that was distinguished from the central upper cavity by 3D-CISS imaging. Further reports using this imaging technique are needed to verify the hypothetic pathology.

Cervical Cord Compression by Aberrant Vertebral Artery Presenting with Neuralgic Pain: A Case Report.

The vertebral artery (VA) is often affected by anatomical variations; however, it is usually asymptomatic and is found incidentally. Herein, we report a case of cervical cord compression caused by bilateral aberrant VAs. A 65-year-old woman presented with paroxysmal lancinating pain in the neck that later extended to the shoulders and upper arms bilaterally. Magnetic resonance imaging and computed tomographic angiography revealed an aberrant course of both VAs entering the spinal canal between the atlas and axis and compressing the cervical cord at the atlas level. Microvascular decompression was performed with transposition of the VAs, and the pain resolved immediately after the surgery. A certain number of anatomical variants of the distal VA can be explained by size variations and connection of the lateral spinal artery (LSA). Considering that an aberrant VA may correspond to an enlarged LSA, optimal transposition should be performed to prevent neurological complications.

Microsurgical Approach for True Posterior Communicating Artery Aneurysms: Literature Review and Illustrative Case.

True posterior communicating artery (PCoA) aneurysms are rare. Although true PCoA aneurysms have been reported to be located close to the internal carotid artery, at the middle part of PCoA, or close to the posterior cerebral artery; the best surgical approach to treat true PCoA aneurysms in each location remains unclear. We conducted a literature review using data from PubMed. Data on demographics, location, and projecting direction of the aneurysm, surgical approach, and surgical complications were collected. A total of 47 true PCoA aneurysms were included. Twenty-nine aneurysms originated from the proximal portion, 10 from the middle portion, and 6 from the distal portion; there were two giant aneurysms. The ipsilateral pterional approach was used for 37 true PCoA aneurysms (27 in proximal portion, 8 in middle portion, and 2 in distal portion of PCoA). The anterior temporal approach was used for two distal-portion aneurysms and one giant aneurysm. The anterior subtemporal approach was used for one distal-portion aneurysm. The subtemporal approach was used for two middle-portion aneurysms and one giant aneurysm. The contralateral pterional approach was used for two proximal-portion and one distal-portion aneurysms. Although most true PCoA aneurysms can be treated by the pterional approach, other means such as anterior temporal and subtemporal approaches can be applicable for aneurysms in the middle and distal portions of the PCoA or giant aneurysms. Surgeons should select an appropriate approach for each aneurysm while considering the advantages and disadvantages of each technique.

A Tumor Suppressor Gene, N-myc Downstream-Regulated Gene 1 (NDRG1), in Gliomas and Glioblastomas.

The development of potent and selective therapeutic approaches to glioblastoma (GBM) requires the identification of molecular pathways that critically regulate the survival and proliferation of GBM. Glioblastoma stem-like cells (GSCs) possess stem-cell-like properties, self-renewal, and differentiation into multiple neural cell lineages. From a clinical point of view, GSCs have been reported to resist radiation and chemotherapy. GSCs are influenced by the microenvironment, especially the hypoxic condition. N-myc downstream-regulated gene 1 (NDRG1) is a tumor suppressor with the potential to suppress the proliferation, invasion, and migration of cancer cells. Previous studies have reported that deregulated expression of NDRG1 affects tumor growth and clinical outcomes of patients with GBM. This literature review aimed to clarify the critical role of NDRG1 in tumorigenesis and acquirement of resistance for anti-GBM therapies, further to discussing the possibility and efficacy of NDRG1 as a novel target of treatment for GBM. The present review was conducted by searching the PubMed and Scopus databases. The search was conducted in February 2022. We review current knowledge on the regulation and signaling of NDRG1 in neuro-oncology. Finally, the role of NDRG1 in GBM and potential clinical applications are discussed.

Case of de novo cerebral microbleeds in ischemic-type pediatric moyamoya disease.

BACKGROUND: Studies on pediatric patients with moyamoya disease who presented with de novo cerebral microbleeds (CMBs) are extremely rare. CASE DESCRIPTION: Herein, we report a 7-year-old boy with moyamoya disease who had de novo CMBs during treatment. He presented with transient left-side motor weakness and was diagnosed with moyamoya disease. He underwent revascularization surgery on the right cerebral hemisphere. Six months after the surgery, he presented with transient right-side motor weakness and MRA revealed progression of stenosis in the left middle cerebral artery. After another 3 months, three de novo CMBs were identified. He underwent revascularization surgery on the left side. The symptom disappeared completely after surgery and no additional de novo CMBs were identified 1 year after surgery. CONCLUSION: This is the first report on de novo CMBs in pediatric patients. Although the significance of de novo CMBs in pediatric patients is completely unknown, attention should be paid to not only ischemic stroke but also hemorrhagic stroke. Although the short-term course is good in the current case, follow-up period is too short to assess for rebleeding and long-term follow-up is still important. Further, more cases should be collected.

Elimination of the check-valve mechanism of the sacral Tarlov cyst using a rotation flap technique in a pediatric patient: technical note.

OBJECTIVE: Symptomatic Tarlov cysts in children are not sufficiently reported and treatment methods for Tarlov cysts are still controversial. The goal of this manuscript is to introduce a new variation of the surgical technique. METHODS: We performed surgery to eliminate the one-way check valve mechanism of the Tarlov cyst in a 7-year-old female who presented with urinary and fecal incontinence. A relatively large S3 nerve root cyst showed a one-way check valve on computed tomography myelography. The inlet of the check valve was enlarged with rotation flap reconstruction. RESULTS: Two months after surgery, the patient had established normal sphincter control. MRI performed two years later showed that the treated cyst was collapsing, and no recurrence occurred. CONCLUSIONS: Rotation flap enlargement of the check valve inlet is a safe and efficacious option for the treatment of pediatric patients with sacral Tarlov cysts.

A case of pediatric moyamoya disease with severe cerebral vasospasm and delayed cerebral infarction following an intraventricular hemorrhage.

Reports on patients with moyamoya disease presenting cerebral ischemic complications after the onset of intraventricular hemorrhage (IVH) and/or intracerebral hemorrhage (ICH) are limited. Herein, we report a case of a 7-year-old girl with moyamoya disease with severe cerebral vasospasm and delayed cerebral infarction following an IVH. Although the case is rare, the potential for vasospasm-induced cerebral infarction should be carefully considered and thus, intensive treatment should be immediately initiated.

Prenatal clinical manifestations in individuals with COL4A1/2 variants.

BACKGROUND: Variants in the type IV collagen gene (COL4A1/2) cause early-onset cerebrovascular diseases. Most individuals are diagnosed postnatally, and the prenatal features of individuals with COL4A1/2 variants remain unclear. METHODS: We examined COL4A1/2 in 218 individuals with suspected COL4A1/2-related brain defects. Among those arising from COL4A1/2 variants, we focused on individuals showing prenatal abnormal ultrasound findings and validated their prenatal and postnatal clinical features in detail. RESULTS: Pathogenic COL4A1/2 variants were detected in 56 individuals (n=56/218, 25.7%) showing porencephaly (n=29), schizencephaly (n=12) and others (n=15). Thirty-four variants occurred de novo (n=34/56, 60.7%). Foetal information was available in 47 of 56 individuals, 32 of whom (n=32/47, 68.1%) had one or more foetal abnormalities. The median gestational age at the detection of initial prenatal abnormal features was 31 weeks of gestation. Only 14 individuals had specific prenatal findings that were strongly suggestive of features associated with COL4A1/2 variants. Foetal ventriculomegaly was the most common initial feature (n=20/32, 62.5%). Posterior fossa abnormalities, including Dandy-Walker malformation, were observed prenatally in four individuals. Regarding extrabrain features, foetal growth restriction was present in 16 individuals, including eight individuals with comorbid ventriculomegaly. CONCLUSIONS: Prenatal observation of ventriculomegaly with comorbid foetal growth restriction should prompt a thorough ultrasound examination and COL4A1/2 gene testing should be considered when pathogenic variants are strongly suspected.

Hemorrhagic stroke associated with essential thrombocythemia: Case report and literature review.

Hemorrhagic stroke associated with essential thrombocythemia (ET) is very infrequent. Herein, we report a case of a 33-year-old woman with a 2-year history of ET who developed intracerebral and subarachnoid hemorrhage. Angiography demonstrated severe vessel irregularity in the bilateral cerebral arteries. Molecular genetic testing revealed a calreticulin mutation. To our knowledge, hemorrhagic stroke has been reported in only six other patients with ET, and this is the first report of hemorrhagic stroke in an ET patient with a calreticulin mutation. We review the current literature and discuss the possible underlying mechanisms.

BMP4 induces asymmetric cell division in human glioma stem-like cells.

Glioblastoma (GBM) is a malignant tumor with a high recurrence rate and has very poor prognosis in humans. The median survival is still <2 years. Therefore, a new treatment strategy should be established. Recently, this cancer has been thought to be heterogeneous, consisting of cancer stem cells (CSCs) that are self-renewable, multipotent, and treatment resistant. So various strategies targeting glioma stem-like cells (GSCs) have been investigated. This study focused on strategies targeting GSCs through the induction of differentiation using bone morphogenetic protein 4 (BMP4). The expression of CD133, a cancer stem cell marker, under BMP4 treatment in GSCs was examined using flow cytometry, western blotting, and quantitative PCR. Immunofluorescent staining of GSCs was also performed to examine the type of cell division: asymmetric cell division (ACD) or symmetric cell division (SCD). We obtained the following results. The BMP4 treatment caused downregulation of CD133 expression. Moreover, it induced ACD in GSCs. While the ACD ratio was 23% without BMP4 treatment, it was 38% with BMP4 treatment (P=0.004). Furthermore, the tumor sphere assay demonstrated that BMP4 suppresses self-renewal ability. In conclusion, these findings may provide a new perspective on how BMP4 treatment reduces the tumorigenicity of GSCs.

Association between extracranial internal carotid artery tortuosity and thromboembolic complications during coil embolization of anterior circulation ruptured aneurysms.

BACKGROUND: The most frequent neurological complication during coil embolization of a ruptured cerebral aneurysm is a thromboembolic event. The association between the tortuosity of the internal carotid artery (ICA) and thromboembolic events (TEEs) during coil embolization of ruptured cerebral aneurysms remains unclear. The present study aimed to investigate the association between extracranial ICA tortuosity and thromboembolic complications during coil embolization of anterior circulation ruptured aneurysms. METHODS: A cohort of 57 patients with 57 anterior circulation ruptured aneurysms who underwent endovascular embolization at a single institution was retrospectively investigated. Patients were divided into two groups, those who experienced TEEs and those who did not that were compared and analyzed based on patient baseline characteristics, procedural factors, and anatomical factors including those of aneurysms and extracranial ICA tortuosity. The anatomical factors of the aneurysms included maximum dome size, neck width, dome-to-neck ratio, and dome-to-neck aspect ratio. Extracranial ICA angles in the proximal and distal curvature were evaluated as ICA tortuosity. RESULTS: Three of the 57 patients were excluded because of unavailability of data regarding ICA tortuosity; 54 patients were finally evaluated. TEEs occurred in six patients with five anterior cerebral and one internal carotid aneurysms. The extracranial distal ICA angle was significantly larger in patients with TEEs than in those without. Procedural factors and anatomical factors of the aneurysms were not associated with TEEs. CONCLUSIONS: Extracranial ICA tortuosity was significantly associated with an increased incidence of thromboembolic events during endovascular coiling of anterior circulation ruptured aneurysms.

Stabilization of vulnerable carotid plaques with proprotein convertase subtilisin/kexin type 9 inhibitor alirocumab.

Proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors, a novel class of monoclonal antibodies, reduce low-density lipoprotein cholesterol levels and improve outcomes of myocardial infarction and stroke. However, the effects of PCSK9 inhibitors on carotid plaques remain unclear. We describe three patients treated with PCSK9 inhibitor alirocumab for progressive carotid stenosis despite lipid-lowering statin therapy. All three patients had vulnerable plaques on magnetic resonance (MR) plaque imaging. After alirocumab treatment initiation, no patients suffered stroke or adverse events, and the stabilization of the carotid plaques was observed on MR plaque imaging.

Significance of simulated conventional images on dual energy CT after endovascular treatment for ischemic stroke.

BACKGROUND AND PURPOSE: CT scans often reveal post-interventional cerebral hyperdensities (PCHDs) immediately after intra-arterial thrombectomy (IAT) for ischemic stroke. Dual energy CT (DE-CT) can indicate whether PCHDs are caused by hemorrhage or iodinated contrast. Hyperdense lesions, detected on DE-CT with the use of iodinated contrast, could be associated with delayed hemorrhagic transformation and poor outcome. However, the quantitative indicators in DE-CT for predicting delayed hemorrhagic transformation remain unclear. We assessed such indicators for predicting delayed hemorrhagic transformation. MATERIAL AND METHODS: We retrospectively analyzed 52 consecutive acute ischemic stroke patients who underwent IAT. Simulated conventional CT (sCCT) images were obtained immediately after a DE-CT scan. Virtual, unenhanced, non-contrast (VNC) imaging was performed after reconstruction. Hounsfield units (HU) of the infarct areas observed on the sCCT were measured. The association of HU on sCCT with hemorrhage on VNC and delayed parenchymal hemorrhage (PH) was evaluated. RESULTS: The HU of sCCT with hemorrhage on VNC was significantly higher than without it (377.9±385 HU vs 83.5±37.9 HU; P<0.0001). The cut-off index was 80 HU, which displayed 100% sensitivity, 63.8% specificity, 22.3% positive predictive value, and 100% negative predictive value (P=0.0001, area under the curve (AUC)=0.89). The HU with delayed PH was substantially higher than without it (250.8±382.2 HU vs 93.7±64.8 HU; P=0.01). The cut-off index was 78 HU, which showed 100% sensitivity, 61% specificity, 25% positive predictive value, and 100% negative predictive value (P=0.049, AUC=0.76). CONCLUSION: sCCT images on DE-CT are useful for excluding intracerebral hemorrhage and delayed PH.

Extensive spinal epidural immature teratoma in an infant: case report.

Here, the authors present an extremely rare case of an extensive spinal epidural teratoma (SET) in an infant and provide a review of the cases in the literature. In this report, the authors focused on the clinical manifestation and management of extensive SET. A 64-day-old girl presented with severe dyspnea and paraparesis caused by a large thoracic mass. Imaging studies revealed that the mass originated from the epidural space of the thoracic spine and extended from C7 to L1. The tumor extended bilaterally through the intervertebral foramina and formed a large posterior mediastinal mass. The tumor was partially resected via laminotomy after an emergency thoracotomy. The remnant grade I immature teratoma grew rapidly. After a re-laminotomy and bilateral thoracotomy, the residual tumor stopped growing. However, the patient's paraparesis improved very little, and her scoliosis progressed gradually. Therefore, SET should be included in the differential diagnosis when an infant patient with paraparesis of the lower extremities is encountered. Timely diagnosis, aggressive treatment, and close monitoring are of critical importance to successful recovery in such patients.

Possible involvement of pericytes in intraplaque hemorrhage of carotid artery stenosis.

OBJECTIVEIntraplaque hemorrhage (IPH) is most often caused by the rupture of neovessels; however, the factors of intraplaque neovessel vulnerability remain unclear. In this study, the authors focused on pericytes and aimed to investigate the relationship between IPH and pericytes.METHODSThe authors retrospectively analyzed the medical records of all patients with carotid artery stenoses who had undergone carotid endarterectomy at their hospitals between August 2008 and March 2016. Patients with carotid plaques that could be evaluated histopathologically were eligible for study inclusion. Intraplaque hemorrhage was analyzed using glycophorin A staining, and patients were divided into the following 2 groups based on the extent of granular staining: high IPH (positive staining area > 10%) and low IPH (positive staining area ≤ 10%). In addition, intraplaque neovessels were immunohistochemically evaluated using antibodies to CD34 as an endothelial cell marker or antibodies to NG2 and CD146 as pericyte markers. The relationship between IPH and pathology for intraplaque neovessels was investigated.RESULTSSeventy of 126 consecutive carotid stenoses were excluded due to the lack of a specimen for histopathological evaluation; therefore, 53 patients with 56 carotid artery stenoses were eligible for study inclusion. Among the 56 stenoses, 37 lesions had high IPH and 19 had low IPH. The number of CD34-positive neovessels was equivalent between the two groups. However, the densities of NG2- and CD146-positive neovessels were significantly lower in the high IPH group than in the low IPH group (5.7 ± 0.5 vs. 17.1 ± 2.4, p < 0.0001; 6.6 ± 0.8 vs. 18.4 ± 2.5, p < 0.0001, respectively).CONCLUSIONSPlaques with high IPH are associated with fewer pericytes in the intraplaque neovessels. This finding may help in the development of novel therapeutic strategies targeting pericytes.

[Surgical Technique for Lumbar Spinal Extraforaminal Schwannoma].

INTRODUCTION: Spinal neurinomas are frequently observed in the cauda equina, particularly within the dura mater. However, extraforaminal schwannomas(EFS), which are neither intradural nor dumbbell type, are relatively rare; hence, different surgical procedures have been employed. Here, we report 12 cases of lumbar EFS with total resection that were safely performed. METHODS AND SUBJECTS: This study was comprised of 12 patients with lumbar EFS(excluding those with neurofibromas)who were previously treated at our hospital. Using the Wiltse paraspinal approach, we inserted the microscope until it reached the tumor surface. After confirming the tumor surface, we inserted a scalpel to make an incision in a portion of the outer capsule. Then, we confirmed that the surfaces of the outer capsule and the tumor within the capsule could be dissected. Next, a thread was attached to the outer capsule and pulled upwards so that there was enough space to perform the required procedures within the capsule. Using a CUSA®, we removed the intracapsular tumor. After its removal, the outer capsule was treated carefully to ensure that the origin nerve was not damaged. RESULTS: In all cases, we successfully performed a total removal of the intracapsular tumor;in the mean postoperative observation period of 8 years, no recurrence was observed. Although temporary paralysis was observed in three patients, this improved over time. CONCLUSION: We report our experience with a surgical technique that ensured the safe removal of lumbar EFS. Satisfactory results were achieved, with no recurrences observed during the mean 8-year postoperative observation period.

Spine and Spinal Cord Injury Associated with a Fracture in Elderly Patients with Ankylosing Spondylitis.

There are few cases of spinal cord injury with ankylosing spondylitis (AS). This study investigated the clinical results of a spinal cord injury with a fracture in elderly patients with AS. Nine patients who had sustained a spinal cord injury with vertebral fractures in ankylosed spines were included in this study. The mean age was 79.3 years; two were male and seven were female. The mechanism of injury, the level of vertebral fractures, clinical methods, the follow-up period, and treatment outcomes were investigated. The mechanism of injury of six cases was a fall and in the others was a slip. The levels of vertebral fractures were a cervical lesion (n = 5), a thoracic lesion (n = 3), and a lumbar lesion (n = 1). Six cases underwent a surgical procedure with posterior fusion and decompression, two cases were treated only with a brace, and one case was treated with a halo vest. The mean follow-up period was 4.3 years. The neurological deficit treatment outcomes were improved or no change in four cases each and one case had died. There was not much difference in treatment outcomes between a surgical treatment and a conservative treatment. Computed tomography imaging to evaluate the entire spine is required in all patients with AS with a possible spinal fracture. A surgical treatment and early rising and rehabilitation should be recommended for patients with fractures and AS to avoid further complications, not from the standpoint of improving the neurological status.