Data for training and testing radiation detection algorithms in an urban environment.

The detection, identification, and localization of illicit nuclear materials in urban environments is of utmost importance for national security. Most often, the process of performing these operations consists of a team of trained individuals equipped with radiation detection devices that have built-in algorithms to alert the user to the presence nuclear material and, if possible, to identify the type of nuclear material present. To encourage the development of new detection, radioisotope identification, and source localization algorithms, a dataset consisting of realistic Monte Carlo-simulated radiation detection data from a 2 in. × 4 in. × 16 in. NaI(Tl) scintillation detector moving through a simulated urban environment based on Knoxville, Tennessee, was developed and made public in the form of a Topcoder competition. The methodology used to create this dataset has been verified using experimental data collected at the Fort Indiantown Gap National Guard facility. Realistic signals from special nuclear material and industrial and medical sources are included in the data for developing and testing algorithms in a dynamic real-world background.

ACTA2 Cerebral Arteriopathy: Not Just a Puff of Smoke.

BACKGROUND: Missense mutations in the gene that codes for smooth muscle actin, ACTA2, cause diffuse smooth muscle dysfunction and a distinct cerebral arteriopathy collectively known as multisystemic smooth muscle dysfunction syndrome (MSMDS). Until recently, ACTA2 cerebral arteriopathy was considered to be a variant of moyamoya disease. However, recent basic science and clinical data have demonstrated that the cerebral arteriopathy caused by mutant ACTA2 exhibits genetic loci, histopathology, neurological sequelae, and radiographic findings unique from moyamoya disease. We conducted a literature review to provide insight into the history, clinical significance, and neurosurgical management of this recently described novel cerebral arteriopathy. SUMMARY: We performed a literature search using PubMed with the key words "ACTA2 mutation," "ACTA2 cerebral arteriopathy," and "multisystemic smooth muscle dysfunction syndrome." Case reports with confirmed ACTA2 mutations and cerebral arteriopathy were included in our review. Our literature search revealed 15 articles (58 cases) of confirmed ACTA2 cerebral arteriopathy. Distinctive features of this arteriopathy included an aberrant internal carotid circulation with dilatation of the proximal segments, occlusive disease at the distal segments, and dolichoectasia. As such, mutant ACTA2 predisposed patients to ischemic strokes as children. Direct and indirect cerebral revascularization procedures are the mainstay treatment options with varying degrees of success. Key Messages: ACTA2 cerebral arteriopathy is a recently described novel cerebrovascular disease seen in patients with MSMDS. Patients currently diagnosed with moyamoya disease who also have dysfunction of smooth muscle organs may benefit from reevaluation by a medical geneticist and ACTA2 genotyping.

Predictors of intracranial hemorrhage volume and distribution in brain arteriovenous malformation.

Background and purpose Despite evidence regarding risk factors for brain arteriovenous malformation (bAVM)-associated spontaneous intracranial hemorrhage (ICH), few data exist describing the spectrum of clinical outcomes that bAVM-associated ICH may manifest. This study aimed to identify the demographical, clinical, and bAVM anatomical variables associated with ICH volume and the presence of intraventricular hemorrhage (IVH) of ruptured bAVMs, two indicators of worse clinical outcome, to help better predict outcome for unruptured bAVMs. Methods Computed tomography images ( n = 169) of patients with ruptured bAVM in a prospectively maintained institutional database were retrospectively reviewed to calculate ICH volume and the presence or absence of IVH. Demographic, clinical, and bAVM characteristics information was summarized and analyzed with univariable and multivariable regression models to identify the associations of these features with ICH volume and the presence of IVH. Results Patient sex, exclusively deep venous drainage, and lobar location were associated with ICH volume in univariable analysis; exclusively deep venous drainage remained significant in multivariable analysis (PI = 0.33, 95% CI: 0.21-0.52, p < 0.001). Exclusively deep venous drainage, multiple feeding arteries, and venous stenosis were associated with IVH in univariable analysis; exclusively deep venous drainage (OR = 7.27, 95% CI: 1.94-27.29, p = 0.003) remained significant in multivariable analysis. Conclusions Variables associated with ICH volume and the presence of IVH in ruptured bAVMs were evaluated and identified. They impart information that may help predict the clinical outcome of unruptured bAVM, in turn aiding clinicians in treatment planning.

Tips and tricks to optimize MRI protocols for cutaneous vascular anomalies.

Magnetic resonance imaging (MRI) serves as the principal imaging modality to diagnose and plan treatment for children with cutaneous vascular anomalies. While routine MRI protocols can lead to the correct diagnosis in the majority of cases, the imaging appearances can sometimes be nonspecific or confusing, yielding more than one possible diagnosis. This review highlights specific MRI sequence recommendations and scanning tips that can optimize the imaging protocol to increase diagnostic confidence.

Onyx embolization of an intraosseous pseudoaneurysm of the middle meningeal artery in a patient with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome.

A 13-year-old boy with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome presented with an enlarging "lump" on his right forehead. A head CT scan revealed a polyostotic fibrous dysplasia involving the entire skull. A 3.4-cm right frontal osseous cavity and an overlying right forehead subcutaneous soft-tissue mass were seen, measuring 5.2 cm in diameter and 1.6 cm thick. Ultrasound of the cavity and overlying mass showed swirling of blood and an arterialized waveform. MRI revealed an en plaque meningioma underlying the cavity. An intraosseous pseudoaneurysm fed by 3 distal anterior division branches of the right middle meningeal artery (MMA) with contrast extravasation was found on angiography. Two MMA feeders were embolized with Onyx, with anterograde filling of the intraosseous cavity with Onyx. A small pocket of residual intracavity contrast filling postembolization from a smaller third MMA feeder eventually thrombosed and the forehead lump regressed.

Initial experience with upfront arterial and perfusion imaging among ischemic stroke patients presenting within the 4.5-hour time window.

BACKGROUND: Although perfusion imaging is being evaluated as a tool to select acute ischemic stroke patients who are most likely to benefit from reperfusion therapies beyond the standard time windows, there are limited data on the utility of perfusion imaging within the intravenous (IV) thrombolytic time window. METHODS: A new stroke imaging protocol was initiated at Emory University Hospital including computed tomographic angiography (CTA) and computed tomographic perfusion (CTP). All patients presenting within 4.5 hours from last known normal time with suspected stroke were prospectively identified. Impact of CTA and CTP on the clinical management was recorded prospectively by stroke team members. RESULTS: During the study period, 87 patients met eligibility criteria for the CTA/CTP protocol, of which 83 (95%) underwent this upfront comprehensive imaging protocol and 30 (34%) received IV thrombolytics. Overall, stroke team members reported that CTA and/or CTP aided their clinical management in 39 (47%) cases, including aiding in identification of a nonstroke diagnosis (n = 18), triage to the neurologic intensive care unit (n = 9), early triage to endovascular therapy (n = 4), and initiation of IV thrombolytic for low National Institutes of Health Stroke Scale score with large vessel occlusion (n = 3). Door to needle time ≤60 minutes was achieved in only 18% of patients receiving IV thrombolysis during the study period, but had improved to 44% in the subsequent 6-month period. CONCLUSIONS: An upfront CTA/CTP protocol aided stroke team decision-making in nearly half of cases. Implementation of a CTA/CTP protocol was associated with a learning curve of 6 months before door to needle time ≤60 minutes returned to similar rates as the pre-CTA/CTP protocol.

Tissue-type plasminogen activator mediates neuronal detection and adaptation to metabolic stress.

Adenosine monophosphate-activated protein kinase (AMPK) is an energy sensor that regulates cellular adaptation to metabolic stress. Tissue-type plasminogen activator (tPA) is a serine proteinase found in the intravascular space, where its main role is as thrombolytic enzyme, and in neurons, where its function is less well understood. Here, we report that glucose deprivation induces the mobilization and package of neuronal tPA into presynaptic vesicles. Mass spectrometry and immunohistochemical studies show that the release of this tPA in the synaptic space induces AMPK activation in the postsynaptic terminal, and an AMPK-mediated increase in neuronal uptake of glucose and neuronal adenosine 5'(tetrahydrogen triphosphate; ATP) synthesis. This effect is independent of tPA's proteolytic properties, and instead requires the presence of functional N-methyl-D-aspartate receptors (NMDARs). In agreement with these observations, positron emission tomography (PET) studies and biochemical analysis with synaptoneurosomes indicate that the intravenous administration of recombinant tPA (rtPA) after transient middle cerebral artery occlusion (tMCAO) induces AMPK activation in the synaptic space and NMDAR-mediated glucose uptake in the ischemic brain. These data indicate that the release of neuronal tPA or treatment with rtPA activate a cell signaling pathway in the synaptic space that promotes the detection and adaptation to metabolic stress.

Tissue-type plasminogen activator regulates the neuronal uptake of glucose in the ischemic brain.

The ability to sense and adapt to hypoxic conditions plays a pivotal role in neuronal survival. Hypoxia induces the release of tissue-type plasminogen activator (tPA) from cerebral cortical neurons. We found that the release of neuronal tPA or treatment with recombinant tPA promotes cell survival in cerebral cortical neurons previously exposed to hypoxic conditions in vitro or experimental cerebral ischemia in vivo. Our studies using liquid chromatography and tandem mass spectrometry revealed that tPA activates the mammalian target of rapamycin (mTOR) pathway, which adapts cellular processes to the availability of energy and metabolic resources. We found that mTOR activation leads to accumulation of the hypoxia-inducible factor-1α (HIF-1α) and induction and recruitment to the cell membrane of the HIF-1α-regulated neuronal transporter of glucose GLUT3. Accordingly, in vivo positron emission tomography studies with 18-fluorodeoxyglucose in mice overexpressing tPA in neurons show that neuronal tPA induces the uptake of glucose in the ischemic brain and that this effect is associated with a decrease in the volume of the ischemic lesion and improved neurological outcome following the induction of ischemic stroke. Our data indicate that tPA activates a cell signaling pathway that allows neurons to sense and adapt to oxygen and glucose deprivation.