Pediatric inflammatory bowel disease (PIBD) is a multisystem disorder characterized by intestinal and extraintestinal manifestations and complications. Cerebrovascular events (CVE) are rare extraintestinal complications in patients with PIBD. Statistics show that 3.3% patients with PIBD and 1.3-6.4% adult patients with inflammatory bowel disease (IBD) experience CVE during the course of the disease. Therefore, this study aimed to review the records of children with IBD who developed CVE during the course of the disease. We retrospectively reviewed 62 cases of PIBD complicated by CVE. The mean patient age at the time of thrombotic events was 12.48±4.13 years. The incidence of ulcerative colitis was significantly higher than that of Crohn\'s disease (43 [70.5%] vs. 13 [21.3%] patients). Most patients (87.93%) were in the active phase of IBD at the time of CVE. The mean time interval between the onset of IBD and CVE was 20.84 weeks. Overall, 11 (26.83%) patients showed neurological symptoms of CVE at disease onset. The most frequent symptom on admission was persistent and severe headaches (67.85%). The most common site of cerebral venous thrombosis was the transverse sinuses (n=23, 53.48%). The right middle cerebral artery (n=3, 33.34%) was the predominant site of cerebral arterial infarction. Overall, 41 (69.49%) patients who were mostly administered unfractionated heparin or low-molecular-weight heparin (56.09%) recovered completely. Patients with IBD are at a risk of thromboembolism. CVE may be the most common type of thromboembolism. Based on these findings, the most common risk factor for CVE is IBD flares. In patients with CVE, anticoagulant therapy with heparin, followed by warfarin, is necessary.

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.
A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

As human life expectancy increases, there is an increased prevalence of neurodegenerative disorders and dementia. There are many ongoing research trials for early diagnosis and management of dementia, and neuroimaging is a critical part of such studies. However, conventional neuroimaging often fails to provide enough diagnostic findings in patients with neurodegenerative disorders. In this context, different MRI sequences are currently under investigation to facilitate the accurate diagnosis of such disorders. Susceptibility-weighted imaging (SWI) is an innovative MRI technique that utilizes \"magnitude\" and \"phase\" images to produce an image contrast that is sensitive for the detection of susceptibility differences of the tissues. As many neurodegenerative disorders are associated with accelerated iron deposition and/or microhemorrhages in different parts of the brain, SWI can be applied to detect these diagnostic clues. For instance, in cerebral amyloid angiopathy, SWI can demonstrate cortical microhemorrhages, which are predominantly in the frontal and parietal regions. Or in Parkinson disease, abnormal swallow-tail sign on high-resolution SWI is highly diagnostic. Also, SWI is a useful sequence to detect the low signal intensity of precentral cortices in patients with amyotrophic lateral sclerosis. Being familiar with SWI findings in neurodegenerative disorders is critical for an accurate diagnosis. In this paper, the authors review the technical parameters of SWI, physiologic, and pathologic iron deposition in the brain, and the role of SWI in the evaluation of neurodegenerative disorders in daily practice.

Morning glory disc anomaly (MGDA) characterizes a congenital dysgenetic disorder of the optic disc, coexisting with arterial intracranial abnormalities, including Moyamoya vascular disease, a significantly rare disease in the European populations. We report a 2.5-year-old female child from Greece previously diagnosed with MGDA, who presented with right-hand paresis, accompanied by focal epileptic spasms, followed by an episode of brief absence seizure, as well as some arm clonic spasms. Magnetic resonance angiography scan revealed the presence of an anomalous origin of the anterior cerebral artery (ACA) from the internal carotid artery (ICA) along with vascular abnormalities, compatible with Moyamoya pattern. To the very best of our knowledge, this is the first reported case of anomalous origin of ACA from the supraclinoid ICA accompanied by severe occlusive intracranial disease (moyamoya-like pattern) in a patient with known MGDA, highlighting the embryonic character of the vascular manifestations in MGDA. It also verifies the association of Moyamoya pattern with MGDA, thus linking vascular dysgenesis as a possible cause of MGDA.

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS.

Pulmonary vascular abnormalities are important causes of hemoptysis. Arteriovenous malformation (AVM), pulmonary arterial aneurysms or invasion of the pulmonary arterial structures by the tumor may cause hemoptysis. Pulmonary artery aneurysms (PAA) are an infrequent disease of the pulmonary vasculature. Endovascular coil application is a convenient treatment option for the treatment of hemoptysis due to vascular anomalies. The migration of intravascular coil to another tissue is a rare complication. To review this extremely rare complication, herein we report two unusual cases who had pulmonary artery aneurysm and who had hemoptysis due to tumor invasion to pulmonary artery, initially treated with endovascular coil successfully. In both cases endovascular coil was migrated to the bronchus subsequently. Lobectomy may be performed in such cases with coil migration into the bronchus or conservative therapy with follow-up chest imaging may be a suitable treatment option for selected patients. The choice of treatment should be made individually for each patient considering the characteristics of the patients. In patients with coils, the biopsy can lead to massive hemorrhages that are fatal.

Cervical artery dissection (CAD) is a leading cause of ischaemic stroke (IS) in young and middle-aged adults. Despite well characterized clinical presentation, the diagnosis of CAD can be quite challenging due to a wide variety of symptoms ranging from minor neck pain to severe neurological symptoms. Invasive diagnostic procedures such as DSA are nowadays being replaced by the sensitive and CAD-specific sequences of MR. The most recent studies confirmed the overall efficacy of antiplatelet and anticoagulant therapies for CAD patients is equivalent, although patients should be qualified for concrete treatment on the basis of recently characterized clinical features. The use of NOAC in CAD-related IS prevention cannot yet be recommended due to the lack of evidences from randomized controlled trials. Endovascular therapies should be considered as the treatment of CAD, especially in the cases of large occlusion or antithrombotic treatment failure. Further research is needed to evaluate the efficacy of new imaging modalities and treatment options. This review summarize the last 5-year development of the diagnosis and treatment for CAD as a causative factor for IS.

The updated American Heart Association/American Stroke Association guidelines include recommendation for thrombectomy in certain adult stroke cases. The safety and efficacy of thrombectomy in children are unknown. An 8-year-old girl experienced acute stroke symptoms on two occasions while therapeutically anticoagulated on Novalung. Computed tomography scans showed proximal vessel thrombi, which were retrieved using a Trevo device without hemorrhagic complications. Postprocedural assessment found respective decreases in the National Institutes of Health Stroke Scale score from 10 to 4 and 12 to 7. The indications for treatment and early benefits observed in our case are consistent with other pediatric thrombectomy cases reported. However, publication bias and the heterogeneity of reported cases prevent drawing conclusions about the safety and efficacy of thrombectomy in children. Anticipating that updates to adult stroke guidelines would likely incite stroke providers to consider thrombectomy in children, our institution developed guidelines for thrombectomy before the index patient. Establishing institutional guidelines before considering thrombectomy in children may optimize patient safety.

We describe two cases of non-aneurysmal subarachnoid hemorrhage (SAH) and multifocal stenosis of the intracranial arteries. The patients\' histories together with magnetic resonance angiography, vessel wall imaging and transcranial Doppler (TCD) indicated that the SAH was due to vasculitis or reversible cerebral vasoconstriction syndrome (RCVS). Differential diagnosis of vasculitis and RCVS is important because the treatment strategies are different: immunosuppressants in vasculitis and calcium channel blockers in RCVS. Vessel wall magnetic resonance imaging and TCD can be helpful in differentiating them.

BACKGROUND: Ruptured intracranial dissecting aneurysms must be secured quickly to prevent re-hemorrhage. Endovascular sacrifice of the diseased segment is a well-established treatment method, however postoperative outcomes of symptomatic stroke and re-hemorrhage rates are not well reported, particularly for the perforator-rich distal vertebral artery or proximal posterior inferior cerebellar artery (PICA).
METHODS: We retrospectively reviewed cases of ruptured distal vertebral artery or PICA dissecting aneurysms that underwent endovascular treatment. Diagnosis was based on the presence of subarachnoid hemorrhage on initial CT imaging and of a dissecting aneurysm on catheter angiography. Patients with vertebral artery aneurysms were selected for coil embolization of the diseased arterial segment based on the adequacy of flow to the basilar artery from the contralateral vertebral artery. Patients with PICA aneurysms were generally treated only if they were poor surgical candidates. Outcomes included symptomatic and asymptomatic procedure-related cerebral infarction, recurrent aneurysm rupture, angiographic aneurysm recurrence, and estimated modified Rankin Scale (mRS).
RESULTS: During the study period, 12 patients with dissecting aneurysms involving the distal vertebral artery (n=10) or PICA (n=2) were treated with endovascular sacrifice. Two patients suffered an ischemic infarction, one of whom was symptomatic (8.3%). One patient (8.3%) died prior to hospital discharge. No aneurysm recurrence was identified on follow-up imaging. Ten patients (83%) made a good recovery (mRS ≤2). Median clinical and imaging follow-up periods were 41.7 months (range 0-126.4 months) and 14.3 months (range 0.03-88.6 months), respectively.
CONCLUSIONS: In patients with good collateral circulation, endovascular sacrifice may be the preferred treatment for acutely ruptured dissecting aneurysms involving the distal vertebral artery.