Cerebral vasculopathy

Acute necrotising encephalitis (ANE) is a rare and life-threatening disorder typically associated with viral pathogens triggering an inflammatory response. It is characterised by rapid neurological deterioration linked to a cytokinetic storm which radiologically manifests with cerebral radiological changes. We present a unique case not previously documented of an immunocompetent 23-year-old male who survived the course of ANE, with widespread involvement of the brain including the deep white matter, cortex, superior frontal gyrus, occipital lobe and cerebellum...

Primary testicular diffuse large B-cell lymphoma (PTL) is characterized by high risk of contralateral testis and central nervous system (CNS) relapse. Chemoimmunotherapy with intrathecal (IT) CNS prophylaxis and contralateral testis irradiation eliminates contralateral recurrences and reduces CNS relapses. The IELSG30 phase 2 study investigated feasibility and activity of an intensified IT and IV CNS prophylaxis. Patients with stage I/II PTL who had not received treatment received 2 cycles of IV high-dose methotrexate (MTX) (1...

Acute ischemic stroke is an uncommon presentation in the pediatric population as compared to the elderly population. COVID-19 infection is associated with several neurological manifestations, with ischemic strokes being underrecognized. Cerebrovascular events associated with COVID-19 may be due to systemic inflammation and hypercoagulable state. Neurofibromatosis type 1 (NF1) is an inherited multisystem disorder caused by dominant loss-of-function mutations of the tumor-suppressor gene neurofibromin 1, which is located at 17q11...

BACKGROUND: The co-occurrence of moyamoya vasculopathy and extra-optic pathway tumors is rare in neurofibromatosis type 1 (NF1), with only four cases described in the literature. Brain surgery in these patients may be challenging because of the risk of brain infarction after skin and dural incision. Given its percutaneous and minimally invasive nature, laser interstitial thermal therapy (LITT) is an ideal option for the treatment of brain tumors in these patients. Here, we report on two patients with NF1 and moyamoya syndrome (MMS) treated for a brain glioma with LITT, after cerebral revascularization...

RATIONALE: The ring finger protein 213 (RNF213) p.R4810K variant has been identified as being associated with Moyamoya disease (MMD), a condition that is more prevalent in East Asians. This association extends beyond cerebral vessels and has been implicated in coronary artery disease. PATIENT CONCERNS: A 36-year-old female was admitted to the emergency room with chest pain. Although the patient had no known underlying conditions or risk factors for atherosclerosis, she was diagnosed with unstable angina and underwent percutaneous coronary intervention...

We present a case of a woman in her 30s with relapsing-remitting multiple sclerosis, treated with natalizumab, who developed ophthalmic varicella zoster virus (VZV) infection, with subsequent vasculopathy causing cerebral ischaemic lesions. She was treated with acyclovir, prednisolone and acetylsalicylic acid and fully recovered. VZV vasculopathy is associated with stroke and immunomodulating treatments may increase the risks of these adverse events. To date, nine VZV-related vasculopathy cases in patients treated with natalizumab have been reported in English literature and are summarised in this paper...

Sickle cell disease (SCD) is an autosomal recessive disorder. Although the molecular mechanisms at the origin of SCD have been well characterized, its clinical expression is highly variable. SCD is characterized by blood rheological abnormalities, increased inflammation and oxidative stress, and vascular dysfunction. Individuals with only one copy of the mutated β-globin gene have sickle cell trait (SCT) and are usually asymptomatic. The first part of this review focuses on the biological responses of SCT carriers during exercise and on the effects of combined SCT and diabetes on vascular function, several biomarkers and clinical complications...

Osteopathia striata with cranial sclerosis (OSCS) is a rare X-linked dominant sclerosing osteodysplasia, due to AMER1 pathogenic variants. Characteristic features include craniofacial sclerosis and long-bone metaphyseal striations. Moyamoya disease (a type of progressive cerebral vasculopathy) and other types of cerebral vascular disease are not currently clearly associated with OSCS (except for two separate case reports), and can often first present with stroke. Through informal networks with UK-based bone experts and the UK skeletal dysplasia group, three cases from the UK and Ireland were identified...

INTRODUCTION: Large vessel vasculopathy, such as carotid stenosis, has been shown to be a side effect of radiotherapy (RT) and has received increasing attention especially in recent decades with the improvement of RT technology. These injuries can lead to a higher risk of cerebrovascular events such as ischemic stroke. The management of these lesions may be performed with surgical repair but also with endovascular technique. OBSERVATION: A 61-year-old man was admitted to the emergency department for an acute ischemic stroke...

Cerebral aneurysms secondary to human immunodeficiency virus vasculopathy are a diagnosis by exclusion and its mechanism is unknown. We report on a 21-year-old male with human immunodeficiency virus infection and suboptimal virological control, despite highly active antiretroviral therapy. An incidental discovery of multiple cerebral aneurysms occurred in this patient, who initially presented with signs of disorientation, acute psychosis, and a history of blunt cranial trauma. A non-contrasted computerized tomography scan of the encephalon showed no intracranial hemorrhage but multiple cerebral (saccular and fusiform) aneurysms...

Bacterial meningitis is a serious, life-threatening infection of the meninges. Several radiological studies highlight prominent structural alterations occurring in the cerebral vasculature, leading to significant cerebrovascular consequences during bacterial meningitis. Beginning with reflexive arterial vasospasm , cerebrovascular disease during bacterial meningitis proceeds through a orderly sequence of arterial vasculitis with inflammatory cell infiltration, medial smooth muscle migration and proliferation, medial necrosis, adventitial fibrosis and eventual intimal stenosis...

BACKGROUND: Basal ganglia and thalamic arteriovenous malformations (AVMs) represent a special subset of malformations. Due to the involvement of vital brain structures and the specifically fine and delicate angioarchitecture of these lesions, it presents unique therapeutic challenges and technical difficulties that require thorough treatment planning, individualized treatment strategies, and advanced techniques for good clinical outcome. METHOD: In this study, we presented a series of ruptured basal ganglia and thalamic AVMs embolized via a transarterial, transvenous or combined approach...

BACKGROUND: The current fungal meningitis outbreak caused by contaminated epidural anesthesia with Fusarium solani among patients who underwent surgical procedures in Matamoros, Mexico remains a cause of concern. Its association with an increased susceptibility for cerebrovascular complications (CVC) has not been reported. This single-center study describes 3 patients with a unique pattern of CVC attributed to fungal meningitis. METHODS: A retrospective case series of patients diagnosed with fungal meningitis following surgical procedures under contaminated epidural anesthesia who developed a unique pattern of CVC during their hospitalization...

Migraines being a possible risk factor for spontaneous multivessel cervical artery dissection has been previously introduced but rarely discussed in literature. We present the case of a 32-year-old man with a history of migraines and a 2-week history of bilateral neck pain who was found to have bilateral Vertebral Artery dissection by CT angiography. The patient's stroke's etiology was spontaneous dissection followed by thromboembolism caused by bilateral Posterior Cerebral Artery (P1) occlusion. Due to an inability to protect his airway, he was scheduled to have a tracheostomy and percutaneous endoscopic gastrostomy (PEG)...

Systemic vascular occlusive disease associated with neurofibromatosis type 1 (NF1) has been reported in the aortic, cerebral, renal, celiac, and mesenteric vessels and is referred to as NF1 vasculopathy. Although retinal vascular involvement in patients with NF1 usually manifests as retinal capillary hemangiomatosis, a few cases of NF1 with retinal vascular occlusive disease have also been described. Here, we report a 2-year-old girl with NF1 who presented with branch retinal vein occlusion and peripheral retinal ischemia secondary to NF1...

Sickle cell anemia (SCA) is a genetic disease caused by the homozygosity of the HBB :c.20A>T mutation, which results in the production of hemoglobin S (HbS). In hypoxic conditions, HbS suffers autoxidation and polymerizes inside red blood cells, altering their morphology into a sickle shape, with increased rigidity and fragility. This triggers complex pathophysiological mechanisms, including inflammation, cell adhesion, oxidative stress, and vaso-occlusion, along with metabolic alterations and endocrine complications...

Previous study has suggested that the loss of microvessel density in the peripheral circulation with evolving metabolic disease severity represents a significant contributor to impaired skeletal muscle oxygenation and fatigue-resistance. Based on this and our recent work, we hypothesized that cerebral microvascular rarefaction was initiated from the increased pro-oxidant and pro-inflammatory environment with metabolic disease and is predictive of the severity of the emergence of depressive symptoms in obese Zucker rats (OZR)...

Assessment of cerebral vasculopathies is challenging and requires understanding the utility of different imaging methods. Various techniques are available to image the vessel lumen, each with unique advantages and disadvantages. Bolus-based CT and MR angiography requires careful timing of a contrast bolus to provide optimal luminal enhancement. Non-contrast MRA techniques do not require a contrast agent and can provide images with little venous contamination. Digital subtraction angiography remains the gold standard but is invasive, while VW-MRI provides a non-invasive way of assessing vessel wall pathology...

Childhood cerebral vasculitis is a condition that affects the blood vessels in the brain of children and is rare but life-threatening. Imaging plays a crucial role in the diagnosis and monitoring of the disease. This article describes the classification, diagnostic algorithm, and various imaging modalities used in the evaluation of childhood cerebral vasculitis and the imaging findings associated with primary and secondary vasculitis. Understanding the imaging features of this condition can assist in early diagnosis, effective treatment, and improve outcomes...

Stroke is a complication of many central nervous system (CNS) infections, but only a few present with stroke without other symptoms or signs of CNS infection. Chief among these are varicella zoster virus (VZV) and syphilis. Delayed cerebral vasculopathy after successful treatment of bacterial meningitis, most commonly pneumococcal, is an emerging entity with uncertain pathogenesis.