Primary Angiitis Of CNS Research Articles

Myelin oligodendrocyte glycoprotein antibody–associated disease with histopathologic features of primary CNS angiitis without demyelination: Case report and literature review

Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease that affects both small- and medium-sized vessels of the CNS, while myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) is a novel antibody-mediated inflammatory demyelinating disorder that causes damage to the myelin in CNS. We report a case diagnosed as MOGAD due to a history of recurrent myelitis, brain lesions, and positive anti-MOG, but the brain biopsy showed vasculitis without demyelination.

Neurovascular Adverse Effects of Sars-Cov-2 Vaccination.

The global deployment of SARS-CoV-2 vaccines has been pivotal in curbing the COVID-19 pandemic, reducing morbidity and mortality associated with the virus. While most of these vaccines have demonstrated high efficacy and overall safety, emerging reports have highlighted potential neurovascular adverse effects, albeit uncommon, associated with these vaccinations. This review aims to assess and summarize the current knowledge on the neurovascular complications arising post-SARS-CoV-2 vaccination. We conducted an extensive literature review, focusing on clinical studies and case reports to identify reported neurovascular events, such as ischemic stroke, cerebral sinus venous thrombosis, intracerebral hemorrhage, pituitary apoplexy and primary CNS angiitis Despite the relative rarity of these events, their impact on affected individuals underscores the importance of ongoing surveillance, early detection, and management strategies. We aim to provide healthcare professionals with the latest evidence on neurovascular adverse effects, facilitating informed decision-making in the context of SARS-CoV-2 vaccination programs. Furthermore, we highlight areas requiring further research to understand the pathophysiology of these adverse events better and to develop targeted prevention and treatment strategies.

Intravascular CNS Lymphoma: An elusive diagnosis (P2-5.016)

<h3>Objective:</h3> To report a rare case of intravascular CNS lymphoma presenting with subacute neuropsychiatric symptoms and multifocal cerebral infarcts and hemorrhages. <h3>Background:</h3> Intravascular lymphoma is a rare and aggressive subtype of extra-nodal non-Hodgkin lymphoma. It is characterized by proliferation of neoplastic B-lymphocytes within the lumen of small blood vessels, especially capillaries and post-capillary venules. Presentation is variable and depends on the organ dysfunction due to small vessel occlusion. Most common organs involved are CNS, skin and lungs. Given the rarity of the disease, variable presentation and absence of an extravascular tumor mass or peripheral blood abnormalities, the diagnosis can be extremely challenging and is often delayed. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 62-year old healthy woman presented with transient left hemiplegia and left facial numbness and was diagnosed with transient ischemic attack. In one month, she developed dysarthria and left hemibody clonic seizures without any systemic complaints. Over the next four months, she had a step-wise decline and developed severe apathy, decreased verbal fluency, diffuse hypertonia and worsening encephalopathy. Serial brain MRIs over four months showed acute-subacute infarcts and intracerebral hemorrhages involving both anterior and posterior circulation bilaterally with scattered micro-hemorrhages. Extensive work up, including CT-angiogram and PET-CT were unrevealing. CSF was notable for high protein level without pleocytosis and negative infectious and autoimmune workup. DSA was unrevealing. She did not respond to high dose steroids, intravenous immunoglobulin and cyclophosphamide. Brain biopsy revealed CNS intravascular large B-cell lymphoma. Patient was started on chemotherapy with HyperCVAD-R protocol with no improvement. <h3>Conclusions:</h3> Intravascular CNS lymphoma is a challenging diagnosis. It presents as subacute-chronic CNS vasculopathy with multifocal infarcts and hemorrhages and can mimic other CNS vasculopathies, including primary CNS angiitis and amyloid-related angiopathy. Tissue biopsy is the diagnostic gold standard and should be considered early if poor response to immunotherapy is noted. Despite high-dose chemotherapy, prognosis is poor. <b>Disclosure:</b> Dr. Ticku has nothing to disclose. Dr. Serra has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen Idec. Dr. Serra has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Bristol Myers Squibb.

Vasculitis as a cause of stroke

The review considers the clinical picture, key aspects of the diagnosis and treatment of vasculitis that are the causes of strokes (giant cell arteritis, polyarteritis nodosa, varicella zoster virus vasculopathy, cerebrovascular pathology caused by herpes simplex virus types 1 and 2, primary CNS angiitis, adenosine deaminase-2 deficiency).

A Case of Primary CNS Angiitis Presenting as Longitudinally Extensive Transverse Myelitis (P6.228)

A Case of Primary CNS Angiitis Presenting as Longitudinally Extensive Transverse Myelitis (P6.228)

FRI0464 Development of the Classification Criteria for GIANT Cell Arteritis in the Diagnostic and Classification Criteria for Vasculitis Study: A Pilot Study Using A Panel Review Methodology

BackgroundThe Diagnostic and Classification Criteria for Vasculitis (DCVAS) Study is a multinational observational study to develop diagnostic criteria and to update classification criteria for the primary systemic vasculitides. By 2015...

The A's and B's of the ABC's of stroke mechanisms and recurrence in pediatric ischemic stroke

Arterial ischemic stroke in children is rare, estimated at 2 per 100,000 children per year,1 but carries a relatively high risk of both recurrent stroke and lifelong neurologic morbidity.2 Ischemic stroke mechanisms in both adults and children can be conceptually broken down into the “ABC's”: arterial, blood, and cardiac. The mechanism of the stroke is important as therapeutic decisions and recurrent risk can vary by etiology. Arterial mechanisms (vasculopathies) are among the most common identified risk factors in childhood stroke, and differ dramatically from those seen in adults. They are traditionally divided into inflammatory (e.g., vasculitis, infectious) and noninflammatory (e.g., focal cerebral arteriopathy, moyamoya, dissection) processes. Little is known about the underlying pathophysiology of one of the most common of these vasculopathies, an entity referred to as focal cerebral arteriopathy (FCA). In fact, much controversy exists concerning the definitions and diagnostic criteria for FCA, and how or if these disorders fit into the spectrum of primary CNS angiitis in childhood.3 Complicating matters, over half of pediatric arterial ischemic stroke patients have more than one risk factor.4 Novel research strategies have been greatly needed to shed new light on the underlying pathomechanisms of vascular disorders in childhood ischemic stroke.

Brain-reactive β-amyloid antibodies in primary cns angiitis with cerebral amyloid angiopathy

Primary CNS angiitis (PCNSA) is a focal or multifocal vasculitis with predilection for small arteries that is restricted to the brain or spinal cord.1,–,3 Both diffuse lymphocytic and granulomatous inflammatory patterns are noted, which may or may not go along with focal necrosis.2,4,5 About 30% of patients with PCNSA exhibit prominent histopathologic signs of cerebral amyloid angiopathy (CAA).2,4 Compared with other patients, patients with β-amyloid (Aβ)-related angiitis (ABRA) are older and have a more acute disease onset with a high prevalence of gadolinium-enhanced MRI lesions,4 suggesting that CAA predisposes to an exacerbated inflammatory response. Whether ABRA shares the same pathomechanisms as other forms of PCNSA or belong to a different disease entity was unknown. ### Case report. A 67-year-old man presented with psychomotor slowing, memory problems, aphasia, right-sided limb …

P235 Primary CNS angiitis – a rare cause of stroke in children, remains diagnostic and therapeutic challenge

P235 Primary CNS angiitis – a rare cause of stroke in children, remains diagnostic and therapeutic challenge

Primary CNS angiitis presenting as short‐term memory loss: a case report and literature review

AbstractPrimary angiitis of the central nervous system (PACNS) is an idiopathic vasculitis of the small to medium vessels that often eludes diagnosis because of its variable signs and symptoms. A 36‐year‐old woman presented with a 4‐week history of progressive memory loss. Neurological examination revealed only severe cognitive deficits including anterograde amnesia. Magnetic resonance imaging demonstrated multiple ring‐enhancing lesions involving the left frontal lobe and uncus and bilateral thalami. Stereotactic biopsy showed findings consistent with CNS angiitis. Further workup revealed no evidence of systemic disease. We report the first case of biopsy‐proven PACNS presenting as profound isolated anterograde amnesia.