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.

Cerebral Amyloid Angiopathy-Related Inflammation and Biopsy-Positive Primary Angiitis of the CNS: A Comparative Study.

Cerebral amyloid angiopathy-related inflammation (CAA-RI) and biopsy-positive primary angiitis of the CNS (BP-PACNS) have overlapping clinicoradiologic presentations. It is unknown whether clinical and radiologic features can differentiate CAA-RI from BP-PACNS and whether both diseases have different relapse rates. The objectives of this study were to compare clinicoradiologic presentations and relapse rates in patients with CAA-RI vs BP-PACNS. Patients with CAA-RI and BP-PACNS were enrolled from 2 retrospective multicenter cohorts. Patients with CAA-RI were biopsy-positive or met probable clinicoradiologic criteria. Patients with BP-PACNS had histopathologic confirmation of CNS angiitis, with no secondary etiology. A neuroradiologist read brain MRIs, blinded to the diagnosis of CAA-RI or BP-PACNS. Clinicoradiologic features were compared using univariable logistic regression models. Relapse rates were compared using a univariable Fine-Gray subdistribution hazard model, with death as a competing risk. This study enrolled 104 patients with CAA-RI (mean age 73 years, 48% female sex) and 52 patients with BP-PACNS (mean age 45 years, 48% female sex). Patients with CAA-RI more often had white matter hyperintense lesions meeting the probable CAA-RI criteria (93% vs 51%, p < 0.001), acute subarachnoid hemorrhage (15% vs 2%, p = 0.02), cortical superficial siderosis (27% vs 4%, p < 0.001), ≥1 lobar microbleed (94% vs 26%, p < 0.001), past intracerebral hemorrhage (17% vs 4%, p = 0.04), ≥21 visible centrum semiovale perivascular spaces (34% vs 4%, p < 0.01), and leptomeningeal enhancement (70% vs 27%, p < 0.001). Patients with BP-PACNS more often had headaches (56% vs 31%, p < 0.01), motor deficits (56% vs 36%, p = 0.02), and nonischemic parenchymal gadolinium enhancement (82% vs 16%, p < 0.001). The prevalence of acute ischemic lesions was 18% in CAA-RI and 22% in BP-PACNS (p = 0.57). The features with the highest specificity for CAA-RI were acute subarachnoid hemorrhage (98%), cortical superficial siderosis (96%), past intracerebral hemorrhage (96%), and ≥21 visible centrum semiovale perivascular spaces (96%). The probable CAA-RI criteria had a 71% sensitivity (95% CI 44%-90%) and 91% specificity (95% CI 79%-98%) in differentiating biopsy-positive CAA-RI from BP-PACNS. The rate of relapse in the first 2 years after remission was lower in CAA-RI than in BP-PACNS (hazard ratio 0.46, 95% CI 0.22-0.96, p = 0.04). Clinicoradiologic features differed between patients with CAA-RI and those with BP-PACNS. Specific markers for CAA-RI were hemorrhagic signs of subarachnoid involvement, past intracerebral hemorrhage, ≥21 visible centrum semiovale perivascular spaces, and the probable CAA-RI criteria. A biopsy remains necessary for diagnosis in some cases of CAA-RI. The rate of relapse in the first 2 years after disease remission was lower in CAA-RI than in BP-PACNS.

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.

High-resolution vessel wall magnetic resonance imaging in intracranial vasculopathies: an experience from eastern India.

To evaluate the role of high-resolution intracranial vessel wall imaging (HR-IVWI) in differentiation of various intracranial vasculopathies in addition to luminal and clinical imaging in the largest cohort of Indian stroke patients. A single-center, cross-sectional study was undertaken recruiting consecutive stroke or TIA patients presenting within a month of onset, with luminal irregularity/narrowing upstream from the stroke territory. The patients were initially classified into TOAST and Chinese ischemic stroke sub-classification (CISS) on the basis of clinical and luminal characteristics and reclassified again following incorporation of HR-IVWI findings. In our cohort of 150 patients, additional use of HR-IVWI led to a 10.7 and 14% change in initial TOAST and CISS classification respectively (p < 0.001). In TOAST classification, 12 "undetermined aetiology" were reclassified into intracranial atherosclerotic disease (ICAD), 1 "undetermined aetiology" into CNS angiitis and 1 "undetermined aetiology" into arterial dissection. Similarly, in CISS 19 "undetermined aetiology" was reclassified into 16 large artery atherosclerosis (LAA) and 3 "other aetiology" consisting of one CNS angiitis, Moyamoya disease (MMD) and arterial dissection each. Two initial classification of MMD by CISS and TOAST were changed into ICAD. The observed change in diagnosis following incorporation of HR-IVWI was proportionately highest in ICAD (LAA) subgroup (TOAST-9.3%, CISS-12%). Adjunctive use of HR-IVWI, to clinical and luminal assessment, can significantly improve diagnostic accuracy during evaluation of intracranial vasculopathies, with its greatest utility in diagnosing in ICAD, CNS angiitis and dissection. HR-IVWI allows clearer etiological distinction of intracranial vasculopathies having therapeutic and prognostic implications.

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).

LMD-11. Pure spinal cord invasion in recurrent classic Hodgkin’s Lymphoma - what is it?

We present the case of a 39-year-old male who initially presented in January 2017 with abdominal pain and weight loss. He was eventually diagnosed with stage IVB Classical Hodgkin’s Lymphoma. Brentuximab, Vinblastine, and Dacarbazine regimen was initiated with dose adjustments made due to cardiomyopathy and neuropathy. By cycle 5, good response was evident on systemic imaging. He was then lost to follow-up.He represented in December 2020 with near paraplegia, preceded by months of tingling, numbness in both legs, gait imbalance and low back pain. MRI spine in January 2021 demonstrated multifocal abnormal cord signal changes with scattered areas of intramedullary cord enhancement as well as extensive enhancement of the cauda equina (Figures 1,2,3). CNS angiitis as a paraneoplastic phenomenon, mimics a similar clinical and radiographic appearance. Unfortunately none of the cord lesions were amenable to biopsy.Full body PET CT showed florid disease concerning for recurrence. 2 lumbar punctures with limited CSF flow cytometry and cytology analyses were negative. Mediastinal lymph node biopsy revealed Classical Hodgkins Lymphoma. DHAP chemotherapy regimen was initiated, soon after. A 3rd time, high volume comprehensive CSF analysis performed later, was unremarkable. MRI spine, after 2 cycles demonstrated significant improvement(Figures 4,5,6)coinciding with systemic response and clinical improvement. This favored diagnosis of CNS Hodgkin lymphoma over secondary CNS angiitis.CNS Hodgkin’s Lymphoma is a rare occurrence1, let alone, pure spinal intramedullary and leptomeningeal metastases. Intramedullary metastases arise from direct hematogenous spread or by, centripetal growth of tumor along spinal nerve roots with secondary invasion of spinal cord2. Systemic Hodgkin’s Lymphoma rarely affects the spine with a prevalence of 0.2% - 0.5%3,4. In an analysis of a large cohort of Hodgkin’s patients comprising 14,868 individuals, a prevalence of less than 0.02%, indicating it had been overestimated in the past5.

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)

Fatal CNS angiitis due to varicella zoster in post renal transplant recipient

Fatal CNS angiitis due to varicella zoster in post renal transplant recipient

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.

Abstract 3780: MR Imaging Of The Intracranial Vessel Wall In Paediatric Stroke

Background Arteriopathy in childhood arterial ischaemic stroke (AIS) is common and is postulated to be a predictor of outcome. The distinction between inflammatory (transient cerebral arteriopathy [TCA], CNS angiitis) and non-inflammatory (arterial dissection, moyamoya) arteriopathies is vital to guide acute management decisions. Currently available non-invasive angiographic imaging techniques are inadequate to reliably distinguish inflammatory from non-inflammatory causes. Conventional catheter angiography, the gold standard, is invasive and not always feasible. Objective: To report the findings of a novel non-invasive arterial wall MR imaging technique in three children with arterial ischemic stroke due to arteriopathy. Method: 3-Tesla T1-weighted 2D FLAIR fast spin echo sequence (slice thickness 3mm, matrix size 512x512) in plane and perpendicular to the vessel of interest were performed in three children with AIS before and after intravenous administration of Gadolinium, in addition to routine sequences. Results: The first child had TCA ( Fig. 1 ), the second had arterial dissection and the third had CNS angiitis (see table ). Conclusion: Our series highlights the utility of a novel non-invasive MR imaging technique of vessel wall as distinct patterns of vessel wall enhancement could be demonstrated in children with TCA, arterial dissection and CNS angiitis. Prospective studies are required to further assess the diagnostic utility of vessel wall enhancement in children with arteriopathy-related AIS. This would also help us understand the pathophysiologic mechanism of this finding. Table 1. y=years; F=female; M=male; dICA=distal internal carotid artery; BA=basilar artery

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.

Cerebral vasculitis

Cerebral vasculitides are infrequent in neurological practice but important for the differential diagnosis of multifocal CNS symptoms. The clinical presentation is typically a combination of chronic headache, multiple strokes and encephalopathy. In systemic vasculitis, a combination of CNS symptoms with inflammatory signs and with peripheral nervous system symptoms (painful multiplex polyneuropathy) or other organ manifestations (skin, joints, kidney, heart, lungs, eye) are frequent. In isolated CNS angiitis, CSF examinations are of particular value. Besides MRI, angiography, CSF and serum examinations, the verification of the diagnosis by biopsy is mandatory.

Multiple cerebral hemorrhages in HTLV‐I‐associated myelopathy

We describe a 66-year-old woman with long-standing HTLV-I-associated myelopathy who developed multiple parenchymal hemorrhages and whose angiogram suggested cerebral vasculitis. After cyclophosphamide and glucocorticoid therapy, both her acute deficits and chronic paraparesis improved. HTLV-I may be an etiologic agent in isolated angiitis of CNS.

Isolated angiitis of CNS: Treatment with pulse cyclophosphamide

A 12-year-old boy with a history of “migraine” headache presented with an increasingly severe headache accompanied by emesis and unsteadiness. Evaluation revealed an acute cerebral hemorrhage with subsequent angiographic studies demonstrating multiple areas of segmental narrowing in both the anterior and posterior cerebral circulations. He was diagnosed with isolated angiitis of the central nervous system and high-dose steroid therapy was administered to which he responded for 6 months. Symptoms recurred and repeat angiography demonstrated persistent segmental narrowing. Pulse cyclophosphamide therapy was begun with resolution of symptoms and normalization of angiography following 6 treatments. Although rare in children, isolated angiitis of the central nervous system can occur in children and aggressive immunosuppression should be considered as the mode of therapy.

Cervical herpes zoster and delayed brainstem infarction

Varicella-zoster (VZ) virus is a rare cause of CNS angiitis, which commonly presents as herpes zoster ophthalmicus with contralateral hemiplegia due to hemispheric infarction. We report the first case of VZ-angiitis with infarction in the ventral pons, following cervical herpes zoster.

Suspected isolated angiitis causing stroke in a child.

HomeStrokeVol. 21, No. 9Suspected isolated angiitis causing stroke in a child. Free AccessAbstractPDF/EPUBAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessAbstractPDF/EPUBSuspected isolated angiitis causing stroke in a child. J LaMancusa and G Steiman J LaMancusaJ LaMancusa Search for more papers by this author and G SteimanG Steiman Search for more papers by this author Originally published1 Sep 1990https://doi.org/10.1161/01.STR.21.9.1380Stroke. 1990;21:1380"Suspected isolated angiitis causing stroke in a child.." Stroke, 21(9), p. 1380 Previous Back to top Next FiguresReferencesRelatedDetailsCited By (2010) Inflammatory Angiopathies and Stroke Toole's Cerebrovascular Disorders, 10.1017/CBO9781139644235.013, (143-159) Barron T, Ostrov B, Zimmerman R and Packer R (1993) Isolated angiitis of CNS: Treatment with pulse cyclophosphamide, Pediatric Neurology, 10.1016/0887-8994(93)90017-7, 9:1, (73-75), Online publication date: 1-Jan-1993. September 1990Vol 21, Issue 9 Advertisement Article InformationMetrics Copyright © 1990 by American Heart Associationhttps://doi.org/10.1161/01.STR.21.9.1380 Originally publishedSeptember 1, 1990 PDF download Advertisement