Angiitis Of Central Nervous System Research Articles

Spontaneous Non-Aneurysmal Convexity Subarachnoid Hemorrhage: A Scoping Review of Different Etiologies beyond Cerebral Amyloid Angiopathy.

Spontaneous convexity subarachnoid hemorrhage (cSAH) is a vascular disease different from aneurysmal SAH in neuroimaging pattern, causes, and prognosis. Several causes might be considered in individual patients, with a limited value of the patient's age for discriminating among these causes. Cerebral amyloid angiopathy (CAA) is the most prevalent cause in people > 60 years, but reversible cerebral vasoconstriction syndrome (RCVS) has to be considered in young people. CAA gained attention in the last years, but the most known manifestation of cSAH in this context is constituted by transient focal neurological episodes (TFNEs). CAA might have an inflammatory side (CAA-related inflammation), whose diagnosis is relevant due to the efficacy of immunosuppression in resolving essudation. Other causes are hemodynamic stenosis or occlusion in extracranial and intracranial arteries, infective endocarditis (with or without intracranial infectious aneurysms), primary central nervous system angiitis, cerebral venous thrombosis, and rarer diseases. The diagnostic work-up is fundamental for an etiological diagnosis and includes neuroimaging techniques, nuclear medicine techniques, and lumbar puncture. The correct diagnosis is the first step for choosing the most effective and appropriate treatment.

Ruptured Saccular Aneurysm Caused by Necrotizing Arteritis of the Polyarteritis Nodosa Type in Primary Angiitis of Central Nervous System.

Primary angiitis of central nervous system (PACNS) is a rare idiopathic vasculitis that typically involves small arteries. An 18-year-old woman was operated on for resection of a ruptured aneurysm in a cerebral artery. Multiple aneurysms of cerebral arteries had been detected by neuroimaging examinations since the age of 12, and she had been administered drugs following a diagnosis of PACNS since the age of 15. The resected aneurysm was a ruptured saccular aneurysm occurring in a medium-sized artery. Histologically, necrotizing arteritis of the polyarteritis nodosa (PAN) type was noted in the aneurysmal wall. It consisted of an admixture of acute and healing stages. In the acute stage, fibrinoid necrosis in the intima and media and intense inflammatory cell infiltrate in the entire wall were present. The inflammatory cells mainly consisted of lymphocytes, including plasma cells, neutrophils, and macrophages. In the healing stage, disappearance of fibrinoid necrosis, fibrosis in the intima and media, and scarce inflammatory cells were noted. The acute stage was mainly present near the ruptured site. From these findings, the aneurysm was considered to have been caused by necrotizing arteritis of the PAN type. Although saccular aneurysmal formation in a medium-sized artery is rare in PACNS, an understanding of aneurysms produced by necrotizing arteritis of the PAN type offers useful information for the diagnosis and treatment of PACNS.

Inflammatory cerebral amyloid angiopathy with poor outcome – case report

The article presents a clinical case of a 62-year-old patient diagnosed with inflammatory amyloid angiopathy after extensive diagnostic imaging and based on the characteristic radiological picture. Inflammatory amyloid angiopathy is a rare condition with intermediate features between the classic form of amyloid angiopathy and primary central nervous system angiitis. Multiple characteristic features suggesting the diagnosis may be seen on brain magnetic resonance imaging. There are no laboratory markers of the disease. Knowledge of this disease is important as it allows for a quick diagnosis and initiation of appropriate treatment. The response to treatment varies, with full symptom resolution in some patients, and resistance to treatment in others, such as the patient described in this case report.

Primary Angiitis of the Central Nervous System in Adults: A Comprehensive Review of 76 Biopsy-Proven Case Reports.

Primary angiitis of the adult central nervous system (PACNS) is an increasingly recognized but limited disease. Using previous case reports, we sought to summarize the clinical symptoms, imaging manifestations, treatment, and prognosis of patients with biopsy-confirmed PACNS to guide clinical diagnosis and management. We searched the Web of Science database for studies published from January 2000 to April 2023, with the language set to English and the document type limited to [Article or Review or Letter or Editorial Material]. A systematic review of all case reports met the inclusion and exclusion criteria was performed. These patients' clinical, pathological, and imaging characteristics were analyzed, and treatment and prognostic data were summarized. We analyzed 69 articles, including 76 patients with biopsy-confirmed PACNS. And 57.9% of the patients were male, the median age at presentation was 47.0 years, and focal neurological deficits were the most common symptom in patients (78.9%), followed by headache (52.6%). The median duration of biopsy was 1.1 months, of which 49 (64.5%) patients were lymphocytic, 13 (17.1%) were granulomatous, 11 (14.5%) were amyloidotic, and 3 (3.9%) were necrotizing PACNS. Relapse events occurred in 41 (53.9%) patients, including 34 (44.2%) relapses and 8 (10.5%) deaths. Univariate logistic regression analysis revealed that symptomatic epilepsy, prolonged biopsy time window, and CD20 expression in pathological tissues might be independent risk factors for recurrent events in patients (HR=4.69, 95% CI: 1.51-14.54, p=0.007; HR=1.11, 95% CI: 1.00-1.22, p=0.043; HR=5.33, 95% CI: 1.07-26.61, p=0.041). Adult PACNS is associated with frequent relapses and high mortality. Symptomatic epilepsy, prolonged biopsy time window, and CD20 expression in pathological tissue may be associated with recurrent events.

Sensitivity and specificity of vessel wall MRI sequences to diagnose central nervous system angiitis

Magnetic resonance imaging (MRI) with intracranial vessel wall (IVW) sequences is able to directly characterize disease processes affecting the VW increasing the accuracy of intracranial vasculopathies differential diagnosis. Nevertheless, data concerning the specificity and sensitivity of this technic for diagnosis of angiitis of the central nervous system (ACNS) are scant. We aimed at quantifying the IVW abnormalities in a cohort of primary and secondary ACNS and assessing the specificity of ACNS-associated IVW MRI abnormalities. We retrospectively included 36 patients with a diagnosis of ACNS with IVW imaging and we compared IVW MRI abnormalities with those of fifty successive patients admitted at the stroke unit for acute neurological deficit (stroke-unit-group). Since an ACNS was retained as cause of the acute neurological deficit in two patients in the stroke-unit-group, they were finally included in the ACNS group. In our cohort, headache and cognitive disorders were present in 29 and 55% of cases respectively, seizure were rare (n = 5, 13%). CSF analysis were abnormal in 29 patients (76%). MRI showed lesion with restricted diffusion in 82% of patients (n = 31). In 71% of our ACNS population (n = 27) multifocal vessel abnormalities were described in angiographic MRI sequences. Digital subtraction angiography (DSA) was performed in 14 patients and it was suggestive of an ACNS in 11 (79%). Brain-meningeal biopsy was performed in two cases (5%). The typical ACNS-associated IVW abnormalities, i.e., the multifocal concentric VW enhancement with wall thickening, was found in the 95% of ACNS patients (n = 36) whereas it was reported in 4% (n = 2) of the stroke-unit-group (specificity and sensitivity of concentric VW enhancement for ACNS diagnosis of 95% and 94%, respectively). IVW enhancement co-localized with multifocal angiographic stenosis in ACNS patients. The clinical, laboratory and imaging findings were comparable to those of previously described ACNS cohorts in the literature, and particularly to those of DSA-diagnosed patients. Our results suggest that concentric VW enhancement could efficaciously identify patients affected by medium-sized vessels CNS vasculitis with a specificity of 95% and sensitivity of 94%. Further studies with larger samples are necessary to confirm our findings.

Primary Central Nervous System Angiitis Mimicking a Space-Occupying Lesion

Abstract Background and Purpose Intracranial space-occupying lesions are a sine qua non for neoplastic lesions; however, occasionally non-neoplastic lesions mimic neoplastic lesions, leading to diagnostic dilemmas. We report our experience with three patients who presented with a progressive hemispheric syndrome and the diagnostic considerations involved in the cases. Materials and Methods In this retrospective study, we included three patients with primary angiitis of central nervous system (PACNS) who underwent craniotomy and biopsy, suspecting it to be mass lesions. Demographic features, clinical features, radiological features, histopathology, treatment, and clinical outcomes were studied. Results Majority were males. The male:female ratio was 2:1. Lobar involvement was common. MR brain with contrast showed features of high-grade glioma. Despite hemispheric involvement, there was no mass effect. Perilesional edema was seen in all cases. All underwent craniotomy and biopsy; histopathology was consistent with PACNS. All patients were treated with corticosteroids and cyclophosphamide. Rituximab was used in addition to cyclophosphamide in one patient. At 2 years follow-up, two patients were in disease remission and one patient died due to disease progression. Conclusion PACNS has a protean clinical manifestation. A high index of suspicion is required in cases with atypical clinical presentations, radiological features, and normal angiograms. Early histological diagnosis and aggressive immunotherapy with high-dose corticosteroids combined with intravenous cyclophosphamide yields favorable outcomes.

Primary Angiitis of the Central Nervous System - Diagnosis and Management.

Primary angiitis of central nervous system (PACNS) is a rare idiopathic disorder affecting blood vessels of brain, spinal cord, and meninges, consequently leading to infarct and less frequently hemorrhage. CNS vasculitis can also occur as part of systemic vasculitis or secondary to autoimmune diseases or infections. The clinical manifestations of PACNS are non-specific and no single laboratory investigation or neuroimaging finding can reliably diagnose this condition. Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature. The differentials of PACNS are exhaustive and include systemic vasculitis, secondary vasculitis, non-inflammatory intracranial vasculopathies, demyelination, and neoplasm. These alternative conditions can often be distinguished by history, examination, immunological testing, cerebrospinal fluid analysis, and neuroimaging. CA can detect vasculitic changes in the large to medium cerebral arteries but the specificity is low. Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis. The disease has considerable morbidity and fatality unless timely treatment with immunosuppressive agents is initiated. Induction therapy with glucocorticoids and cyclophosphamide followed by azathioprine, mycophenolate mofetil, or methotrexate as maintenance therapy is the cornerstone of management. Biological agents such as rituximab and anti-tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases. This review discusses the approach to the diagnosis, determinants of outcome, and management.

ЦЕРЕБРОВАСКУЛЯРНЫЕ ЗАБОЛЕВАНИЯ: ТИПИЧНЫЕ И НЕТИПИЧНЫЕ КЛИНИЧЕСКИЕ ПРОБЛЕМЫ

There is a big number of patients with chronic brain ischemia both among in-patients and out-patients seen by neurologists. This is a typical clinical situation. Retino-cochleo-cerebral vasculopathy (also called Susac syndrome) is a rare autoimmune disorder affecting brain vessels, the 8th cranial nerve and retina. Chronic brain ischemia usually manifests as cognitive decline, affective (emotional) disorders and focal neurological signs. We studied a “portrait” of patients with chronic brain ischemia, admitted to the hospital As in-patient of rehabilitation unit. Here we present the typical case using as an example cerebral ischemic conditions and unusual ones describing retino-cochleo-cerebral vasculopathy as one of the conditions from the group of primary angiitis of central nervous system in adults. All the case were observed by us in neurological and neurorehabilitation wards. It is a pilot project with 43 patients as the total number of patients included into this study. Their mean age was 62.8 years. The mean duration of in-patient observation was 14 days at the hospital. In this paper we discuss the clinical characteristics of our cohort of patients. The least affected here was the short-term memory for numbers. The most affected domain included emotions, anxiety, and depression. We followed this situation in most of our cases. The unusual clinical case of Susac syndrome in a male patient of 59 years is described. The diagnosis was confirmed using modern instrumental tests such as magnetic-resonance tomography and fluorescent angiography of eye retina.

Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey.

Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Diagnosis and treatment of cPACNS relies on personal experience, expert opinion and case reports/case series. To collect information on diagnostic and therapeutic approaches to transient and progressive cPACNS, a survey was shared among international clinicians (German Society for Pediatric Rheumatology, the Pediatric Rheumatology European Society, the German speaking “Network Pediatric Stroke,” and members of the American College of Rheumatology/CARRA Pediatric Rheumatology list server). Results from this survey will be used to define statements toward a consensus process allowing harmonization of diagnostic and therapeutic approaches and the generation of evidence in a rare condition.

High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System.

Background:High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS.Materials and Methods:Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study. Magnetic resonance image of brain, conventional four-vessel cerebral digital subtraction angiogram, and HRVWI were read by a neuroradiologist. The vessel wall parameters assessed were T1W and T2W appearances, pattern of wall thickening and contrast enhancement, and remodeling index.Results:HRVWI done in 21 patients with PACNS yielded abnormality in 20 (95.2%) who were included in the analysis. The mean age at presentation was 42.55 ± 9.48 years and 14 (70%) were males. The median number of vessels involved were four (range 2–12). The commonest vessels affected were proximal middle cerebral artery (70%) and internal carotid artery (55%). Vessel wall thickening was concentric, eccentric, and absent in 12 (60%), 1 (5%), and 7 (35%) patients, respectively. Vessel wall enhancement was diffuse in 17 (85%), eccentric in 1 (5%), and absent in 2 (10%) patients. One patient had T2W hyperintense stenotic lesion. Remodeling index was negative in 11 (55%) patients.Conclusion:Distinctive vessel wall appearances were observed by HRVWI in PACNS, concentric vessel wall thickening and enhancement being more frequent. Hence, HRVWI can be considered as an additional noninvasive imaging modality in the diagnosis of PACNS.

Udar niedokrwienny mózgu w przebiegu dziecięcego pierwotnego zapalenia naczyń ośrodkowego układu nerwowego (cPACNS) - opis przypadku i przegląd literatury

Introduction: Primary angiitis of central nervous system in childhood (cPACNS) is a rare idiopathic inflammatory disease and an increasing cause of acute ischemic stroke in children. Due to heterogenous classification and poorly understood pathomechanism there are no estimated prevalence and treatment guidelines for PACNS in pediatric population. Case report: A 6-year-old boy with symptoms of acute ischemic stroke was admitted. Neurological examination revealed left hemiparesis. After exclusion of brain hemorrhage the patient was primarily treated with anticoagulants. Brain magnetic resonance (MRI) showed reduced flow with longitudinal stenosis in right internal carotid artery and right middle cerebral artery. Numerous ischemic focuses acute and chronic were found in right cortical and subcortical regions. Hematologic disorders were excluded, as were other inflammatory disorders. Due to imaging studies and clinical features primary angiitis was suspected. Corticosteroids were administrated and regression of neurological deficits was observed. Eventually cPACNS was diagnosed. Conclusion: cPACNS should be considered after exclusion of systematic, rheumatological and secondary causes of vasculitis. Fast diagnosis and administration of therapy is absolutely essential as most of the case series carried on adult population suggest a favorable clinical outcome of cPACNS early treated with steroids.

Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety

ObjectiveTo evaluate the diagnostic accuracy and safety of extended stereotactic brain biopsy (ESBB) in a single center cohort with suspected primary angiitis of the central nervous system (PACNS).MethodsA standardized stereotactic biopsy targeting MRI-positive lesions and collecting samples from the meninges and the cortex as well as from the white matter was performed in 23 patients with clinically suspected PACNS between 2010 and 2017. The relationship between biopsy yield and clinical characteristics, cerebrospinal fluid parameters, MR-imaging, time point of biopsy and exact localization of biopsy as well as number of tissue samples were examined.ResultsPACNS was confirmed in 7 of 23 patients (30.4%). Alternative diagnoses were identified in 7 patients (30%). A shorter time period between the onset or worsening of symptoms (p = 0.018) and ESBB significantly increased the diagnostic yield. We observed only minor and transient postoperative complications in 3 patients (13.0%). ESBB led to a direct change of the therapeutic regime in 13 of 23 patients (56.5%). Careful neuropathological analysis furthermore revealed that cortical samples were crucial in obtaining a diagnosis.ConclusionESBB is a safe approach with good feasibility, even in critically ill patients, and high diagnostic accuracy in patients with suspected PACNS changing future therapies in 13 of 23 patients (56.5%). Early biopsy after symptom onset/worsening is crucial and (sub)acute MRI-lesions should be targeted with a particular need for biopsy samples from the cortical layer.

CNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system.

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis. Each condition will be reviewed in terms of epidemiology, pathology, clinical presentation, diagnostic approach, and treatment. Distinguishing these mimics from the primary angiitis of the CNS is important for proper treatment and prognosis.

An ischemic stroke as the presenting manifestation of rapidly progressive primary angiitis of central nervous system in a 17-year-old boy

BackgroundChildhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease in children. Case presentationWe present a case of a 17-year-old boy with recurrent ischemic events over a short time period. Diagnosis of angiography positive cPACNS was made based on neuroimaging findings while secondary causes or mimics of CNS vasculitis were meticulously excluded. The patient exhibited rapid deterioration of his condition with poor initial response to immunosuppressive treatment. ConclusionsRecognition of cPACNS remains a challenge because of rarity of disease, unexplained etiopathogenesis, protean clinical presentation, as well as lack of specific laboratory and neuroimaging markers.

CLIPPERS and its mimics: evaluation of new criteria for the diagnosis of CLIPPERS

ObjectiveTo evaluate the accuracy of the recently proposed diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).MethodsWe enrolled 42 patients with hindbrain punctate and/or linear...

Atypical presentation of fulminant primary central nervous system angiitis

BackgroundPrimary Angiitis of the Central Nervous System (PACNS) is a rare cause of CNS vasculitis that should be included as part complete differential diagnosis, especially in cases with suggestive imaging findings and an absence of secondary causes for CNS vasculitis. Case presentationWe describe a case of a 47-year-old previously healthy Caucasian male presenting with rapid progression of encephalopathy and fevers. Extensive infectious, autoimmune, and imaging workups were unrevealing. A diagnosis of PACNS was made posthumously on histopathology. ConclusionsPACNS is a challenging diagnosis owing to frequent discrepancies between radiologic and histopathologic findings. Tissue biopsy is key to diagnosing PACNS.

Primary angiitis of the central nervous system - An Indian experience.

Primary angiitis of the central nervous system - An Indian experience.

Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system

Introduction: To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). Methods: Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. Results: 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ± 10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ± 18 years) were analyzed. Cognitive impairment (11 out of 13 patients vs. 5 out of 15, p = .006), ataxia (7 out of 13 vs. 2 out of 15, p = .042) and auditory disturbances (7 out of 13 vs. 0 out of 15, p = .003) were more frequent in SuS patients; whereas seizures were more frequent in PACNS patients (8 out of 15 vs. 1 out of 13, p = .035). On MRI, corpus callosum (CC) involvement was observed more often in SuS, with abnormalities in CC genu, in 13 out of 13 SuS patients vs. only 2 out of 15 PACNS patients (p < .001); in CC body these were present in 13 out of 13 SuS patients vs. 1 out of 15 PACNS patients, (p < .001); and in CC splenium in 12 out of 13 Sus patients vs. 1 of 15 PACNS, p < .001). Cortical lesions were more frequent in PACNS patients (10 out of 15 vs. 3 out of 13 SuS patients, p = .02), as were hemorrhages (5 out of 15 vs. 0 out of 13 SuS, p = .04) and multiple basal ganglia infarcts (7 out of 15 vs. 1 out of 13 Sus, p = .037). Conclusion: Specific clinical and/or MRI findings may help distinguish SuS from PACNS with potential therapeutic implications.

Diagnosis and Treatment of Primary Central Nervous System Angiitis

Primary central nervous system angiitis (PCNSA) is a rare disease. Varying clinical pictures coupled with lack of sensitive and specific diagnostic tests lead to challenges in management of these patients. This unfortunately may lead to both under- or over-diagnosis and unnecessary treatment. It is important to recognize the different conditions that may mimic the clinical and radiologic presentation. We present a contemporary literature review that should update physicians who encounter this patient population. Recent additions to the literature in form of case reports and single-center series show that digital subtraction angiography was the most widely used test to diagnose PCNSA. It is also important to note that brain biopsy is still considered "gold standard" and should be pursued as it not only has information that establishes the diagnosis but also may help in ruling out the diagnosis from mimics. In around 39% of suspected cases, an alternate diagnosis was eventually confirmed highlighting the importance of a comprehensive work-up. For treatment, almost all the literature supports the use of glucocorticoids as the initial treatment and if the patient has a relapse or develops steroid toxicity then the second most commonly used agent was cyclophosphamide. There are increasing reports of benefits with other agents such as methotrexate, azathioprine, mycophenolate, infliximab, and etanercept. The diagnosis and management of PCNSA continues to be a challenge. Formation of prospective patient registries with continued immunologic research for novel targets and immunomodulatory modalities may hold promise for future care of these patients.

Corpus Callosum lesions as diagnostic keys for differentiating Susac Syndrome and primary angiitis of central nervous system (P5.417)

Corpus Callosum lesions as diagnostic keys for differentiating Susac Syndrome and primary angiitis of central nervous system (P5.417)