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At least 19 recordsLinked to original sources
PubMed1998

Central nervous system vasculitis.

Central nervous system vasculitis occurs in a variety of clinical settings. Some exhibit a distinct age preference; others a tissue tropism. Most frequently encountered are giant cell arteritis (temporal arteritis) and vasculitis secondary to infections. The central nervous system may be involved in the antineutrophil cytoplasmic antibody-associated systemic vasculitides and occasionally neurologic abnormalities appear as a presenting manifestation of disease. Isolated angiitis of the central nervous system, a rare form of vasculitis that is restricted to the central nervous system, must be distinguished from other causes of central nervous system inflammation and from noninflammatory vascular disease. We are learning a great deal about the cellular mechanisms of vascular inflammation in general. Some manifestations of the clinical disease result from histologic features of the infiltrate and the size of affected vessel. However, the local consequences of inflammation, such as increased coagulation and altered vasomotor tone, as well as the systemic consequences, such as activation of the central noradrenergic systems, trigeminovascular system, and hypothalamic pituitary adrenal axis, contribute both to pathogenesis of disease and to recovery.

Behcet Syndrome↗
PubMed1997

Radiographic features of central nervous system vasculitis.

Central nervous system (CNS) vasculitis refers to primary and secondary disorders of the CNS vasculature. Most authorities agree that CNS vasculitis is a potentially serious disorder; therefore, prompt diagnosis and initiation of therapy are high priorities in treatment. Remarkable progress has been made in the diagnosis, evaluation, and treatment of this disorder. This article examines many aspects of the radiographic evaluation of CNS vasculitis.

Adult↗
PubMed1989

Plasma concentrations of complement-activation complexes correlate with disease activity in patients diagnosed with isolated central nervous system vasculitis.

Isolated central nervous system (CNS) vasculitis is rare medium- sized vessel disease limited to intracerebral vessels. The two most common symptoms of this inflammatory disorder observed at entry to a hospital are headaches and mild memory deficits. Further progression of this disease may result in focal neurologic alterations and seizures. Currently, the most common laboratory abnormality noted is an elevated erythrocyte sedimentation rate. The complement (C) system is known to play a role in many inflammatory processes; it may also be involved in CNS vasculitis. In this longitudinal study of patients with CNS vasculitis, we detected C activation by highly sensitive and specific assays that are capable of identifying breakdown products formed after C activation: C3a des arg, C4a des arg, C5a des arg, C1rC1s-C1-inhibitor complex, and terminal C complex (C5b-9). We present two cases of documented CNS vasculitis in which serial measurements of these C-activation products correlate with disease activity. Our results indicate that a temporal association exists between C activation and the clinical presentation of CNS vasculitis. We conclude that physicians should monitor C-activation by-products in plasma when they attempt to follow the clinical course of CNS vasculitis.

Adult↗
PubMed2006

Central nervous system vasculitis in children.

Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.

Biomarkers↗
PubMed2003

Central nervous system vasculitis.

Vasculitis of the central nervous system (CNS) is classified as primary angiitis or as vasculitis secondary to a variety of diseases. A wide spectrum of clinical features may occur. A definite diagnosis is hampered by the difficulty in obtaining tissue for histology. Consequently, a diagnosis is frequently made on the basis of clinical presentation, brain magnetic resonance imaging, and cerebral angiography without pathologic confirmation. Recent experience shows that there are multiple other conditions that can mimic CNS vasculitis, many of which have different therapies. Most patients with CNS vasculitis should be treated aggressively with a combination of immunosuppressive medications. The prognosis is greatly improved with early recognition and therapy.

Autoantibodies↗
PubMed1998

Pediatric Wegener's granulomatosis complicated by central nervous system vasculitis.

An unusual case of central nervous system vasculitis in pediatric Wegener's granulomatosis, a rare disorder that infrequently presents during childhood, is reported. A 13-year-old girl with Wegener's granulomatosis, whose initial presentation resembled Henoch-Schonlein purpura, developed recurring seizures. MRI of the brain demonstrated multiple areas of increased signal in the occipital, parietal, and frontal lobes, consistent with central nervous system vasculitis. Although both peripheral and cranial neuropathies have been reported in patients with Wegener's granulomatosis, cerebral vasculitis is unusual, particularly in childhood. This case emphasizes the need to consider Wegener's granulomatosis in the differential diagnosis of both unexplained seizures and central nervous system vasculitis in children with systemic illness.

Adolescent↗
PubMed2005

Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease.

Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.

Adolescent↗
PubMed1995

Evaluation and treatment of central nervous system vasculitis.

Angiitis of the central nervous system (CNS) remains a poorly understood and clinically challenging form of vascular inflammatory disease. Primary angiitis of the CNS (PACNS) has been viewed as a relentless and uniformly fatal disorder if untreated. In addition, recent trends have demonstrated an increasing reliance on angiographic diagnosis without tissue confirmation. It has been suggested that PACNS is clinically more heterogeneous than previously appreciated and may include relatively benign subsets. A reappraisal of diagnostic approaches has suggested caution in the diagnosis of CNS angiitis on purely angiographic grounds. Secondary vasculitis of the CNS is even more heterogeneous. Clinicians involved in the evaluation of patients with presumed CNS vasculitis need to be aware of the clinical spectrum of vascular inflammatory disease within the CNS as well as the strengths and limitations of currently available diagnostic modalities.

Central Nervous System↗
PubMed1989

[Vasculitis of the central nervous system].

Vasculitis of the central nervous system (CNS) is a rare disease characterized inflammation and/or sclerosis of the vascular wall, usually due to an immunological mechanism. The positive diagnosis of CNS vasculitis is difficult owing to its protean clinical manifestations, to its variable course and to the nonspecificity of CT and MRI images. Angiography itself may be of no avail since the segmental narrowing images, although suggestive, are neither constant nor pathognomonic. The causes of CNS vasculitis are very numerous. The aetiological diagnosis is easy when the causal systemic disease is already known or when an inflammatory syndrome and other localizations are present, but it is difficult in those exceptional cases where the vasculitis is limited to the CNS. Biopsy of the cortex and leptomeninges may them be required. Treatment varies according to the cause; it rests essentially on corticosteroid therapy, sometimes combined with cyclophosphamide.

Brain↗
PubMed2004

Central nervous system vasculitis in children.

PURPOSE OF THE REVIEW: To summarize the current literature on central nervous system vasculitis in childhood because this condition remains a diagnostic and therapeutic challenge. RECENT FINDINGS: Central nervous system vasculitis in childhood may be primary or secondary to a variety of conditions including infections, collagen vascular diseases, systemic vasculitides, and malignancies. Conditions that result in vasospasm or are associated with noninflammatory vasculopathies may mimic the features of central nervous system vasculitis. Recent studies have described the clinical spectrum of CNS vasculitis in childhood. The most common presenting features are headaches and focal neurologic deficits. The diagnosis of central nervous system vasculitis remains particularly difficult because the available investigative modalities have limited sensitivities and specificities. The most helpful diagnostic tests include cerebrospinal fluid analysis, MRI of the brain, and angiography. However, brain biopsy may be required to diagnose small vessel vasculitis. SUMMARY: This review summarizes recent data on primary central nervous system vasculitis and some of the secondary CNS vasculitides in children. Awareness of the presenting clinical features of CNS vasculitis should lead to consideration of the diagnosis. Awareness of the sensitivity and specificity of the various diagnostic tests should lead to the more prudent use of invasive diagnostic tests including angiography and brain biopsy.

Brain↗
PubMed1998

Uveitis and central nervous system vasculitis.

Vasculitis confined to the central nervous system (CNS) is a rare disease usually characterized by headache and focal neurologic symptoms. Patients with primary vasculitis of the CNS may have symptoms and laboratory findings of systemic disease such as fatigue and elevated erythrocyte sedimentation rate, but by definition, focal inflammation should not be present outside the CNS. We describe 3 patients with uveitis in association with this diagnosis. The recognition of this association adds to the complex differential diagnosis of uveitis in association with CNS disease, and indicates that "isolated" angiitis of the CNS may display clinical features outside the brain and spinal cord.

Adult↗
PubMed1993

Miscellaneous vasculitic syndromes including Behçet's disease and central nervous system vasculitis.

Behçet's disease is a chronic, relapsing, inflammatory process with clinical features of aphthous oral and genital ulcerations, uveitis, cutaneous vasculitis, arthritis, and central nervous system involvement. Large-vessel vasculitis, thrombophlebitis, and gastro-intestinal ulcerations may occur. As in other rheumatologic diseases, a genetic predisposition coupled with a triggering event leads to alterations in immune function and response, with variable clinical disease manifestations. Mononuclear cell activation and cytokine release, neutrophil chemotaxis, and humoral immune response with elevations of circulating immune complexes contribute to the pathogenesis of Behçet's disease. Vasculitis and vascular involvement are highlighted. There is no single diagnostic test for Behçet's disease. Treatment is comprised of anti-inflammatory and immunosuppressive agents and, in selected individuals, anticoagulation. Isolated central nervous system vasculitis or noninfectious primary (granulomatous) angiitis of the central nervous system is reviewed briefly. This condition is rare, predominantly affects young women, and includes symptoms of headache, weakness, and confusion. Characteristically, erythrocyte sedimentation rate is normal, serologic studies are negative, and cerebral angiography shows multifocal and segmental vascular narrowing and beading. Typical histopathology is granulomatous in less than 50% of patients, followed by necrotizing or lymphocytic vasculitis, or both, often with thrombosis. Treatment with immunosuppressive agents improves symptoms and outcome.

Behcet Syndrome↗
PubMed1991

Scleroderma and central nervous system vasculitis.

We describe a patient with scleroderma (CREST syndrome) and central nervous system vasculitis. While angiography demonstrated segmental symmetrical arterial narrowing characteristic of vasculitis, results of leptomeningeal biopsy were normal. There was no evidence of systemic vasculitis, renal failure, or malignant hypertension previously thought to be required to explain central nervous system dysfunction in patients with scleroderma. Signs and symptoms attributable to vasculitis were reversible with aggressive immunosuppressive therapy.

Cerebrovascular Circulation↗
PubMed2002

[Central nervous system vasculitis and of the peripheral nerves in the elderly].

Vasculitis of the nervous system are rare in the elderly. When present, they may constitute an urgent diagnosis and a therapeutic emergency. Clinical expression is rich and without specificity. Atypical signs (unusual course of dementia, systemic signs) or atypical laboratory results (inflammatory syndrome) may suggest the diagnosis of vasculitis. However, as multiple comorbidity is the rule in elderly subjects, searching for intercurrent factors (e.g. atrial fibrilation due to infectious disease causing embolic stroke) may be more contributive than searching for proof of a rare disease (vasculitis) with invasive procedures in this population. Giant cell (temporal) arteritis is the only vasculitis specifically related with age; the vital prognosis of vision may be compromised. Corticosterid therapy must be instituted without delay. Periartritis nodosa begins in 30% of cases after 60 years of age. The clinical features are the same as in younger subjects. Other vasculidis are rare in the elderly. In absence of specific studies in this population, therapeutic protocols are the same as in younger subjects but may have to be adjusted.

Aged↗
PubMed2006

Primary central nervous system vasculitis in children.

OBJECTIVE: Primary angiitis of the central nervous system (PACNS) is a severe and ill-defined neurologic disease. The goal of this study was to characterize the presenting features, treatment, and neurologic outcome of PACNS in children (cPACNS) and to define the predictors of disease progression in order to identify high-risk patients with cPACNS. METHODS: The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical and vascular imaging findings, including identification of arterial stenosis on conventional angiography or magnetic resonance (MR) angiography. Disease progression was defined angiographically at >3 months after initial angiography. Clinical data obtained in prospectively collected standardized assessments and results of laboratory tests, including detection of cerebrospinal fluid abnormalities, were noted, and neuroimaging studies were reanalyzed. Predictors of progression were identified and tested in multivariate regression models. RESULTS: Sixty-two consecutive patients with cPACNS (38 male, 24 female, median age 7.2 years) were included. Two distinct subgroups were identified, those with progressive disease and those with nonprogressive disease. Progressive cPACNS was found in 20 of 62 children and was predicted by a clinical presentation of neurocognitive dysfunction, multifocal parenchymal lesions on MR imaging, and evidence of distal stenoses on angiography. CONCLUSION: The spectrum of PACNS in children includes both progressive and nonprogressive forms. Characteristic features at diagnosis can be used to predict later progression, to identify a distinct high-risk cPACNS cohort, and to help guide selection of patients for immunosuppressive therapy.

Adolescent↗
PubMed2003

Central nervous system vasculitis.

Vasculitis is an inflammation of the vessel wall. It may be either primary or secondary. Primary vasculitis includes systemic vasculitis (large, medium, and small-vessel vasculitis) and localized vasculitis (isolated angiitis of the central nervous system and non-systemic vasculitic neuropathy). Secondary vasculitis may be present in connective tissue disorders or may be caused by infections, neoplasms, and substance abuse. Patients presenting with symptoms suggestive of vasculitis require brain neuroimaging, lumbar puncture, and angiography, but only biopsy allows a definite diagnosis.

Cerebral Angiography↗