Diagnosis Of Central Nervous System Vasculitis Research Articles

Central nervous system vasculitis in a patient with axial spondyloarthritis treated with infliximab: A case report and literature review.

Central nervous system (CNS) vasculitis is a rare form of vasculitis involving the blood vessels of the brain. It may be primary when it is confined to the CNS or secondary in the context of systemic inflammatory conditions such as systemic lupus erythematosus, rheumatoid arthritis, or infections. However, there is no known association with axial spondyloarthritis. Herein, we present the case of a 37-year-old man, with axial spondyloarthritis treated with infliximab for 9 years, who presented with persistent fevers, elevated inflammation markers, lateral medullary syndrome, and right-sided hemiparesis. Magnetic resonance imaging of the brain demonstrated multiple cerebral infarcts. Examination of cerebrospinal fluid showed mild lymphocytic pleocytosis and protein elevation. Digital subtraction angiography and transcranial ultrasonography of the cerebral blood vessels revealed luminal narrowing of the basilar and the left posterior cerebral artery. The diagnosis of CNS vasculitis was made and intravenous methylprednisolone and cyclophosphamide pulses were administered, leading to fever remission with gradual improvement and resolution of the neurological manifestations.

Diagnosis and follow-up evaluation of central nervous system vasculitis: an evaluation of vessel-wall MRI findings

ObjectiveTo approach the clinical value of MRI with vessel wall imaging (VWI) in patients with central nervous system vasculitis (CNSV), we analyzed patterns of VWI findings both at the time of initial presentation and during follow-up.MethodsStenoocclusive lesions, vessel-wall contrast enhancement (VW-CE) and diffusion-restricted lesions were analyzed in patients with a diagnosis of CNSV. On available VWI follow-up, progression, regression or stability of VW-CE were evaluated and correlated with the clinical status.ResultsOf the 45 patients included, 28 exhibited stenoses visible on MR angiography (MRA-positive) while 17 had no stenosis (MRA-negative). VW-CE was found in 2/17 MRA-negative and all MRA-positive patients (p < 0.05). 79.1% (53/67) of stenoses showed VW-CE. VW-CE was concentric in 88.3% and eccentric in 11.7% of cases. Diffusion-restricted lesions were found more frequently in relation to stenoses with VW-CE than without VW-CE (p < 0.05). 48 VW-CE lesions in 23 patients were followed over a median time of 239.5 days. 13 VW-CE lesions (27.1%) resolved completely, 14 (29.2%) showed partial regression, 17 (35.4%) remained stable and 4 (8.3%) progressed. 22/23 patients received immunosuppressive therapy for the duration of follow-up. Patients with stable or progressive VW-CE were more likely (p < 0.05) to have a relapse (14/30 cases) than patients with partial or complete regression of VW-CE (5/25 cases).ConclusionConcentric VW-CE is a common finding in medium/large-sized vessel CNSV. VW-CE might represent active inflammation in certain situations. However, follow-up VWI findings proved ambiguous as persisting VW-CE despite immunosuppressive therapy and clinical remission was a frequent finding.

CNS Vasculitis: an Approach to Differential Diagnosis and Management.

The goal of this review is to provide an up-to-date approach to diagnosis and management of patients with central nervous system (CNS) vasculitis. Challenges in diagnosis of CNS vasculitis still exist due to the broad differential diagnosis and generally nonspecific initial clinical manifestations. Differentiation between primary angiitis of the CNS (PACNS) and secondary causes is important in guiding management. Recent longitudinal cohort studies have improved our understanding of PACNS. Advances in neuroimaging and molecular testing have enhanced diagnostic decision-making. Therapy remains largely empiric, guided by observational data. Despite the limited use of targeted therapies, glucocorticoids and cyclophosphamide remain the mainstays of therapy in PACNS. Securing a diagnosis through a careful, team-based approach with emphasis on ruling out possible mimics is paramount in the management of patients with CNS vasculitis.

Pediatric Inflammatory Diseases

Central nervous system (CNS) vasculitis can affect both adults and children, but some of these occur almost exclusively in childhood. In children may develop as a primary condition or secondary to an underlying systemic disease. Cerebral vasculitis can be classified on the basis of the diameter of the involved vessels, although there is no univocal consensus. The diagnosis of CNS vasculitis is particularly difficult because the available investigative modalities have limited sensitivities and specificities. The most helpful diagnostic tests include cerebrospinal fluid analysis, MRI (MR angiography/venography (MRA/MRV) of the brain, and angiography. However, brain biopsy may be required to diagnose small vessel vasculitis in order to make differential diagnosis with a wide range of conditions, such as degenerative vasculopathies, embolic diseases, or coagulation disorders. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitis in children in which are involved small vessel.

Vascular involvement of the central nervous system and systemic diseases: etiologies and MRI findings

The objective of this study was to review magnetic resonance imaging (MRI) findings in patients with vascular involvement of the central nervous system (CNS) associated with systemic diseases. We reviewed the MRI findings in clinically suspected cases of vascular involvement of the CNS associated with systemic diseases. Vascular CNS involvement was considered in the presence of characteristic clinical, MRI and/or MR angiography findings. In order to be included in the study, patients needed to have a complete clinical and laboratory investigation and a follow-up of a minimum of 6 months. Twenty-four patients (17 women and 7 men), with mean age of 29.5 years had diagnosis of CNS vasculitis and were included. The clinical presentation was variable, but the most common complaints were headache in 18, focal deficits in 9, disturbances of consciousness in 9, and seizures in 8 patients. Underlying causes for CNS vasculitis were identified in all patients and included systemic lupus erythematosus in eight, tuberculosis in three, bacterial meningitis in three, Takayasu arteritis in two, polyarteritis nodosa in two, syphilis in two, drug abuse in two, yellow fever in one and varicella in one patient. Nonspecific high intensity T2WI/FLAIR lesions in white matter were the most common finding, present in ten patients. Eight patients had infarctions in large cerebral arteries territory, associated or not with high intensity T2WI/FLAIR small foci. Vascular involvement of the CNS can be found in a great variety of systemic diseases, including rheumatologic, infectious and drug abuse. Clinical findings are unspecific and MRI/MRA may help to establish the correct diagnosis.

Central Nervous System Vasculitis in Systemic Lupus Erythematosus

Necrotizing central nervous system (CNS) vasculitis in systemic lupus erythematosus (SLE) is rare, and its prognosis can be ominous. We describe a 49-year-old male with active SLE developing progressively deteriorating CNS symptoms due to CNS vasculitis. CT and MRI of the brain suggested vasculitis, and this was confirmed by brain biopsy. The patient was successfully treated with oral cyclophosphamide (2 mg/kg/day) and prednisone (1 mg/kg/day) following a 5-day course of intravenous (IV) pulse methylprednisolone (1 g/day). The diagnosis of CNS vasculitis should be considered in an SLE patient with deteriorating CNS symptoms and investigated first with CT and/or MRI; in suspected cases, brain biopsy should be performed. Once the diagnosis is established, early institution of daily oral cyclophosphamide and high-dose prednisone following a short course of pulse dose IV methylprednisolone can be used for its treatment and can result in improvement in outcome.