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Central Nervous System Vasculitis

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Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61)

Also Known As:

Vasculitis, Central Nervous System; CNS Vasculitis; Central Nervous System Angiitis; Granulomatous Arteritis; Primary Central Nervous System Vasculitis; Vasculitis, CNS, Secondary; Angiitis, Cerebral; Angiitis, Granulomatous; Arteritis, Granulomatous; Arteritis, Postzoster; CNS Vasculitis, Primary; CNS Vasculitis, Secondary; Vasculitis, CNS; Vasculitis, Cerebral; Vasculitis, Primary CNS; Vasculitis, Secondary CNS; Angiitis, Central Nervous System; Cerebral Angiitis; Cerebral Vasculitis; Granulomatous Angiitis; Postzoster Arteritis; Primary CNS Vasculitis; Secondary CNS Vasculitis

Networked: 263 relevant articles (11 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Vasculitis (Vasculitides)
2.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
3.	Hodgkin Disease (Hodgkin's Disease)
4.	Opportunistic Infections (Opportunistic Infection)
5.	Central Nervous System Vasculitis

Experts

1.	Salvarani, Carlo: 5 articles (01/2016 - 02/2008)
2.	Hunder, Gene G: 5 articles (01/2016 - 02/2008)
3.	Brown, Robert D: 5 articles (08/2015 - 08/2007)
4.	Benseler, Susanne M: 5 articles (10/2012 - 07/2005)
5.	Huston, John: 4 articles (08/2015 - 02/2008)
6.	Giannini, Caterina: 4 articles (08/2015 - 06/2006)
7.	Miller, Dylan V: 3 articles (08/2015 - 02/2008)
8.	Halliday, William: 3 articles (11/2012 - 11/2010)
9.	Hawkins, Cynthia: 3 articles (11/2010 - 07/2005)
10.	Pagnoux, Christian: 2 articles (01/2016 - 10/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Central Nervous System Vasculitis:

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/2001 - "Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission." 07/05/2010 - "In two patients with progressive CNS vasculitis, the treatment was supplemented by intravenous cyclophosphamide and azathioprin, respectively. " 03/01/2007 - "Cerebral vasculitis is usually treated with monthly glucocorticoid and cyclophosphamide boluses separated by continuous glucocorticoid therapy. " 06/01/1986 - "Three weeks after a single intravenous dose of cyclophosphamide for central nervous system vasculitis she died. " 10/16/2012 - "Cyclophosphamide treatment for unrelenting CNS vasculitis secondary to tuberculous meningitis."
2.	SteroidsIBA 01/01/1994 - "In group II significant improvement was noticed at six months concerning the clinical index (1.77 pts vs 7.17) and the daily dose of steroids, 3 patients died because of cerebral vasculitis refractory to IVCY. " 07/01/2001 - "Pathogenic mechanisms are not clear but probably involve autoimmune cerebral vasculitis because of the efficacy of steroids." 05/01/2015 - "Cerebral vasculitis associated with AMPPE was diagnosed and treated with systemic steroids and immune suppression. " 09/01/1978 - "Isolated cerebral vasculitis was the clinical diagnosis made by careful exclusion; the illness reponded well to steroids and there was later angiographic evidence of healing." 01/01/2012 - "Cerebral vasculitis complicating postoperative meningitis: the role of steroids revisited."
3.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 08/01/2014 - "We retrospectively reviewed 3T-high-resolution MR imaging vessel wall studies performed on 26 patients with a confirmed diagnosis of CNS vasculitis and reversible cerebral vasoconstriction syndrome during a follow-up period. " 08/01/2014 - "Postgadolinium 3T-high-resolution MR imaging appears to be a feasible tool in differentiating vessel wall patterns of CNS vasculitis and reversible cerebral vasoconstriction syndrome changes during a follow-up period." 08/01/2014 - "High-resolution MRI vessel wall imaging: spatial and temporal patterns of reversible cerebral vasoconstriction syndrome and central nervous system vasculitis." 09/01/2013 - "Vessel wall enhancement in herpes simplex virus central nervous system vasculitis." 01/29/2013 - "Because of continued clinical deterioration, stereotactic brain biopsy was performed, showing small-vessel CNS vasculitis. "
4.	Methylprednisolone FDA LinkGeneric 12/04/2012 - "Fatal exserohilum meningitis and central nervous system vasculitis after cervical epidural methylprednisolone injection." 03/01/1992 - "The hypothesis of primary CNS vasculitis was made, and pulse methylprednisolone therapy was introduced with good recovery of neurological syndrome except for persistent amaurosis." 01/01/2008 - "Although high-dose methylprednisolone and cyclophosphamide are thought to cause RPLS, we think that it is justified to use these agents, at least in difficult cases, for making a clear-cut differentiation from CNS vasculitis, as long as blood pressure and fluid volume are well controlled. " 01/15/2013 - "Apart from the iatrogenic effects of treatment, neurologic complications of Hodgkin lymphoma (HL) can be divided into direct (meningeal or intracranial/spinal localization) and indirect (paraneoplastic/immune complications).(1) Here, we present a patient with granulomatous angiitis of the CNS (GANS) associated with HL that dramatically improved after the treatment of the angiitis by cyclophosphamide, methylprednisolone, and specific chemotherapy." 04/01/2015 - "The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). "
5.	Adrenal Cortex Hormones (Corticosteroids)IBA 01/01/2014 - "Patients with CNS vasculitis are commonly treated with corticosteroids. " 01/01/1986 - "Seizures and mental abnormalities responded excellently to corticosteroids only, supporting the view that encephalopathy could be the clinical manifestation of autoimmune cerebral vasculitis." 12/01/1991 - "The common features of isolated benign cerebral vasculitis are as follows: 1) benign evolution, 2) sensitiveness to corticosteroids, 3) absence or minimal change of CSF findings, 4) angiographic pattern of arteritis, 5) the most common symptom is headache. " 01/01/2009 - "Standard therapy of cerebral vasculitis includes corticosteroids often combined with immunosuppressants." 05/01/2015 - "A dramatic and sustained neurological improvement, and likewise of the eosinophilia, following treatment with corticosteroids and cyclophosphamide, emphasises the tractability of this newly described form of CNS vasculitis."
6.	Antigen-Antibody Complex (Immune Complex)IBA 06/01/1986 - "Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation." 02/01/2005 - "In conclusion, we propose that plasmapheresis is a reliable and effective therapy and may be considered as the first-line treatment in this critical condition to prevent massive intracerebral hemorrhagic complication of this immune complex-mediated cerebral vasculitis leading to irreversible neurological sequelae or mortality." 08/31/2000 - "MRL/MpJ-lpr/lpr (MRL/lpr) mice spontaneously develop immune complex-mediated glomerulonephritis, granulomatous arteritis, and thrombocytopenia. " 09/01/2015 - "Infection can cause cerebral vasculitis not only by direct invasion of the vessel wall, but by immune complex deposition, or through secondary cryoglobulineamia. " 05/01/1998 - "The present case had these characteristics, and the cerebral vasculitis mediated by IgG-rheumatoid factor and immune complex was indicated as a probable cause of multiple cerebral infarctions."
7.	Immunosuppressive Agents (Immunosuppressants)IBA 01/01/2004 - "The potential for response of the process to antimicrobials and/or immunosuppressants, and the potential for devastating consequences if the process is left untreated, has heightened the urgency in recognizing CNS vasculitis. " 01/01/2009 - "Standard therapy of cerebral vasculitis includes corticosteroids often combined with immunosuppressants." 04/01/2004 - "Like in all cases of CNS vasculitis, exclusion of other systemic diseases or its mimickers associated with cerebral involvement is important.BACNS could respond favorably to treatment with corticosteroids alone or in combination with a calcium channel blocker, thereby avoiding unnecessary and prolonged treatment with other immunosuppressants like cyclophosphamide with their side effects. "
8.	Calcium Channels (Calcium Channel)IBA 05/01/2006 - "No standard management has been established, but most authors agree that 1) acute-phase treatment includes cessation of vasoconstrictors, treatment of associated conditions, vasospasm treatment (calcium channel antagonists), and corticosteroids; 2) other measures include headache relief, blood pressure control, and stroke, cerebral edema, and seizure treatment; 3) definitive diagnosis requires conventional angiography and exclusion of alternative diagnosis; 4) a second arterial examination after 4 to 6 weeks is mandatory to confirm reversibility of vasoconstriction; 5) brain biopsy is indicated to rule out cerebral vasculitis in severe cases with clinical deterioration under steroid treatment or atypical findings; 6) immunosuppression should be reserved for patients with brain-leptomeningeal biopsy-proven vasculitis or used while waiting for a brain biopsy result; and 7) long-term measures include secondary stroke prevention and treatment of complications." 04/01/2004 - "Like in all cases of CNS vasculitis, exclusion of other systemic diseases or its mimickers associated with cerebral involvement is important.BACNS could respond favorably to treatment with corticosteroids alone or in combination with a calcium channel blocker, thereby avoiding unnecessary and prolonged treatment with other immunosuppressants like cyclophosphamide with their side effects. "
9.	Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA 02/15/2008 - "Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment." 04/01/2009 - "Refractory central nervous system vasculitis and gastrocnemius myalgia syndrome in Crohn's disease successfully treated with anti-tumor necrosis factor-alpha antibody."
10.	Intravenous Immunoglobulins (IVIG)FDA Link 04/01/2005 - "IVIG therapy resulted in remission and has been effective not only for the clearance of B19, but also for the improvement of clinical and radiological findings of CNS vasculitis. "

Therapies and Procedures

1.	Transplants (Transplant) 10/01/2015 - "Cerebral Vasculitis in X-linked Lymphoproliferative Disease Cured by Matched Unrelated Cord Blood Transplant." 08/01/2002 - "Mycotic cerebral vasculitis in a paediatric cardiac transplant patient excludes misadventure." 04/01/2005 - "Central nervous system vasculitis secondary to parvovirus B19 infection in a pediatric renal transplant patient." 03/01/2005 - "We describe a case of cerebral vasculitis seen on angiography in a patient with a history of non-Hodgkin lymphoma and graft-versus-host disease (GVHD) 2 years after match-related bone-marrow transplant. " 04/01/1998 - "Cerebral vasculitis during FK 506 treatment in a liver transplant patient."
2.	Plasmapheresis 12/01/2000 - "In conclusion, we propose that plasmapheresis may be used as a first-line therapy or rescue therapy for cerebral vasculitis in HSP." 12/01/2000 - "To the best of our knowledge, this is the first description of MRI abnormalities and multiple retinal artery branch occlusion of cerebral vasculitis in a patient with HSP that was successfully treated by plasmapheresis alone. " 10/01/1993 - "Response to plasmapheresis and absence of CNS vasculitis at autopsy." 08/01/2003 - "Cerebral vasculitis in Henoch-Schönlein purpura: MRI and MRA findings, treated with plasmapheresis alone." 12/01/2000 - "Cerebral vasculitis in Henoch-Schönlein purpura: a case report with sequential magnetic resonance imaging changes and treated with plasmapheresis alone."
3.	Drug Therapy (Chemotherapy) 01/15/2013 - "Apart from the iatrogenic effects of treatment, neurologic complications of Hodgkin lymphoma (HL) can be divided into direct (meningeal or intracranial/spinal localization) and indirect (paraneoplastic/immune complications).(1) Here, we present a patient with granulomatous angiitis of the CNS (GANS) associated with HL that dramatically improved after the treatment of the angiitis by cyclophosphamide, methylprednisolone, and specific chemotherapy."
4.	Aftercare (After-Treatment) 01/01/2009 - "To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. " 10/01/2012 - "The main objective was to develop a trajectory for von Willebrand factor (vWF) antigen in childhood primary CNS vasculitis (cPACNS) after treatment and compare this with disease activity and other inflammatory markers. " 04/01/2015 - "The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). "
5.	Hemispherectomy 07/01/1990 - "Pathological study of a subtotal hemispherectomy specimen revealed widespread cerebral vasculitis with immunofluorescence staining for IgG, IgM, IgA, C3, and Clq, and ultrastructural evidence of vascular injury in addition to severe cortical atrophy with marked neuronal loss. "