Abstract
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.
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References and Recommended Reading
Gallagher KT, Shaham B, Reiff A, et al.: Primary angiitis of the central nervous system in children: 5 cases. J Rheumatol 2001, 28:616–623.
Lanthier S, Lortie A, Michaud J, et al.: Isolated angiitis of the CNS in children. Neurology 2001, 56:837–842.
Benseler S, Schneider R: Central nervous system vasculitis in children. Curr Opin Rheumatol 2004, 16:43–50.
Patsalides AD, Wood LV, Atac GK, et al.: Cerebrovascular disease in HIV-infected pediatric patients: neuroimaging findings. AJR Am J Roentgenol 2002, 179:999–1003.
Ueno M, Oka A, Koeda T, et al.: Unilateral occlusion of the middle cerebral artery after varicella-zoster virus infection. Brain Dev 2002, 24:106–108.
Lloyd-Still JD, Tomasi L: Neurovascular and thromboembolic complications of inflammatory bowel disease in childhood. J Pediatr Gastroenterol Nutr 1989, 9:461–466.
von Scheven E, Lee C, Berg BO: Pediatric Wegener’s granulomatosis complicated by central nervous system vasculitis. Pediatr Neurol 1998, 19:317–319.
Deshpande PV, Gilbert R, Alton H, Milford DV: Microscopic polyarteritis with renal and cerebral involvement. Pediatr Nephrol 2000, 15:134–135.
Nadeau SE: Neurologic manifestations of systemic vasculitis. Neurol Clin 2002, 20:123–150, vi.
Belostotsky VM, Shah V, Dillon MJ: Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol 2002, 17:754–761.
Krespi Y, Akman-Demir G, Poyraz M, et al.: Cerebral vasculitis and ischaemic stroke in Behcet’s disease: report of one case and review of the literature. Eur J Neurol 2001, 8:719–722.
Regan M, Haque U, Pomper M, et al.: Central nervous system vasculitis as a complication of refractory dermatomyositis. J Rheumatol 2001, 28:207–211.
Steens SC, Bosma GP, ten Cate R, et al.: A neuroimaging follow up study of a patient with juvenile central nervous system systemic lupus erythematosus. Ann Rheum Dis 2003, 62:583–586.
Song DK, Boulis NM, McKeever PE, Quint DJ: Angiotropic large cell lymphoma with imaging characteristics of CNS vasculitis. AJNR Am J Neuroradiol 2002, 23:239–242.
Sheehy N, Sheehan K, Brett F, et al.: Hodgkins disease presenting with granulomatous angiitis of the central nervous system. J Neurol 2003, 250:112–113.
Aydin K, Okuyaz C, Gucuyener K, et al.: Moyamoya disease presented with migrainelike headache in a 4-year-old girl. J Child Neurol 2003, 18:361–363.
Bowen MD, Burak CR, Barron TF: Childhood ischemic stroke in a nonurban population. J Child Neurol 2005, 20:194–197.
Nagaratnam N, James WE: Isolated angiitis of the brain in a young female on the contraceptive pill. Postgrad Med J 1987, 63:1085–1086.
Aoki S, Hayashi N, Abe O, et al.: Radiation-induced arteritis: thickened wall with prominent enhancement on cranial MR images report of five cases and comparison with 18 cases of Moyamoya disease. Radiology 2002, 223:683–688.
Nagaraja D, Verma A, Taly AB, et al.: Cerebrovascular disease in children. Acta Neurol Scand 1994, 90:251–255.
Cravioto H, Feigin I: Noninfectious granulomatous angiitis with predilection for the nervous system. Neurology 1959, 9:599–609.
Calabrese LH, Mallek JA: The treatment of vasculitis of the central nervous system. JAMA 1987, 258:778.
Calabrese LH, Mallek JA: Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine (Baltimore) 1988, 67:20–39.
Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ: Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med 1992, 59:293–306.
Benseler SM, Silverman E, Aviv RI, et al.: Primary central nervous system vasculitis in children. Arthritis Rheum 2006, 54:1291–1297. Comprehensive review of the clinical presentation, laboratory features, imaging characteristics, and outcome of angiography-positive cPACNS.
Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH: Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum 2002; 47:662–9.
Hajj-Ali R, Villa-Forte AL, Abou-Chebel A, et al.: Long term outcome of patients with primary angiitis of the central nervous system (PACNS). Arthritis Rheum 2000, 43:S162.
Calabrese LH: Vasculitis of the central nervous system. Rheum Dis Clin North Am 1995, 21:1059–1076.
Golomb MR, Weiss SK, Ibrahim SH, deVeber GA: A 15-year-old boy with central nervous system vasculopathy presenting with dysarthria-clumsy hand syndrome. J Child Neurol 2002, 17:241–243.
Lasner TM, Sutton LN, Rorke LB: Multifocal noninfectious granulomatous encephalitis in a child. Case report. J Neurosurg 1997, 86:1042–1045.
Yaari R, Anselm IA, Szer IS, et al.: Childhood primary angiitis of the central nervous system: two biopsy-proven cases. J Pediatr 2004, 145:693–697.
Chabrier S, Rodesch G, Lasjaunias P, et al.: Transient cerebral arteriopathy: a disorder recognized by serial angiograms in children with stroke. J Child Neurol 1998, 13:27–32.
Benseler SM, deVeber G, Hawkins C, et al.: Angiographynegative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum 2005, 52:2159–2167. Recognition of a novel disease entity in the spectrum of inflammatory brain diseases of childhood, angiography-negative small-vessel vasculitis.
Askalan R, Laughlin S, Mayank S, et al.: Chickenpox and stroke in childhood: a study of frequency and causation. Stroke 2001, 32:1257–1262.
Lanthier S, Armstrong D, Domi T, deVeber G: Post-varicella arteriopathy of childhood: natural history of vascular stenosis. Neurology 2005, 64:660–663.
Sebire G: Transient cerebral arteriopathy in childhood. Lancet 2006, 368:8–10.
White TM, Gilden DH, Mahalingam R: An animal model of varicella virus infection. Brain Pathol 2001, 11:475–479.
Bilge I, Sadikoglu B, Emre S, et al.: Central nervous system vasculitis secondary to parvovirus B19 infection in a pediatric renal transplant patient. Pediatr Nephrol 2005, 20:529–533.
Bosche B, Dohmen C, Impekoven P, et al.: Stroke after initiation of interferon-beta treatment for relapsing-remitting disseminated white matter disease. Acta Neurol Scand 2006, 113:433–436.
Gilden DH, Kleinschmidt-DeMasters BK, et al.: Neurologic complications of the reactivation of varicella-zoster virus. N Engl J Med 2000, 342:635–645.
Stone JH, Pomper MG, Roubenoff R, et al.: Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging. J Rheumatol 1994, 21:1277–1282.
Aviv RI, Benseler SM, Silverman ED, et al.: MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol 2006, 27:192–199. Comprehensive review of the MRI characteristics of children with cPACNS and the correlation of cPACNS parenchymal findings with affected cerebral vessels on angiography.
Alhalabi M, Moore PM: Serial angiography in isolated angiitis of the central nervous system. Neurology 1994, 44:1221–1226.
Aviv RI, Benseler SM, Silverman ED, et al.: Angiography of primary CNS vasculitis of childhood (cPACNS): Conventional angiography (CA) versus magnetic resonance angiography (MRA). AJNR Am J Neuroradiol 2006, in press.
Calabrese LH, Duna GF: Evaluation and treatment of central nervous system vasculitis. Curr Opin Rheumatol 1995, 7:37–44.
Zhang X, Zhu Z, Zhang F, et al.: Diagnostic value of singlephoton-emission computed tomography in severe central nervous system involvement of systemic lupus erythematosus: a case-control study. Arthritis Rheum 2005, 53:845–849.
Lowe LH, Morello FP, Jackson MA, Lasky A: Application of transcranial Doppler sonography in children with acute neurologic events due to primary cerebral and West Nile vasculitis. AJNR Am J Neuroradiol 2005, 26:1698–1701.
Lie JT: Primary (granulomatous) angiitis of the central nervous system: a clinicopathologic analysis of 15 new cases and a review of the literature. Hum Pathol 1992, 23:164–171.
Alrawi A, Trobe JD, Blaivas M, Musch DC: Brain biopsy in primary angiitis of the central nervous system. Neurology 1999, 53:858–860.
Lanthier S, Lortie A, Michaud J, et al.: Isolated angiitis of the CNS in children. Neurology 2001, 56:837–842.
Starke JR: Tuberculosis of the central nervous system in children. Semin Pediatr Neurol 1999, 6:318–331.
Akpek EK, Ahmed I, Hochberg FH, et al.: Intraocular-central nervous system lymphoma: clinical features, diagnosis, and outcomes. Ophthalmology 1999, 106:1805–1810.
Barron TF, Ostrov BE, Zimmerman RA, Packer RJ: Isolated angiitis of CNS: treatment with pulse cyclophosphamide. Pediatr Neurol 1993, 9:73–75.
Contreras G, Pardo V, Leclercq B, et al.: Sequential therapies for proliferative lupus nephritis. N Engl J Med 2004, 350:971–980.
Kleinschmidt-DeMasters BK, Gilden DH: Varicella-Zoster virus infections of the nervous system: clinical and pathologic correlates. Arch Pathol Lab Med 2001, 125:770–780.
deVeber GA, MacGregor D, Curtis R, Mayank S: Neurologic outcome in survivors of childhood arterial ischemic stroke and sinovenous thrombosis. J Child Neurol 2000, 15:316–324.
Calabrese LH, Gragg LA, Furlan AJ: Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system. J Rheumatol 1993, 20:2046–2050.
Sebire G, Meyer L, Chabrier S: Varicella as a risk factor for cerebral infarction in childhood: a case-control study. Ann Neurol 1999, 45:679–680.
Moriuchi H, Rodriguez W: Role of varicella-zoster virus in stroke syndromes. Pediatr Infect Dis J 2000, 19:648–653.
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Benseler, S.M. Central nervous system vasculitis in children. Curr Rheumatol Rep 8, 442–449 (2006). https://doi.org/10.1007/s11926-006-0040-4
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DOI: https://doi.org/10.1007/s11926-006-0040-4