Childhood primary angiitis of the central nervous system
- Marinka Twilt, MD, PhD
Marinka Twilt, MD, PhD
- Assistant Professor of Pediatrics
- University of Calgary, Alberta
- Susanne Benseler, MD, PhD
Susanne Benseler, MD, PhD
- Associate Professor of Pediatrics
- University of Calgary
- Calgary, Alberta, Canada
- Section Editors
- Robert Sundel, MD
Robert Sundel, MD
- Section Editor — Pediatric Rheumatology
- Associate Professor of Pediatrics
- Harvard Medical School
- Douglas R Nordli, Jr, MD
Douglas R Nordli, Jr, MD
- Section Editor — Pediatric Neurology
- Chief of Neurology
- Children’s Hospital Los Angeles
- Vice Chair of Neurology
- USC Keck School of Medicine
Childhood primary angiitis of the central nervous system (cPACNS) is a rare form of central nervous system (CNS) vasculitis or inflammatory brain disease affecting children. It typically presents with neurologic deficits, often a stroke-like event, and/or psychiatric symptoms. The diagnostic evaluation focuses on ruling out other forms of inflammatory brain disease and noninflammatory vasculopathies in addition to performing confirmatory studies, such as brain magnetic resonance imaging (MRI) and angiography. Treatment includes anticoagulation and/or antiinflammatory therapy to prevent disease progression and complications.
A general overview of childhood vasculitis is discussed in detail separately, as is primary angiitis of the central nervous system (PACNS) in adults. (See "Vasculitis in children: Classification and incidence" and "Vasculitis in children: Evaluation" and "Vasculitis in children: Management overview" and "Primary angiitis of the central nervous system in adults".)
cPACNS criteria are based upon the modified Calabrese criteria for adult PACNS and include [1-3]:
●Newly acquired, otherwise unexplained neurologic or psychiatric symptom in a patient less than 18 years of age
●Angiographic and/or histopathologic features of central nervous system (CNS) angiitisTo continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Benseler SM, Silverman E, Aviv RI, et al. Primary central nervous system vasculitis in children. Arthritis Rheum 2006; 54:1291.
- Benseler SM, deVeber G, Hawkins C, et al. Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum 2005; 52:2159.
- 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.
- Hajj-Ali RA, Calabrese LH. Central nervous system vasculitis. Curr Opin Rheumatol 2009; 21:10.
- Golomb MR, Fullerton HJ, Nowak-Gottl U, et al. Male predominance in childhood ischemic stroke: findings from the international pediatric stroke study. Stroke 2009; 40:52.
- Hutchinson C, Elbers J, Halliday W, et al. Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol 2010; 9:1078.
- Elbers J, Halliday W, Hawkins C, et al. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol 2010; 68:602.
- 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.
- Lanthier S. Primary angiitis of the central nervous system in children: 10 cases proven by biopsy. J Rheumatol 2002; 29:1575.
- 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.
- Aviv RI, Benseler SM, DeVeber G, et al. Angiography of primary central nervous system angiitis of childhood: conventional angiography versus magnetic resonance angiography at presentation. AJNR Am J Neuroradiol 2007; 28:9.
- Eleftheriou D, Cox T, Saunders D, et al. Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography. Dev Med Child Neurol 2010; 52:863.
- Golomb MR. Stroke: TOAST to CASCADE--a childhood stroke classification system. Nat Rev Neurol 2012; 8:184.
- Kleinschmidt-DeMasters BK, Gilden DH. Varicella-Zoster virus infections of the nervous system: clinical and pathologic correlates. Arch Pathol Lab Med 2001; 125:770.
- Küker W, Gaertner S, Nagele T, et al. Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis 2008; 26:23.
- Mandell DM, Matouk CC, Farb RI, et al. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke 2012; 43:860.
- Matsell DG, Keene DL, Jimenez C, Humphreys P. Isolated angiitis of the central nervous system in childhood. Can J Neurol Sci 1990; 17:151.
- Twilt M, Benseler SM. The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol 2011; 8:97.
- Néel A, Pagnoux C. Primary angiitis of the central nervous system. Clin Exp Rheumatol 2009; 27:S95.
- Verhey LH, Branson HM, Laughlin S, et al. Development of a standardized MRI scoring tool for CNS demyelination in children. AJNR Am J Neuroradiol 2013; 34:1271.
- Venkateswaran S, Hawkins C, Wassmer E. Diagnostic yield of brain biopsies in children presenting to neurology. J Child Neurol 2008; 23:253.
- Cellucci T, Benseler SM. Central nervous system vasculitis in children. Curr Opin Rheumatol 2010; 22:590.
- Cellucci T, Benseler SM. Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr 2010; 22:731.
- Barron TF, Ostrov BE, Zimmerman RA, Packer RJ. Isolated angiitis of CNS: treatment with pulse cyclophosphamide. Pediatr Neurol 1993; 9:73.
- Batthish M, Banwell B, Laughlin S, et al. Refractory primary central nervous system vasculitis of childhood: successful treatment with infliximab. J Rheumatol 2012; 39:2227.
- Lo W, Gordon AL, Hajek C, et al. Pediatric stroke outcome measure: predictor of multiple impairments in childhood stroke. J Child Neurol 2014; 29:1524.
- Cellucci T, Tyrrell PN, Pullenayegum E, Benseler SM. von Willebrand factor antigen--a possible biomarker of disease activity in childhood central nervous system vasculitis? Rheumatology (Oxford) 2012; 51:1838.
- CLINICAL FEATURES
- Angiography-positive, nonprogressive cPACNS
- Angiography-positive, progressive cPACNS
- Angiography-negative, brain biopsy-positive, small-vessel cPACNS
- DIFFERENTIAL DIAGNOSIS
- Psychosis and behavioral complications