Santral Sinir Sistemi Vaskülitleri
Özet
Çocukluk çağı santral sinir sistemi primer anjiiti (cPACNS), santral sinir sistemindeki kan damarlarının izole inflamasyonu olup başlangıçta sıklıkla yanlış tanı alabilmektedir. Hastalık, tutulan damar çapı ve anjiyografik özelliklere göre anjiyografi pozitif (orta-büyük damar) ve negatif (küçük damar) olmak üzere sınıflandırılmaktadır. Nöbet, inme benzeri semptomlar ve bilişsel bozukluklar gibi geniş bir klinik tabloyla seyreden bu durum, erken dönemde tanınıp tedavi edildiğinde geri dönüşümlüdür. Kesin tanı için benzer klinik bulgular gösteren enfeksiyöz ve otoimmün hastalıkların ayırıcı tanısının titizlikle yapılması gerekmektedir.
Pediatric primary angiitis of the central nervous system (cPACNS) involves isolated inflammation of blood vessels within the CNS and carries a significant risk of initial misdiagnosis. Classification is based on vessel diameter and angiographic findings, distinguishing between angiographic-positive (medium-to-large vessel) and angiographic-negative (small vessel) types. Presenting with diverse symptoms such as seizures, stroke-like episodes, and cognitive impairment, the condition is reversible if detected and treated early. Accurate diagnosis requires careful exclusion of other infectious and autoimmune conditions that may mimic the clinical presentation.
Referanslar
Salvarani C, Brown RD, Jr., Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007; 62: 442-451. 2007/10/11. DOI: 10.1002/ana.21226.
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: 52-57. DOI: 10.1161/STROKEAHA.108.521203.
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: 2159-2167. 2005/06/30. DOI: 10.1002/art.21144.
Benseler SM, Silverman E, Aviv RI, et al. Primary central nervous system vasculitis in children. Arthritis Rheum 2006; 54: 1291-1297. 2006/04/01. DOI: 10.1002/art.21766.
Twilt M and Benseler SM. Central nervous system vasculitis in adults and children. Handb Clin Neurol 2016; 133: 283-300. 2016/04/27. DOI: 10.1016/b978-0-444-63432-0.00016-5.
Cellucci T, Tyrrell PN, Sheikh S, et al. Childhood primary angiitis of the central nervous system: identifying disease trajectories and early risk factors for persistently higher disease activity. Arthritis Rheum 2012; 64: 1665-1672. 2012/05/01. DOI: 10.1002/art.34527.
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: 1078-1084. 2010/10/05. DOI: 10.1016/s1474-4422(10)70243-x.
Calabrese LH and 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. 1988/01/01. DOI: 10.1097/00005792-198801000-00002.
Cellucci T, Tyrrell PN, Pullenayegum E, et al. von Willebrand factor antigen--a possible biomarker of disease activity in childhood central nervous system vasculitis? Rheumatology (Oxford) 2012; 51: 1838-1845. 2012/06/29. DOI: 10.1093/rheumatology/kes156.
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. 2006/01/19.
Küker W, Gaertner S, Nagele T, et al. Vessel wall contrast enhancement: a diagnostic sign of cerebral vasculitis. Cerebrovasc Dis 2008; 26: 23-29. 2008/05/31. DOI: 10.1159/000135649.
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: 863-867. 2010/02/06. DOI: 10.1111/j.1469-8749.2009.03591.x.
Elbers J, Halliday W, Hawkins C, et al. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol 2010; 68: 602-610. 2010/10/30. DOI: 10.1002/ana.22075.
Twilt M and Benseler SM. The spectrum of CNS vasculitis in children and adults. Nat Rev Rheumatol 2011; 8: 97-107. 2011/12/21. DOI: 10.1038/nrrheum.2011.197.
Quan AS, Brunner J, Rose B, et al. Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey. Front Pediatr 2021; 9: 654537. 2021/04/13. DOI: 10.3389/fped.2021.654537.
Beelen J, Benseler SM, Dropol A, et al. Strategies for treatment of childhood primary angiitis of the central nervous system. Neurol Neuroimmunol Neuroinflamm 2019; 6: e567. 2019/07/30. DOI: 10.1212/nxi.0000000000000567.
Smitka M, Bruck N, Engellandt K, et al. Clinical Perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS). Front Pediatr 2020; 8: 281. 2020/07/29. DOI: 10.3389/fped.2020.00281.
Gowdie P, Twilt M and Benseler SM. Primary and secondary central nervous system vasculitis. J Child Neurol 2012; 27: 1448-1459. 2012/09/26. DOI: 10.1177/0883073812459352.
