Autopsy and Case Reports
https://www.autopsyandcasereports.org/article/doi/10.4322/acr.2021.391
Autopsy and Case Reports
Autopsy Case Report

Vertebrobasilar circulation hemorrhages in childhood primary angiitis of the central nervous system

Ahmad Hassan; Kieren Allinson

Downloads: 1
Views: 647

Abstract

Childhood primary angiitis of the CNS (cPACNS) is a poorly understood, rare, and diagnostically challenging neurologic disease. We describe an unusual and autopsy-confirmed case of cPACNS presenting as vertebrobasilar circulation hemorrhagic strokes in a 4-year-old girl. The presentation and clinical features were inconsistent with primary CNS vasculitis and skewed the diagnosis. Autopsy and histopathological analyses revealed a progressive lymphocytic vasculitis affecting the medium to large vessels of vertebrobasilar circulation and sparing the anterior circulation. It is imperative to raise the index of suspicion for cPACNS in any case of unusual or unexplained neurological presentation, especially in the absence of cerebrovascular risk factors and/or coagulation disorders.

Keywords

Autopsy, Brain Death, Vasculitis, Central Nervous System, Diagnosis, Hemorrhagic Stroke, Mortality

References

1 Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum. 1997;40(7):1189-201. http://dx.doi.org/10.1002/1529-0131(199707)40:7<1189::AID-ART2>3.0.CO;2-4. PMid:9214418.

2 Beelen J, Benseler SM, Dropol A, Ghali B, Twilt M. Strategies for treatment of childhood primary angiitis of the central nervous system. Neurol Neuroimmunol Neuroinflamm. 2019;6(4):e567. http://dx.doi.org/10.1212/NXI.0000000000000567. PMid:31355303.

3 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. http://dx.doi.org/10.3389/fped.2021.654537. PMid:33842414.

4 Scolding NJ, Wilson H, Hohlfeld R, Polman C, Leite I, Gilhus N. The recognition, diagnosis and management of cerebral vasculitis: a European survey. Eur J Neurol. 2002;9(4):343-7. http://dx.doi.org/10.1046/j.1468-1331.2002.00422.x. PMid:12099915.

5 Smitka M, Bruck N, Engellandt K, Hahn G, Knoefler R, von der Hagen M. Clinical perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS). Front Pediatr. 2020;8:281. http://dx.doi.org/10.3389/fped.2020.00281. PMid:32719754.

6 Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol. 2011;10(6):561-72. http://dx.doi.org/10.1016/S1474-4422(11)70081-3. PMid:21601163.

7 Benseler SM, Silverman E, Aviv RI, et al. Primary central nervous system vasculitis in children. Arthritis Rheum. 2006;54(4):1291-7. http://dx.doi.org/10.1002/art.21766. PMid:16575852.

8 Shiner EA, Zagami AS. An illustrative case of primary angiitis of the central nervous system. SAGE Open Med Case Rep. 2014;2:X14559638. http://dx.doi.org/10.1177/2050313X14559638. PMid:27489666.

9 Cellucci T, Benseler SM. Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr. 2010;22(6):731-8. http://dx.doi.org/10.1097/MOP.0b013e3283402d4c. PMid:21610331.

10 Alhaboob AA, Hasan GM, Malik MA, Rehman MZ. Therapeutic benefits and side effects of Azathioprine and Aspirin in treatment of childhood primary arterial stroke. Ann Neurosci. 2014;21(1):10-3. http://dx.doi.org/10.5214/ans.0972.7531.210104. PMid:25206047.

11 Harbitz F. Unknown forms of arteritis, with special reference to their relation to syphilitic arteritis and periarteritis nodosa. Am J Med Sci. 1922;163(2):250-71. http://dx.doi.org/10.1097/00000441-192202000-00010.

12 Elbers J, Halliday W, Hawkins C, Hutchinson C, Benseler SM. Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol. 2010;68(5):602-10. http://dx.doi.org/10.1002/ana.22075. PMid:21031577.

13 Beuker C, Schmidt A, Strunk D, et al. Primary angiitis of the central nervous system: diagnosis and treatment. Ther Adv Neurol Disord. 2018;11:1756286418785071. http://dx.doi.org/10.1177/1756286418785071. PMid:30034536.

14 Aviv RI, Benseler SM, Silverman ED, et al. MR imaging and angiography of primary CNS vasculitis of childhood. AJNR Am J Neuroradiol. 2006;27(1):192-9. PMid:16418382.

15 Malik MA, Zia-ur-Rehman M, Nadeem MM, et al. Childhood primary angiitis of the central nervous system. J Coll Physicians Surg Pak. 2012;22(9):570-4. PMid:22980610.

16 Pistracher K, Gellner V, Riegler S, Schökler B, Scarpatetti M, Kurschel S. Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis: a review. Childs Nerv Syst. 2012;28(8):1141-8. PMid:22684518.

17 Rice CM, Scolding NJ. The diagnosis of primary central nervous system vasculitis. Pract Neurol. 2020;20(2):109-14. http://dx.doi.org/10.1136/practneurol-2018-002002. PMid:31649101.

18 Eleftheriou D, Cox T, Saunders D, Klein NJ, Brogan PA, Ganesan V. Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography. Dev Med Child Neurol. 2010;52(9):863-7. http://dx.doi.org/10.1111/j.1469-8749.2009.03591.x. PMid:20132140.

19 Berlit P. Primary angiitis of the CNS: an enigma that needs world-wide efforts to be solved. Eur J Neurol. 2009;16(1):10-1. http://dx.doi.org/10.1111/j.1468-1331.2008.02348.x. PMid:19087144.

20 Salvarani C, Brown RDJ Jr, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007;62(5):442-51. http://dx.doi.org/10.1002/ana.21226. PMid:17924545.

21 Woolfenden AR, Tong DC, Marks MP, Ali AO, Albers GW. Angiographically defined primary angiitis of the CNS: is it really benign? Neurology. 1998;51(1):183-8. http://dx.doi.org/10.1212/WNL.51.1.183. PMid:9674800.

22 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(3):860-2. http://dx.doi.org/10.1161/STROKEAHA.111.626184. PMid:22156692.

23 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. 2012;51(10):1838-45. http://dx.doi.org/10.1093/rheumatology/kes156. PMid:22740622.
 


Submitted date:
10/18/2021

Accepted date:
07/02/2022

Publication date:
07/15/2022

62d157e9a9539563e43eb7f2 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections