Journal of Cerebrovascular and Endovascular Neurosurgery

Korean Society of Cerebravascular Surgeons (대한뇌혈관외과학회)
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Primary Angiitis of Central Nervous System related intracranial aneurysm with spontaneous occlusion after immunomodulatory treatment

  • Seby John (Department of Neurology and Neurointerventional Surgery, Neurological Institute (NI), Cleveland Clinic Abu Dhabi (CCAD)) ;
  • Muhammad Khan (Department of Biomedical Engineering, Khalifa University) ;
  • Praveen Kesav (Department of Neurology, Neurological Institute (NI), Cleveland Clinic Abu Dhabi (CCAD)) ;
  • Divya Raj (Department of Neurology, Neurological Institute (NI), Cleveland Clinic Abu Dhabi (CCAD)) ;
  • Syed Irteza Hussain (Department of Neurology and Neurointerventional Surgery, Neurological Institute (NI), Cleveland Clinic Abu Dhabi (CCAD))
  • Received : 2023.04.14
  • Accepted : 2024.01.02
  • Published : 2024.09.30
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Abstract

Primary Angiitis of the Central Nervous System (PACNS) is an uncommon disease with kaleidoscopic clinical manifestations. Ischemic strokes are commoner than their hemorrhagic counterpart. Intracranial pseudoaneurysms are rarely reported in PACNS cohorts. We hereby describe the case of a 39-year-old female, who presented for evaluation of acute onset of left middle cerebral artery (MCA) ischemic stroke, with cerebral angiogram showing multifocal stenosis and irregularities in intracranial blood vessels with an aneurysm arising from the lenticulostriate branch of the left MCA M1 segment. A diagnosis of probable PACNS was made and patient initiated on immunomodulatory treatment with corticosteroids. 12 weeks follow up neuroimaging studies revealed resolution of the previously described intracranial aneurysm, thereby postulating the possibility of a pseudoaneurysm related to the underlying angiitis.

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References

  1. Calabrese LH, Duna GF, Lie JT. Vasculitis in the central nervous system. Arthritis Rheum. 1997 Jul;40(7):1189-201. 
  2. de Boysson H, Zuber M, Naggara O, Neau JP, Gray F, Bousser MG, et al. Primary angiitis of the central nervous system: Description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol. 2014 May;66(5):1315-26. 
  3. Gaillard N, Bertrand JL, Dumitrana A, Sablot D. Microaneurysms in primary angiitis of the central nervous system revealed by MRI. Cerebrovasc Dis. 2014;38(4):308-10. 
  4. Pillai SH, Sreedharan SE, Menon G, Kannoth S, Pn S. Primary CNS vasculitis presenting as intraventricular bleeding. Ann Indian Acad Neurol. 2016 Jul-Sep;19(3):406-8. 
  5. Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB. CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. AJNR Am J Neuroradiol. 1999 Jan;20(1):75-85. 
  6. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Huston J 3rd, Meschia JF, et al. Primary central nervous system vasculitis presenting with intracranial hemorrhage. Arthritis Rheum. 2011 Nov;63(11):3598-606. 
  7. Salvarani C, Brown RD Jr, Calamia KT, Christianson TJ, Weigand SD, Miller DV, et al. Primary central nervous system vasculitis: Analysis of 101 patients. Ann Neurol. 2007 Nov;62(5):442-51. 
  8. Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet. 2012 Aug;380(9843):767-77. 
  9. Zheng Y, Lu Z, Shen J, Xu F. Intracranial pseudoaneurysms: Evaluation and management. Front Neurol. 2020 Jul;11:582.