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OP-129 Primary central nervous system vasculitis in children
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  1. Zsófia Szkiba1,
  2. Beáta Rosdy1,
  3. Orsolya Dancsó1,
  4. György Balázs2,
  5. Gabriella Vértesi3
  1. 1Heim Pál National Pediatric Institute, Department of Neurology, Budapest, Hungary
  2. 2Heim Pál National Pediatric Institute, Center of MRI and CT Diagnostics, Budapest, Hungary
  3. 3Heim Pál National Pediatric Institute, Department of Internal Medicine, Budapest, Hungary

Abstract

Aim Childhood primary cerebral vasculitis is an immune-mediated inflammatory disease of the CNS, which can affect small-, medium- and large vessels and may lead to acute ischemic stroke, among other things.

Material and Method -

Results We are presenting a currently 18-year-old adolescent who started having migraine episodes with right superior quadrantanopia as an aura from the age of 11. We admitted her for the first time at the age of 13 with increasingly frequent migraines, isolated auras and persistent visual field loss on the day of her admission. MRI and MRA examinations of the head and neck showed multiple ischemic stroke but no abnormal calibre difference, vessel wall enhancement or any signs of dissection. After excluding other possible etiologies, we suspected the presence of cerebral vasculitis. A few weeks after starting steroids, a repeat MRI was performed due to recurrent unilateral limb weakness, which revealed segmental filling defect in the C1 section of the left vertebral artery, which finding however seemed to support the existence of dissection. A recanalized vertebral artery was detected during subsequent control examinations. 2,5 years after her first admission, the number of migraine episodes began to increase again, with no new intracranial lesions on the control MRIs taken during each presentation. However, since the isolated auras continued to occur frequently, a repeat head and neck MRI was performed, which showed novum ischemic lesions, and the bilateral vertebral and basilar arteries were significantly more narrow and irregular with left-sided predominance compared to the previous images. Thus, based on the clinical signs and radiological findings, the probability of vasculitis was once again highlighted.

Conclusions Considering all of this, the question may arise whether an injury to the weakened and inflamed vessel wall due to the already present vasculitis could have led to the dissection of the vertebral artery after also causing ischemic stroke.

  • primary central nervous system vasculitis
  • acute ischemic stroke
  • arterial dissection

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