Pediatric Case of Life-Threatening Stroke Caused by Reversible Cerebral Vasoconstriction Syndrome with Spontaneous Cervical Internal Carotid Artery Vasospasm: A Case Report.

ABSTRACT

Pediatric reversible cerebral vasoconstriction syndrome (RCVS) and spontaneous cervical internal carotid artery (ICA) vasospasm are rare conditions; the former is commonly associated with a favorable prognosis. A healthy 13-year-old girl presented with thunderclap headache, followed by left hemiparesis, during a curling match. Six days after onset, left hemiparesis worsened to hemiplegia. Magnetic resonance imaging showed progressive cerebral infarction caused by severe right middle cerebral artery and cervical ICA stenosis. She became comatose because of impending uncal herniation. Emergent surgical decompression was performed. Then, 59 days after onset, her multiple stenoses improved, which was consistent with RCVS concomitant with spontaneous cervical ICA vasospasm. This is the first case of RCVS that concurrently developed spontaneous cervical ICA vasospasm. The patient developed life-threatening stroke due to the hemodynamic impairment of the affected intracranial and cervical arteries. Spontaneous extracranial supra-aortic artery vasospasm can be a poor prognostic predictor of RCVS.