Reversible Cerebral Vasoconstriction Syndrome with Massive Ischemic Brain Infarcts and Poor Clinical Outcome

A 58-year-old female patient presented with elevated arterial blood pressure, severe occipital headaches, personality change, and left hemiparesis. Her medical history was remarkable for depression and arterial hypertension. Her usual medication included escitalopram (a selective serotonin reuptake inhibitor antidepressant) and antihypertensive drugs. Cranial computed tomography (CT) showed a small hemorrhage in the right parietal cortex and cortical swelling. CT angiography revealed caliber irregularities in all major cerebral arteries, with a “string of beads” pattern. Transcranial Doppler ultrasonography showed peak flow values of 160 cm/sec for both middle cerebral arteries. The only notable laboratory value was a C-reactive protein level of 1.9 mg/dl. The initial diagnoses included cerebral vasculitis and reversible cerebral vasoconstriction syndrome. The patient received 1 g paracetamol, 1 g plus 2 g metamizole, 20 mg urapidil, and 0.075 mg clonidine IV. After her condition stabilized, she was referred for digital subtraction angiography (DSA) and possible endovascular treatment. DSA on day 2 after clinical onset confirmed the beaded caliber irregularities in most cerebral arteries. No aneurysm or arteriovenous shunt was found. The patient remained intubated, sedated, and ventilated from day 2 to day 20. Nimodipine at 5 mg/h IV was started on day 2. A generalized epileptic seizure on day 2 was treated with 1 mg midazolam IV, followed by 3000 mg levetiracetam IV. Prednisolone (300 mg/d IV) was given from day 6 for 4 days after administration of a bolus of 1000 mg IV on day 5. CT and magnetic resonance imaging examinations during the first 2 weeks demonstrated a progressive evolution of infarcts in the frontal and parieto-occipital region and the posterior inferior cerebellar artery territory bilaterally. DSA on day 10 confirmed substantial regression of the caliber abnormalities of the cerebral arteries. The final DSA examination 11 weeks after the clinical onset showed nearly normal cerebral arteries. The patient was transferred for rehabilitation 19 days after clinical onset in a comatose state and remained in a vegetative state. At the last clinical follow-up 1 year after the ictus, the patient showed tetraparesis with severe cognitive deficits and was in need of care (modified Rankin scale score of 5). Diagnosis and treatment of reversible cerebral vasoconstriction syndrome is the main topic of this chapter.