A Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome

Abstract

BACKGROUND Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disease that is classified as a condition of diffuse cerebral artery constriction. RCVS can be complicated with transient neurological deficits, seizures, ischemic strokes, and hemorrhagic strokes. A thunder-clap headache, described as being the worst headache a patient can experience, is the predominant symptom in RCVS, which contributes to why RCVS is underdiagnosed as an ischemic stroke or migraine. CASE REPORT In this case study, we present a healthy 34-year-old Black woman who presented to the Emergency Department 3 times over a period of 4 days with concerns of severe headaches. In her first Emergency Department visit, she had a normal computed tomography scan of the brain. Her third Emergency Department visit resulted in hospitalization due to seizures, and a computed tomography brain scan done then showed acute intracranial hemorrhaging. The patient ultimately received a diagnosis of RCVS during her hospitalization. CONCLUSIONS RCVS is the most notable mimicker of other similar-presenting vasculopathies, such as primary angiitis of the central nervous system and posterior reversible encephalopathy syndrome. It is important to note that thunder-clap headache, as well as complications such as intracranial hemorrhaging and seizures, can arise not just from other diseases but from RCVS as well; hence, an early diagnosis is critical to avoid complications, especially if initial imaging is negative.

Figure 1.

Computed tomography scan of the brain without contrast. Focal parenchymal hemorrhage within the paramedian left parietal lobe (yellow cross) is shown.

Figure 2.

Computed tomography scan of the brain without contrast. Focal parenchymal hemorrhage within the paramedian posterior left occipital lobe (yellow cross) is shown.

Figure 3.

Magnetic resonance imaging shows a parenchymal hematoma in the left paramedial parietal lobe (yellow arrow).

Figure 4.

Digital subtraction angiography of the left internal carotid artery, lateral view. Red arrows represent multifocal irregularities of the left middle cerebral artery branches. The red oval represents the magnified view of the area of interest, concerning for reversible vasoconstrictive syndrome.

Figure 5.

Computed tomography scan of the brain showing that the previously noted hemorrhages are resolved.

Figure 6.

Patient timeline.