Idiopathic reversible cerebral vasoconstriction syndrome (RCVS)

Abstract

Reversible cerebral vasoconstriction syndrome is characterised by severe thunderclap headache with associated characteristic transient, multifocal, segmental vasoconstriction of cerebral arteries lasting several weeks to months. We describe a 50-years old woman who presented with a severe sudden onset occipital headache. Neuroimaging revealed segmental vasospasm affecting the intracerebral arteries. The pain improved gradually over the next 6 weeks. Repeat brain MR angiography at 12 weeks showed complete resolution of the segmental narrowing.

Reversible cerebral vasoconstriction syndrome: literature review

“…It can affect any age group, including children [ 10 ] and young adolescents [ 7 ]. However, it primarily affects individuals between 20 and 50 years of age [ 1 , 2 , 9 – 11 , 13 , 20 , 38 , 39 ], with a peak around 42 years of age [ 7 , 40 ]. It is more common in women than in men, with incidence rates of 2.6:1 to 10:1 [ 1 , 5 – 7 , 10 , 12 , 17 , 20 , 28 , 41 – 43 ].…”

“…However, it primarily affects individuals between 20 and 50 years of age [ 1 , 2 , 9 – 11 , 13 , 20 , 38 , 39 ], with a peak around 42 years of age [ 7 , 40 ]. It is more common in women than in men, with incidence rates of 2.6:1 to 10:1 [ 1 , 5 – 7 , 10 , 12 , 17 , 20 , 28 , 41 – 43 ]. In men, it usually manifests a decade earlier than the average described for women [ 10 ].…”

“…The largest studies suggest that 50–80% of patients will have a secondary cause for RCVS [ 1 , 10 , 19 , 35 , 41 , 44 ]. The main precipitating factors described are: postpartum state [ 7 , 10 , 13 , 14 , 16 , 25 , 31 , 35 , 39 , 45 – 47 ], drugs with vasoactive properties [ 10 , 12 – 14 , 36 , 39 , 47 , 48 ], such as cocaine, marijuana, and heroin [ 33 , 39 ], catecholamine-secreting tumors [ 35 , 49 – 51 ], autoimmune disorders, such as systemic vasculitis, systemic lupus erythematous, antiphospholipid syndrome [ 13 ], blood transfusions, ginseng [ 5 , 6 ], sexual intercourse, temperature differences (baths too hot or too cold), air travel [ 39 , 48 ], Corona Virus Disease 2019 (COVID-19) infection [ 36 , 52 – 54 ] and medications [ 6 , 29 , 39 , 48 ].…”

“…Thunderclap headache has a reported prevalence between 95% and 100% in individuals diagnosed with RCVS [ 9 , 10 , 20 ] and may be the only clinical manifestation in 76–85% of patients [ 1 , 10 ]. Characteristically, headache is severe, sudden onset, and peaks in approximately 1 min [ 5 , 7 , 9 , 10 , 12 , 13 , 46 , 58 ].…”

“…The most common manifestation is thunderclap headache, which has a sudden onset and peak intensity in 1 min [ 5 , 9 , 12 , 13 ], and may be accompanied by nausea, vomiting, photophobia, and phonophobia [ 5 , 8 , 10 ]. In addition, focal neurological deficits may occur in 8–43% [ 1 , 14 ] and epileptic seizures in up to 17% of patients with RCVS [ 1 ].…”