Review Article

Reversible Cerebral Vasoconstriction Syndrome: An Important Cause of Acute Severe Headache

Table 3

Distinguishing features of RCVS, cervical artery dissection, PACNS and SAH [9].

RCVSCervical artery dissectionPACNSSAH

HistorySudden onset headache, often thunderclapSudden or subacute, can have thunderclap featuresInsidious, constant, progressive, dullSudden onset headache, often thunderclap
More common in femalesNo sex predilectionNo sex predilectionMore common in females
Age 20–50 years oldAge less than 50 years oldAge 40–60 years oldAge 40–60 years old
Risk increases with age
Likely to be younger in familial SAH

Risk factorsDrugs, pregnancy, tumours, neuro injury, idiopathicAtherosclerosis, cervical trauma, connective tissue disease. Can be idiopathicFamily history
Known cerebral aneurysm

ExaminationPresence or absence of neurological deficitPresence or absence of neurological deficit. Important to rule out in younger patients.Presence or absence of neurological deficit, 5% spinal involvementDepends on severity of haemorrhage

CT brainMajority normal
Cortical SAH, ICH
Normal in the absence of cerebral infarct (60%); crescenteric intramural haematoma on CTAMajority abnormal—diffuse, multiple small infarctsMajority abnormal.
SAH, cerebral oedema, hydrocephalus

CSF studiesMajority normalNormalMajority abnormal—raised protein, cell countAbnormal—xanthochromia, raised red cell count

MRI brainMajority normalMRA may reveal intramural haematoma as well as demonstrate flow abnormalities. More sensitive than CT or early infarctionNonspecific changes
Multifocal, cortical or subcortical infarcts, diffuse white matter changes, or leptomeningeal enhancement
Areas of infarct corresponding to vascular territory involved

Cerebral angiographyConsidered gold standard.
Useful in recurrent TCH
Diffuse segmental stenosis—medium, large arteries
Long-segmental stenosis, intimal flaps, arterial pseudoaneurysmUnable to visualise changes in small arteriesAneurysm, arterio-venous malformation
Vasospasm (not multifocal) at Day 4

CNS biopsyNot indicatedGold standard.
Skip, segmental vascular lesions