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Reversible Cerebral Vasoconstriction Syndrome (RCVS)

Summary

  • RCVS is characterised by severe thunderclap headaches and reversible multifocal cerebral arterial constriction
  • Typically affects women aged 20-50 years
  • Diagnosis relies on clinical presentation, exclusion of other causes, and neuroimaging findings

Pathophysiology

  • Exact mechanism unclear, but involves transient dysregulation of cerebral vascular tone
  • Proposed triggers:
    • Sympathetic overactivity
    • Endothelial dysfunction
    • Oxidative stress
  • Associated with:
    • Vasoactive substances (e.g., serotonergic drugs, cannabis)
    • Postpartum state
    • Migraine

Demographics

  • Predominantly affects women (F:M ratio 2-10:1)
  • Peak incidence: 20-50 years of age
  • Rare in children and elderly

Diagnosis

  • Clinical features:
    • Sudden-onset, severe 'thunderclap' headaches
    • Nausea, vomiting, photophobia
    • Focal neurological deficits (in some cases)
  • Diagnostic criteria (International Classification of Headache Disorders-3):
    1. Acute severe headache, often thunderclap-like
    2. Multifocal segmental vasoconstriction of cerebral arteries
    3. No evidence of aneurysmal subarachnoid haemorrhage
    4. Normal or near-normal CSF analysis
    5. Complete or substantial normalisation of arteries within 3 months

Imaging

  • CT brain:
    • Initially normal in most cases
    • May show subarachnoid haemorrhage, intracerebral haemorrhage, or ischaemic infarction in complicated cases
  • CT angiography:
    • 'String of beads' appearance of cerebral arteries
    • Multifocal segmental narrowing of cerebral arteries
  • MRI brain:
    • T2/FLAIR: may show vasogenic oedema, especially in posterior regions
    • DWI: may reveal acute ischaemic changes
  • MR angiography:
    • Similar findings to CT angiography
    • Useful for follow-up imaging to demonstrate reversibility
  • Digital subtraction angiography:
    • Gold standard for diagnosis
    • Shows characteristic multifocal segmental arterial constriction
    • Useful when non-invasive imaging is inconclusive

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  • 25-year-old patient had an emergency c-section for eclampsia.
  • MRI was performed after the patient developed an occipital headache, visual disturbance, and vomiting.
  • MRI showed cortical and subcortical T2 and FLAIR hyperintensity and a trace of subarachnoid blood.
  • MRA showed stenoses and irregularity of the A2 ACAs and the right M2 MCAs.
  • The findings fully resolved on imaging 3 months later.

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  • A 40-year-old patient presented with a severe occipital and temporal headache, nausea, vomitting and dizziness.
  • MRI showed focal stenoses in the M2 MCAs and both PCAs, which resolved after 6 months.

Treatment

  • Supportive care and removal of potential triggers
  • Calcium channel blockers:
    • Nimodipine: first-line treatment (60mg every 4-8 hours for 4-12 weeks)
    • Verapamil: alternative option
  • Short course of glucocorticoids in severe cases
  • Management of complications:
    • Anticonvulsants for seizures
    • Blood pressure control for hypertension
  • Follow-up imaging at 3 months to confirm resolution of vasoconstriction
  • Patient education on avoiding triggers and recognising symptoms

Differential diagnosis

Differential Diagnosis Distinguishing Feature
Aneurysmal SAH with vasospasm Aneurysm visible on CTA/DSA; diffuse basilar subarachnoid blood on CT; vasospasm develops 4-14 days after ictus rather than at headache onset
Primary angiitis of CNS (PACNS) Irreversible arterial narrowing on follow-up MRA; vessel wall enhancement on high-resolution MRI; cortical and subcortical infarcts in multiple vascular territories
Posterior reversible encephalopathy syndrome Posterior-predominant vasogenic oedema on T2/FLAIR; elevated ADC values (not restricted); no multifocal arterial beading on MRA
Cerebral venous sinus thrombosis Filling defects in dural venous sinuses on CT/MR venography; venous infarcts crossing arterial territories; no arterial beading
Cervical arterial dissection Crescentic intramural haematoma on T1 fat-saturated images; eccentric arterial narrowing; may show double lumen
Infectious vasculitis (fungal or bacterial meningitis) Basilar meningeal enhancement; multifocal perforator territory infarcts; stenosis does not resolve on follow-up imaging