Synonyms:
Reversible cerebral vasoconstriction syndrome RCVS a.k.a. Call-Fleming syndrome a.k.a. benign angiopathy of the CNS a.k.a. thunderclap headache with vasoconstriction
Diagnosis:
Clinical:
- Female >Male, thunderclap headache with out without neurological symptoms.
- Hemiplegia, visual field defects,
- May be postpartum
- +Imaging
Findings on investigations:
CT: infarcts usually posterior/watershed or hemorrhagic transformation, no SAH
MRI: infarcts usually posterior/watershed or hemorrhagic transformation or similar to posterior reversible leucoencephalopathy
+CTA, MRA or Catheter Angiogram:
- Segmental vasoconstriction, multifocal.
- +Resolution within 12 weeks.
- Catheter angiography is the gold standard
- CTA, MRA have poor sensitivity
Transcranial Doppler TCD: increased velocities
CSF: normal, no SAH, no pleocytosis (helps differentiate from PACNS)
Brain biopsy: no vasculitis, may have reactive gliosis (helps differentiate from PACNS)
Investigations to consider:
Vasculitis screen: ESR, CRP, ANA screen, ENA panel (anti- dsDNA, anti-Sm, anti-RNP, SSA, SSB, anti-Jo-1, antitopoisomerase ‘formerly anti Scl-70’, antinucleolar, anticentromere), ANCA, Complement C3, C4 and CH50
Catheter angiogram
MRI brain with and without contrast
Consider lumbar puncture and CSF analysis
Treatment:
Calcium channel blockers:
- Verapamil
- Or Nimodipine