Retinal Patterns in Reversible Cerebral Vasoconstriction Syndrome
Condition: Reversible Cerebrovascular Vasoconstriction Syndrome
MeSH terms: Syndrome
Study type: Observational

Summary: 
      Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico radiological entity
      characterized by severe headaches (associated or not with neurological complications) during
      one to 3 weeks, associated with a characteristic 'string and beads' appearance on cerebral
      arteries, which resolves spontaneously in 3 months. The pathway is unknown. At early stage of
      the disease (at the first medical consultation) cerebral arterial abnormalities which are
      necessary for diagnosis are identified in only 20% of patients (brain magnetic resonance
      imagery (MRI) ,CT scan angiography), appearing with a delay on 2th or 3rd week after the
      first severe headache..

      Retinal artery network is considered to be a window on brain microvasculature by sharing the
      same embryologic origin and physiopathology. A retinal arteriolar examination at early stage
      of RCVS could provide non invasively early clue to confirm diagnosis by identifying
      anatomical change and /or functional abnormalities at the microvascular level, whereas large
      cerebral artery abnormalities are still normal.
    

Detailed description: 
      Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico radiological entity
      characterized by severe headaches (associated or not with neurological complications) during
      one to 3 weeks, associated with a characteristic 'string and beads' appearance on cerebral
      arteries, which resolves spontaneously in 3 months.

      The pathway is unknown. One strong hypothesis is that RCVS is a vasospasm and-vasodilatation
      disorder starting from small distal cerebral arteries progressing toward to medium sized and
      large sized cerebral arteries, and disappearing in 3 months.

      At early stage of the disease (generally at the first medical consultation round 7 days after
      the first headache), arterial caliber anomalies cannot be identified on usual investigation
      (brain MRI, angioscan) in most of the case (80%). They are appearing secondary on repeated
      angiogram around the 2nd week or 3rd week, permitting to confirm the diagnosis, but with
      delay. Currently, small cerebral vessel arteries can't be studied directly . Retinal artery
      network is easy to study. It is considered to be a window on brain microvasculature by
      sharing the same embryologic origin and physiopathology. The investigators thus hypothesized
      that retinal arteriolar examination a early stage of RCVS could provide non invasively early
      clue to confirm diagnosis by identifying anatomical change and /or functional abnormalities
      at the microvascular level, whereas large cerebral artery abnormalities are still normal.

      Hypothesis Arteriolar caliber and vasoreactivity abnormalities at the retinal microvascular
      level could be an early, non invasive and sensitive diagnostic marker of the RCVS at the
      first medical consultation in emergency.
    

Observational model: Cohort
Time perspective: Prospective
Sampling method: Probability Sample
Study population: 
        male and female with Reversible cerebral vasoconstriction syndrome
      
Keywords: Reversible cerebral vasoconstriction syndrome; Anatomic and morphological retinal network analysis; Retinal vessel analyser; retinography; Prospective analysis