Very rare. Neuroradiological entity, probably caused by a transient dysregulation of the arterial vascular tone, presenting with:

-        acute, bilateral, lightning headache (acute onset and maximal intensity in less than 5 minutes)

-        angioscan images of areas of vasoconstriction and vasodilation of cerebral arteries (appearance of 'string of sausages'); sometimes: reversible cerebral edema

There are frequent signs of intracranial hypertension at clinical examination.

Usual evolution: repetition of the headache during 1 to 4 weeks with frequent intercurrent painful background. Spontaneous disappearance in 3 to 4 weeks.

Triggers in children: physical exercise, Valsalva maneuver, vasoconstrictor agents, immersion in cold water.

In adults: sex, postpartum, cannabis or cocaine

Differential diagnosis: subarachnoid hemorrhage

Possible complications: subarachnoid or intracerebral hemorrhage.

Treatment: sometimes calcium channel blockers in order to prevent vasospasm.

Anesthetic implications:

maintain normocapnia as well as a normal cerebral perfusion pressure; risk of intracranial hypertension.

References :

• Trolliet M, Sevely A, Albucher J-F, Nasr N et al.
  Syndrome de vasoconstriction cérébrale réversible : une cause de céphalée en coup de tonnerre peu connue chez l’enfant.
  Arch Pédiatr 2016 ; 23 : 1254-9.

Updated: April 2017