Reversible Cerebral Vasoconstriction Syndrome with Atypical Subarachnoid Hemorrhage: A First Case Report

Reversible cerebral vasoconstriction syndrome represents a group of syndromes characterised by prolonged, reversible, segmental vasoconstriction of the cerebral arteries, usually associated with acute, severe, “thunderclap” headache with or without additional focal neurological deficits and seizures. The major complications of this syndrome are localised cortical subarachnoid haemorrhages and ischaemic or haemorrhagic strokes. Subarachnoidhemorrhage represents the most frequent hemorrhagic complication and it is often mild, focal and superficial and involves few high sulci in the convexity. To our knowledge this report represents the first case of atypical subarachnoid hemorrhage localised in the basal cistern associated with reversible cerebral vasoconstriction syndrome. We report a 52 year old woman presented severe sudden onset occipital headache episodes. The neurological examination revealed only horizontal nystagmus in left sight direction; vital signs were normal. Neuroimaging scans (CT and MRI) showed a mild diffuse cerebral swelling without focal lesions. The following days she a thunderclap type headache recurred acutely and a new brain CT showed subarachnoid hemorrhage in the basal cisterns. Non invasive angiography demonstrated a wide segmental vasoconstriction of the cerebral arteries with dilatations of the post-stenotic segments suggesting reversible cerebral vasoconstriction syndrome. New non invasive angiography obtained after 3 months showed a complete resolution of the previous segmental arterial vasoconstrictions.