Possible overlap between reversible cerebral vasoconstriction syndrome and symptomatic vasospasm after aneurysmal subarachnoid hemorrhage

A 34-year-old woman developed acute-onset occipital headache with loss of consciousness. She had a history of episodic severe headache treated by nonsteroidal anti-inflammatory drugs. There was no clear precipitating factor. The headache was described as generalised and bilateral. The pain was excruciating and lasted usually less than 1 h, being sometimes accompanied by nausea and vomiting.

On arrival, she had a GCS of 15, and also with a mild hypo-esthesia in the left arm. The brain CT confirmed the diagnosis of SAH (Fisher 3 radiological score), with a focal hematoma in the territory of the left anterior cerebral artery (ACA), blood in the subarachnoid spaces of the left sylvian fissure and into the occipital horn of the left lateral ventricle. The cerebral angiography revealed four distinct aneurysms. The ruptured aneurysm (8 mm × 9 mm) was located on the junction of the A1–A2 segments of the left ACA. The three unruptured aneurysms were on the A1 segment of the left ACA (1.5 mm diameter), on the A2 segment (3 mm × 2 mm) and at the origin of the posterior communicating artery. The clipping of the four aneurysms was performed without peri-operative complications and after extubation the patient presented only neck stiffness and photophobia. She received oral nimodipine and continuous infusion of magnesium sulphate. On day 5 from bleeding, she was found drowsy, with a slurry speech, and a vasospasm on the left middle cerebral artery (MCA) was suspected on transcranial Doppler (TCD). Systolic arterial pressure was increased to 150 mmHg by norepinephrine infusion followed by rapid neurological improvement. She complained on days 9 and 10 of acute-onset but short-lasting excruciating headache. Norepinephrine infusion was tapered down and stopped on day 12. Velocities normalised on TCD performed on days 7 and 10. She was discharged from ICU on day 12 with a normal neurological examination but was readmitted the same night with right hemiplegia and aphasia. The diffusion-weighted imaging (DWI) during brain MR showed a limited hyperintense lesion in the left cingulum. As the symptoms persisted after 12 h of intensive treatment, rescue therapy with intra-arterial vasodilatory agents was considered. Nimodipine (3 mg over 20 min) was infused in loco (left MCA) during cerebral angiography, followed by intravenous continuous infusion of milrinone (0.75 μg/kg per min). There was a prompt recovery of aphasia and a regression of the motor deficit in the upper right limb. The patient was able to move the right lower limb on day 16. Milrinone infusion was continued for a period of 11 days, and during this treatment, norepinephrine infusion had to be increased to 2 μg/kg per min to obtain an arterial systolic blood pressure of 150 mmHg. The evolution of the clinical course and of the TCD/radiological investigations is summarised in Table 1. On day 25, the patient became drowsy and complained again from severe headache. Bilateral vasospasm was demonstrated by TCD and angiography (Fig. 1a, b). Intra-arterial infusion of milrinone (15 mg over 60 min, divided in two doses in both MCA) was performed, and intravenous infusion was started again as in the previous episode, for a total of 5 days (together with high doses of norepinephrine). Symptoms regressed, except for headache (multiple episodes every day). Finally, on day 30, dysarthria and right hemiparesia were transiently noted. Magnesium sulphate infusion and norepinephrine (as a part of the triple H therapy) were stopped on day 32. The patient was discharged from the ICU on day 38 after a normal neurological examination but with persisting high velocities at TCD examination of the right MCA. The velocities normalised completely only on day 43. The patient made a complete recovery (including headache) at 3 months follow-up.