Article

Intra‐Arterial Milrinone for Reversible Cerebral Vasoconstriction Syndrome

Department of Neurological Sciences, CHA (Enfant-Jésus), Faculty of Medicine, Laval University, Quebec City, QC, Canada.
Headache The Journal of Head and Face Pain (Impact Factor: 3.19). 01/2009; 49(1):142-5. DOI: 10.1111/j.1526-4610.2008.01211.x
Source: PubMed

ABSTRACT Reversible cerebral vasoconstriction syndrome (RCVS) usually presents with recurrent thunderclap headaches and is characterized by multifocal and reversible vasoconstriction of cerebral arteries that can sometimes evolve to severe cerebral ischemia and stroke. We describe the case of a patient who presented with a clinically typical RCVS and developed focal neurological symptoms and signs despite oral treatment with calcium channel blockers. Within hours of neurological deterioration, she was treated with intra-arterial milrinone, a phosphodiesterase inhibitor, which resulted in a rapid and sustained neurological improvement.

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    • "In patients with refractory vasoconstrictions, intra-arterial therapy might be considered. Calcium channel blockers such as nimodipine [Elstner et al. 2009; Klein et al. 2009] or the phosphodiesterase inhibitor milrinone [Bouchard et al. 2009] have been employed in some cases and led to satisfactory outcomes. However, more studies are required. "
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    ABSTRACT: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and reversible cerebral vasoconstrictions. RCVS is more common than previously thought and should be differentiated from aneurismal subarachnoid hemorrhage. RCVS can be spontaneous or evoked by pregnancy or exposure to vasoactive substances. Patients tend to be middle-aged women but pediatric patients have been seen. Up to 80% of sufferers have identifiable triggers. Thunderclap headaches tend to recur daily and last for a period of around 2 weeks, while the vasoconstrictions may last for months. About one-third of patients have blood pressure surges accompanying headache attacks. The potential complications of RCVS include posterior reversible encephalopathy syndrome, ischemic strokes over watershed zones, cortical subarachnoid hemorrhage and intracerebral hemorrhage. Magnetic resonance images including angiography and venography and lumbar punctures are the studies of choice, whereas catheter angiography should not be implemented routinely. Patients with a mean flow velocity of the middle cerebral artery greater than 120 cm/s shown by transcranial color-coded sonography have a greater risk of ischemic complications than those without. The pathophysiology of RCVS remains unknown; sympathetic hyperactivity may play a role. Open-label trials showed calcium channel blockers, such as nimodipine may be an effective treatment in prevention of thunderclap headache attacks. In severe cases, intra-arterial therapy may be considered. Most patients with RCVS recover without sequelae; however, relapse has been reported in a small proportion of patients.
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    ABSTRACT: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches, as well as vasoconstriction of cerebral arteries, which resolves spontaneously in one to three months. This condition has a moderate female preponderance. The mean age of onset is around 45 years. About 60% of the cases are secondary, mainly occurring during postpartum and/or after exposure to vasoactive substances. The main clinical presentation includes multiple recurrent thunderclap headaches over one to three weeks. The major complications of RCVS are localized cortical subarachnoid hemorrhages (cSAH) (20-25%) and parenchymal strokes (5-10%). Complications occur with different time courses: hemorrhages (cSAH and intracerebral hemorrhages), and posterior reversible encephalopathy syndrome are early events occurring during the first week, while ischemic events including TIAs and cerebral infarcts occur significantly later, during the second week. Diagnosis requires the demonstration of the "string and beads" aspect of cerebral arteries by a cerebral angiogram (MRA, CTA or conventional) and the demonstration of the complete or marked normalisation of arteries by a repeat angiogram performed within 12 weeks of onset. Treatment is based on nimodipine that seems to reduce thunderclap headaches within 48h. However, nimodipine has not proven any efficacy against the hemorrhagic and ischemic complications of RCVS. Relapses are possible but rare and have not been reported yet in prospective series. It seems appropriate to advise the patients to avoid sympathomimetic and serotoninergic substances.
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