Douglas Gonsales1, Fabiano das Gracas1, Roberta Santos1, Pedro Aguilar-Salinas1, Ricardo A Hanel2. 1. Baptist Neurological Institute, Lyerly Neurosurgery-Baptist Health, Jacksonville, Florida, USA. 2. Baptist Neurological Institute, Lyerly Neurosurgery-Baptist Health, Jacksonville, Florida, USA. Electronic address: rhanel@lyerlyneuro.com.
Abstract
BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare entity with an unknown pathophysiology. RCVS has been reported to occur more frequently in women age 20-50 years. Several causative mechanisms have been postulated involving transient deregulation of cerebral arterial tone, small vessel endothelial dysfunction, biochemical factors, hormonal deregulation, oxidative stress, and genetic predisposition. All these mechanisms and triggers are related to sympathetic overactivation, which eventually produces vasoconstriction. RCVS is distinguished by acute severe recurrent thunderclap headaches with or without other neurologic symptoms. However, the diagnosis can be challenging, requiring a high level of suspicion by the clinician, and RCVS is often underdiagnosed. CASE DESCRIPTION: We present an unusual case of an 18-year-old female who developed RCVS after embolization of a dural arteriovenous fistula with onyx embolic material. Cerebral angiography was performed and verapamil was administered intra-arterially, resulting in a slight decrease in vasoconstriction with clinical improvement. The patient was maintained on oral verapamil during hospitalization. At a 7-month follow-up, the patient was neurologically stable, and cerebral angiography demonstrated no signs of vasoconstriction. CONCLUSIONS: Endovascular procedures are a rare trigger for the development of RCVS and may be misdiagnosed. Prompt recognition of symptoms and diagnosis with treatment are necessary to reduce the risk of stroke. The management approach should be based on the premise of discontinuing precipitating drugs and administering calcium channel blocking agents.
BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare entity with an unknown pathophysiology. RCVS has been reported to occur more frequently in women age 20-50 years. Several causative mechanisms have been postulated involving transient deregulation of cerebral arterial tone, small vessel endothelial dysfunction, biochemical factors, hormonal deregulation, oxidative stress, and genetic predisposition. All these mechanisms and triggers are related to sympathetic overactivation, which eventually produces vasoconstriction. RCVS is distinguished by acute severe recurrent thunderclap headaches with or without other neurologic symptoms. However, the diagnosis can be challenging, requiring a high level of suspicion by the clinician, and RCVS is often underdiagnosed. CASE DESCRIPTION: We present an unusual case of an 18-year-old female who developed RCVS after embolization of a dural arteriovenous fistula with onyx embolic material. Cerebral angiography was performed and verapamil was administered intra-arterially, resulting in a slight decrease in vasoconstriction with clinical improvement. The patient was maintained on oral verapamil during hospitalization. At a 7-month follow-up, the patient was neurologically stable, and cerebral angiography demonstrated no signs of vasoconstriction. CONCLUSIONS: Endovascular procedures are a rare trigger for the development of RCVS and may be misdiagnosed. Prompt recognition of symptoms and diagnosis with treatment are necessary to reduce the risk of stroke. The management approach should be based on the premise of discontinuing precipitating drugs and administering calcium channel blocking agents.