We report an additional series of 11 medically refractory cases of presumed or proved reversible cerebral vasoconstriction syndrome successfully treated with intra-arterial verapamil infusion. This article has not yet been cited by articles in journals that are participating in Crossref Cited-by Linking. RCVS can be easily mistaken for primary angiitis of the central nervous system even on digital subtraction angiography. Except for 1 groin pseudoaneurysm successfully treated with thrombin injection, there were no complications reported from the angiographic procedures. All imaging, clinical records, laboratory results, and treatment and follow-up data were obtained from the electronic medical records and PACS of the hospitals and were reviewed by 2 experienced neurointerventionalists (K.A.B./A.R.) Other additional medications (mimodipine, morphine, diazepam, levetiracetam) were also stopped. All patients met the 5 criteria for RCVS (Table 1); 4 patients had ambulatory imaging documentation of complete vasoconstriction resolution, and the remaining patients without ambulatory follow-up had reversibility of the vasoconstriction at the time of treatment. The Neurological Institute is a leader in treating and researching the most complex neurological disorders and advancing innovations in neurology. All patients retrospectively met the angiographic criteria for treatment, and all territories treated met the criteria for an angiographic response. This outcome can be interpreted as an early confirmation of the reversible nature of the vasoconstriction. INTRODUCTION — Reversible cerebral vasoconstriction syndrome (RCVS) represents a group of conditions that show reversible multifocal narrowing of the cerebral arteries with clinical manifestations that typically include thunderclap headache and sometimes include neurologic deficits related to brain edema, stroke, or seizure. Reversible Cerebral Vasoconstriction Syndrome: Treatment with Combined Intra-Arterial Verapamil Infusion and Intracranial Angioplasty, Reversible cerebral vasoconstriction syndrome, Reversible Cerebral Vasoconstriction Syndrome and Intracranial Hemorrhage. for confirmation of treatment indications and results. In this case series, we present 11 patients with documented or presumed RCVS who presented with or progressed to clinically relevant vasoconstriction and in whom IA treatment with verapamil resulted in clinical and radiographic improvement. Treatment options include medical optimization of risk factors, corticosteroids and oral or intra-arterial calcium channel blockers. The caliber increases markedly following verapamil injection, with some residual focal narrowing (arrows in D). We thank Dr Selina Ackermann from the University Hospital Basel for editorial assistance. © 2020 by the American Society of Neuroradiology | Print ISSN: 0195-6108 Online ISSN: 1936-959X. IA verapamil was administered proximally in the affected vascular territory. Cleveland Clinic is a non-profit academic medical center. He presented twice to an outside hospital, and ultimately CT and CTA were performed, which showed vasoconstriction. Five patients returned to the angiography suite multiple times because of recurrent symptoms or elevated transcranial Doppler studies (mean middle cerebral artery velocity greater than 120 cm/sec or greater than 20 percent elevation of flow velocity above baseline). A semiquantitative classification of grade was performed by visually assessing the luminal narrowing compared with the best normal vessel caliber on either the same study or prior cerebral angiograms using the following categories: 1) mild: luminal narrowing of <30% of normal caliber; 2) moderate: between 30% and 60% of normal caliber; and 3) severe: luminal narrowing of >60% of normal caliber, similar to the stratification described by Jun et al.10 At least 1 segmental focus of at least moderate proximal vasoconstriction or any distal vasoconstriction would indicate the need for intra-arterial treatment. Encephalopathic symptoms resolved, steroid medication was stopped, and oral verapamil was continued. Radiopaedia. * *Money was paid to the individual. The patient returned to the angiography suite for uncomplicated angioplasty of the severe narrowing of the right ICA. In most cases, RCVS is a benign and self-limiting condition with spontaneous resolution; however, it can also be a complex clinicoradiologic challenge.4 Despite oral and intravenous calcium channel blockers,1,13⇓⇓-16 additional pathologic imaging features besides the typical “string of beads” appearance of vasoconstriction have been described in 12%–81% of patients.1 Unfortunately, the diagnosis is often delayed, and the initial clinical treatment is variable if not disorganized due to inexperience with the condition and its overlap with other disorders such as aneurysmal or nonaneurysmal SAH and vasculitis. At 5-year follow-up, a slight weakness of the lower extremities and left lower quadrantanopia was still present. Advertising on our site helps support our mission. Enter multiple addresses on separate lines or separate them with commas. Policy, Get useful, helpful and relevant health + wellness information. Careful clinical and imaging evaluation is crucial, and a high index of suspicion for possible complications must be maintained to guide optimal management. Her main symptoms were headache, confusion, and hallucinations; she also developed left hemivisual field inattention. Oral nimodipine was discontinued on hospital day 12 due to resolution of symptoms. Since 2006, the authors have used intra-arterial verapamil as a treatment option in patients with suspected RCVS and clinical deterioration. Reversible Cerebral Vasoconstriction Syndrome: Treatment with Combined Intra-Arterial Verapamil Infusion and Intracranial Angioplasty Accessed 3/30/2016. She was ultimately transferred to our hospital. Cortical SAH has been described in up to 34%,7,14 and posterior reversible encephalopathy syndrome, in 9%–38% of patients with RCVS. We propose that in cases of severe RCVS, PTA plus intra-arterial verapamil administration should be considered a therapeutic option to reverse neurologic deficits. Furthermore, we propose that the reversal of vasoconstriction, as seen on angiography, could fulfill a diagnostic criterion. Multifocal vasoconstriction was seen in the left anterior circulation and was most severe in the bilateral posterior circulation (Fig 1). Verapamil was diluted in saline to a final concentration of 1 mg/mL and pulse-infused at a rate of 1 mL/min through the diagnostic catheter, meaning that 1 mL of verapamil was manually injected every minute through the side port of a 3-way valve connected to the diagnostic catheter and also to a continuous heparinized saline flush, allowing intermittent flush. RCVS remains a challenging diagnosis, and its severity can be underestimated. A total amount of 30 mg of IA verapamil was injected in both ICAs (10 mg, respectively) and the left vertebral artery (10 mg), which led to improvement of intracranial vessel caliber and improvement of symptoms. The use of calcium channel blockers such as Cardizem® and nimodipine can reduce headaches. Numerous inciting factors for RCVS have been described, but the list of triggers continues to grow.1,2 Treatment options include medical optimization of risk factors, corticosteroids, and oral or intra-arterial calcium channel blockers.3 If left untreated, RCVS can lead to permanent neurologic disability and even death.4⇓-6 Complications such as hemorrhage and stroke are well-documented.1,7 In the past decade, on the basis of the experience with treatment of patients with vasospasm secondary to SAH, an endovascular approach using the intra-arterial (IA) infusion of calcium channel blockers in patients with refractory RCVS has emerged, with case reports and a small series of promptly improved vessel caliber and also symptoms.8,9 The underlying hypothesis is that the vasoconstriction of RCVS is vasospasm as observed in SAH and that similar treatment approaches will similarly prevent the development of permanent neurologic deficits. Endovascular treatment was performed using a standard biplane angiographic technique starting with a diagnostic series of both internal carotid arteries and the dominant vertebral artery, with the patient under systemic heparinization.
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