Reversible Segmental Cerebral Vasoconstriction (Call-Fleming Syndrome): the Role of Calcium Antagonists

Abstract

In 1988, Call and colleagues described four patients and reviewed a total of 19 patients from the literature who presented with a self-limited syndrome of recurrent, sudden, high-intensity headaches ('thunderclap headache') associated with nausea, vomiting, photophobia, generalized seizures (7/19), recurrent transient neurological symptoms (7/19), or permanent neurological deficits such as hemiparesis, cortical blindness (4/19) (1). Three patients became comatose and died. The common finding in all patients was the presence of diffuse segmental cerebral vasospasm involving the distal internal carotid, basilar, and major arteries of the circle of Willis which was fully reversible within weeks to months. Brain and/or cerebral blood vessel biopsies were negative in 6/19 patients and CSF examinations were mildly abnormal in five patients (three patients with elevated CSF protein 51-125), two patients with CSF lymphocytic pleocytosis (6-16 leucocytes). They felt that the segmental vasospasm was probably due to a physiological abnormality, given the lack of a structural correlate, cellular infiltrate, or hypertension in most patients.Similar clinical-angiographic patterns (sudden severe headache and diffuse vasospasm) have been described in patients with a history of migraine, as well as after carotid endarterectomy, postpartum period, Guillain-Barré syndrome, sexual intercourse, unruptured intracranial aneurysm, exertion, severe hypertension, and after administration of serotonergic, sympathomimetic medications, and illicit drugs such as cocaine (1-8).Nowak and colleagues describe the case of a 63-year-old woman who presented with multiple discrete thunderclap headaches over a 2-week period with delayed neurological deficits (cortical visual field loss and hemiparesis), diffuse segmental cerebral vasospasm, and bilateral occipital-parietal cerebral infarctions (9). Resolution of vasospasm was documented by transcranial Doppler 1 day after the intravenous administration of nimodipine and magnesium sulphate, only to recur after nimodipine was discontinued. Ultimately, headaches and vasospasm resolved 17 days after the initial symptoms. The authors conclude that calcium channel antagonists may be important therapeutic agents in the treatment of reversible cerebral vasoconstriction in patients with Call-Fleming syndrome. Their conclusion is supported by a similar case reported by Sturm and Macdonell, who described a similar clinical scenario in a 58-yearold woman who presented with recurrent severe thunderclap headaches over a 14-day period, diffuse reversible cerebral arterial vasospasm, and delayed bilateral watershed ischaemic stroke with cortical blindness (10). Vasospasm and clinical deterioration appeared to be halted by the use of intravenous nimodipine.As the literature continues to grow with case reports of this unusual disorder, the clinical spectrum is becoming more refined. As these and other cases illustrate, the clinical-angiographic syndrome of recurrent thunderclap headaches, diffuse reversible vasospasm,...

Reversible Cerebral Vasoconstriction Syndrome: Treatment with Combined Intra-Arterial Verapamil Infusion and Intracranial Angioplasty

“…Multisegmental vasoconstriction has been reported in patients with a history of migraines, as well as in the postpartum period, following sexual intercourse, following aneurysmal SAH, and after admin- istration of serotonergic or sympathomimetic drugs. 9 Similar angiographic findings can also be associated with cerebral vasculitis or primary angiitis of the CNS, but these patients present with gradually progressive neurologic symptoms, encephalopathy, and CSF abnormalities in 80%-90% of cases. 10 The clinical context of pseudoephedrine ingestion, acute thunderclap headache, the rapidly progressive clinical course, and the imaging findings combined led to a diagnosis of RCVS in our case.…”

“…16 Alleviation of symptoms and reversal of vascular abnormalities have been reported with calcium channel blockers, glucocorticoids, magnesium sulfate, and observation. 1,9 Unfortunately, the clinical course may be devastating; symptoms may clear initially only to return within days. 17 Prolonged vasoconstriction leads to infarction of Յ54% of patients, most commonly in watershed areas and the posterior circulation.…”

Reversible cerebral angiopathy

“…C Initial MRI/MRA showing a small right frontal hemorrhage (gradient-echo T2*-weighted sequence) and segmental stenoses on both middle cerebral arteries (arrows). D No more abnormality was present on MRA one month later [2]. Close relationship between IV nimodipine induction and BFV improvement on TCD sonography has suggested some direct pharmacological intervention on the vasospasms [7].…”

Treatment of Acute Ischemic Stroke: Beyond Thrombolysis and Supportive Care

“…Some cases may recover spontaneously [42]. Optimal treatment has not been clearly established; however, agents used have included calcium channel blockers to treat vasospasm (particularly nimodipine and verapamil), magnesium (in the postpartum period) and high-dose steroids [42][43][44][45][46]. Patients should have follow-up vascular imaging to determine the evolution of their vasoconstriction.…”