The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a 'string and beads' appearance on cerebral arteries, which resolves spontaneously in 1-3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 +/- 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.

Decreased dopamine brain reactivity in marijuana abusers is associated with negative emotionality and addiction severity

“…To ensure that the DV responses in the controls were consistent with prior findings, we performed a secondary analysis on the effects of MP on the cerebellar DV in an independent cohort of controls, which showed a 12% reduction, and in a sample of adults with attention deficit hyperactivity disorder (ADHD), which also showed an 11% reduction (for controls of the current cohort, the cerebellar DV decrease was 13 ± 11%). The mechanism underlying the lack of an effect of MP in cerebellar DV in abusers is unclear but could reflect the effects of chronic marijuana on cerebrovascular reactivity (increased cerebral vascular resistance) (23)(24)(25), which might have prevented MP-induced vasoconstriction and associated reductions in radiotracer delivery to the brain. The attenuated decreases in DV with MP in the marijuana abusers were observed throughout the brain but were most accentuated in cerebellum.…”

“…However, prior studies are limited by their small sample sizes (ranging from six to 16 subjects). Also, prior studies did not control for the potential confounds that the changes in cerebral vascular resistance associated with marijuana abuse (23)(24)(25) could have on the delivery of the radiotracer to the brain when using a stimulant drug as pharmacological challenge, because stimulants decrease cerebral blood flow (26). Thus, the extent to which there are changes in brain DA signaling in marijuana abusers is still unclear.…”

Basi‐parallel anatomical scanning–magnetic resonance imaging for the diagnosis of reversible cerebral vasoconstriction syndrome of the basilar artery: a case report

“…Although CT angiography can also be used to assess the intracerebral vessels if MRI is not readily available, radiation exposure limits its use 5. Magnetic resonance angiography has been confirmed as valid to evaluate vasoconstriction in patients with RCVS 6. However, MRA is potentially affected by flow‐related inhomogeneities.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Angiography may reveal typical findings of beading, with arterial narrowing followed by arterial dilation. The differential diagnosis of PACNS includes the reversible cerebral vasoconstriction syndrome, which is a self-limited disorder that occurs in individuals following exposure to vasoactive substances [115,116].…”