Reversible cerebral vasoconstriction syndrome: current and future perspectives

Figures & data

Figure 1. Characteristic angiographic findings of reversible cerebral vasoconstriction syndrome.

(A) Multi-focal segmental vasoconstrictions and (B) their normalization in a patient with reversible cerebral vasoconstriction syndrome (vasoconstrictions are indicated by black arrows).

Figure 2. Complications of reversible cerebral vasoconstriction syndromes.

(A) Posterior reversible encephalopathy syndrome; (B) cerebral infarction; (C) intracerebral hemorrhage.

Figure 3. Proposed pathophysiology of reversible cerebral vasoconstriction syndromes.

cSAH: Cortical subarachnoid hemorrhage, ICH: Intracerebral hemorrhage; PRES: Posterior reversible encephalopathy syndrome; TCH: Thunderclap headache.

Figure 4. Algorithm of diagnosis and treatment of thunderclap headache.

CCB: Calcium-channel blockers; CSF: Cerebrospinal fluid; CT: Computed tomography; ia.: Intra-arterial; iv.: Intravenous; MRA: Magnetic resonance angiography; MRV: Magnetic resonance venography; RCVS: Reversible cerebral vasoconstriction syndromes; SAH: Subarachnoid hemorrhage; TCCS: Transcranial color-coded sonography; TCH: Thunderclap headache.

Table 1. Possible etiologies and associated conditions of reversible cerebral vasoconstriction syndrome.

General categories	Specific entities
Primary
Spontaneous or evoked by triggers	Primary thunderclap headache Exertional headache Headache associated with sexual activity Cough headache Bath-related thunderclap headache
Secondary
Pregnancy and the postpartum period	Postpartum angiopathy (± use of vasoactive drugs) Eclampsia/preeclampsia
Vasoactive substances	Illicit drugs: Cannabis, cocaine, ecstasy, amphetamines, LSD Ergots: Ergotamine tartrate, methergine, methylergometrine, lisuride, bromocriptine Sympathomimetics: Ephedrine, isometheptene, pseudoephedrine, diet pills, phenylpropanolamine Serotonergic drugs: Selective serotonin-reuptake inhibitors, triptans Immunosuppressants: Tacrolimus (FK-506), cyclophosphamide, IFN-α Others: Nicotine patches, Ginseng, licorice, indomethacin, binge drinking, oral contraceptive pills, hormonal ovarian stimulation for intrauterine insemination
Catecholamine secreting tumors	Pheochromocytoma, bronchial carcinoid tumor, glomus tumors
Extra- or intra-cranial arterial disorders or procedures	Cervical artery dissection, aortic dissection, unruptured intracranial aneurysm, fibromuscular dysplasia, postcarotid endarterectomy
Blood products	Erythropoietin, intravenous immunoglobulin, massive blood transfusion
Intracranial disorders or surgery	Intracranial hypotension, intracranial hemorrhage, spinal subdural hematoma, neurosurgery, head trauma
Miscellaneous	Hypercalcemia, systemic lupus erythematosus, porphyria, microangiopathic hemolytic anemia, triplet cesarean delivery, ascent to high altitude, tonsillectomy, autonomic dysreflexia

LSD: Lysergic acid diethylamide.

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