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reversible cerebral vasoconstriction syndrome (Call-Fleming syndrome)

Etiology: - 9% of patients postpartum, pregnancy - preeclampsia, eclampsia - HELLP syndrome - 42% exposed to vasoconstrictor agent - sympathomimetics (ampethamine, others) - triptans, ergot alkaloids - SSRI, SNRI, MAO inhibitors - nicotine - cocaine, methamphetamine, ecstasy - cannabis - head & neck trauma & neurosurgery - acute cerebrovascular disorders - cervical artery dissection - ceebral endovascular procedures, cerebral angiography - cerebral venous sinus thrombosis - blood products (packed RBC, immune globulin) - meningitis - catecholamine-releasing tumors (pheochromocytoma) [3] Epidemiology: - mean age, 42 years - 81% women - 2nd most common cause of thunderclap headache after subarachnoid hemorrhage [3[ Pathology: - transient segmental cerebral vasoconstriction Clinical manifestations: - generally presents with severe recurrent thunderclap headache - occurs over a few days or weeks - focal neurological deficits (50%) - aphasia - hemiparesis - ataxia - case presentation of abrupt-onset severe headache lasting 6-8 hours [3] - case presentation with throbbing, nausea, photophobia involving the entire cranium [3] - seizures (17%) Special laboratory: - lumbar puncture (after head CT) - CSF analysis - no evidence of subarachnoid hemorrhage Radiology: - non-contrast CT of the head (emergency) - cerebrovascular imaging - modalities - magnetic resonance angiography (MRA)* - CT angiography* - cerebral angiography (invasive) - findings - narrowing of multiple cerebral arteries bilaterally - abnormalities reversed over time - infarction (39%) - convexity subarachnoid hemorrhage (34%) - hemorrhage (20%) - white matter hyperintensities with dynamic temporal evolution that parallels disease severity [5] - cerebral edema [3] * screening imaging modalities of choice Differential diagnosis: - subarachnoid hemorrhage - isolated central nervous system vasculitis Management: - normalization of blood pressure [3] - eliminate offending agents [3] - agents used of uncertain value - glucocorticoids may worsen outcomes [3] - calcium channel blockers - prognosis: - 78% of patients have no substantial residual disability - 9% with severe disability - mortality 2%

General

syndrome vasoconstriction thunderclap headache

References

  1. Singhal AB et al. Reversible cerebral vasoconstriction syndromes: Analysis of 139 cases. Arch Neurol 2011 Aug; 68:1005 PMID: 21482916
  2. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol. 2009 Oct;9(5):256-67 PMID: 19762885
  3. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  4. Yancy H, Lee-Iannotti JK, Schwedt TJ, Dodick DW. Reversible cerebral vasoconstriction syndrome. Headache. 2013 Mar;53(3):570-6 PMID: 23489219
  5. George J. White Matter Lesions May Be Partially Reversible in RCVS. Dynamic pattern distinct from aging or other neurological disorders. MedPage Today. June 04, 2018 https://www.medpagetoday.com/neurology/generalneurology/73272? - Chen SP, Chou KH, Fuh JL et al Dynamic changes in white matter hyperintensities in reversible cerebral vasoconstriction syndrome. JAMA Neurol. Published online June 4, 2018 PMID: 29868878 https://jamanetwork.com/journals/jamaneurology/article-abstract/2682655
  6. Ducros A, Wolff V. The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers. Headache. 2016 Apr;56(4):657-73. Review. PMID: 27015869